Endocrinology and Metabolism

Review Article

Miscellaneous
Toward Systems-Level Metabolic Analysis in Endocrine Disorders and Cancer: Aliya Lakhani, Da Hyun Kang, Yea Eun Kang, Junyoung O. Park; Endocrinol Metab. 2023;38(6):619-630. Published online November 21, 2023; DOI: https://doi.org/10.3803/EnM.2023.1814

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Metabolism is a dynamic network of biochemical reactions that support systemic homeostasis amidst changing nutritional, environmental, and physical activity factors. The circulatory system facilitates metabolite exchange among organs, while the endocrine system finely tunes metabolism through hormone release. Endocrine disorders like obesity, diabetes, and Cushing’s syndrome disrupt this balance, contributing to systemic inflammation and global health burdens. They accompany metabolic changes on multiple levels from molecular interactions to individual organs to the whole body. Understanding how metabolic fluxes relate to endocrine disorders illuminates the underlying dysregulation. Cancer is increasingly considered a systemic disorder because it not only affects cells in localized tumors but also the whole body, especially in metastasis. In tumorigenesis, cancer-specific mutations and nutrient availability in the tumor microenvironment reprogram cellular metabolism to meet increased energy and biosynthesis needs. Cancer cachexia results in metabolic changes to other organs like muscle, adipose tissue, and liver. This review explores the interplay between the endocrine system and systems-level metabolism in health and disease. We highlight metabolic fluxes in conditions like obesity, diabetes, Cushing’s syndrome, and cancers. Recent advances in metabolomics, fluxomics, and systems biology promise new insights into dynamic metabolism, offering potential biomarkers, therapeutic targets, and personalized medicine.

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Development and validation of an interpretable machine learning model for predicting intraoperative HDI in PPGL based on intratumoral and peritumoral CT radiomics
Shurong Li, Zhiqiang Zhang, Yubing Zhang, Yulong Chen, Jian Ling, Qingfen Hong, Xuanling Wu, Fufu Zheng, Cheng Luo
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A. S. Zhdanova, Z. E. Belaya, G. A. Melnichenko
Problems of Endocrinology.2026; 71(6): 56. CrossRef
Factors Associated with the Development of Skin Lesions in Hospitalized Patients Admitted to a Nursing Preventive Care Program in Colombia
Gaby E. Escobar, Ángela F Espinosa, Olga L. Cortés, Nicolás Molano González
Investigación y Educación en Enfermería.2025;[Epub] CrossRef
Editorial: Tumor metabolism and programmed cell death
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Molecular subtypes of clear cell renal carcinoma based on PCD-related long non-coding RNAs expression: insights into the underlying mechanisms and therapeutic strategies
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Brief Report

Adrenal Gland
Urinary Free Metanephrines for Diagnosis of Pheochromocytoma and Paraganglioma: Jiyeon Ahn, Ji Yun Park, Gyuri Kim, Sang-Man Jin, Kyu Yeon Hur, Soo-Youn Lee, Jae Hyeon Kim; Endocrinol Metab. 2021;36(3):697-701. Published online June 1, 2021; DOI: https://doi.org/10.3803/EnM.2020.925

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Background
Pheochromocytoma and paraganglioma (PPGL) is diagnosed through biochemical confirmation of excessive catecholamines in urine and plasma. Recent technological developments have allowed us to measure urinary free metanephrines; however, the diagnostic accuracy of these new methods and the diagnostic cutoff values have not been evaluated.
Methods
This is a retrospective study of 595 subjects, including 71 PPGL cases and 524 controls. PPGL was based on pathological confirmation. Subjects with no evidence of PPGL over 2 years were included in the control group.
Results
Urinary free metanephrines yielded similar area under the curve (AUC) to urinary fractionated metanephrines and plasma free metanephrines. However, urinary free normetanephrine yielded a better AUC than did urinary fractionated normetanephrine. The optimal cutoff for urinary free metanephrine and normetanephrine corrected for urinary creatinine yielded 97.2% sensitivity and 98.1% specificity.
Conclusion
Urinary free metanephrines are a reliable method for diagnosing PPGL in Asian populations compared with existing biochemical methods.

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Special Article

Adrenal gland
Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force: Eu Jeong Ku, Kyoung Jin Kim, Jung Hee Kim, Mi Kyung Kim, Chang Ho Ahn, Kyung Ae Lee, Seung Hun Lee, You-Bin Lee, Kyeong Hye Park, Yun Mi Choi, Namki Hong, A Ram Hong, Sang-Wook Kang, Byung Kwan Park, Moon-Woo Seong, Myungshin Kim, Kyeong Cheon Jung, Chan Kwon Jung, Young Seok Cho, Jin Chul Paeng, Jae Hyeon Kim, Ohk-Hyun Ryu, Yumie Rhee, Chong Hwa Kim, Eun Jig Lee; Endocrinol Metab. 2021;36(2):322-338. Published online April 6, 2021; DOI: https://doi.org/10.3803/EnM.2020.908

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Pheochromocytoma and paraganglioma (PPGLs) are rare catecholamine-secreting neuroendocrine tumors but can be life-threatening. Although most PPGLs are benign, approximately 10% have metastatic potential. Approximately 40% cases are reported as harboring germline mutations. Therefore, timely and accurate diagnosis of PPGLs is crucial. For more than 130 years, clinical, molecular, biochemical, radiological, and pathological investigations have been rapidly advanced in the field of PPGLs. However, performing diagnostic studies to localize lesions and detect metastatic potential can be still challenging and complicated. Furthermore, great progress on genetics has shifted the paradigm of genetic testing of PPGLs. The Korean PPGL task force team consisting of the Korean Endocrine Society, the Korean Surgical Society, the Korean Society of Nuclear Medicine, the Korean Society of Pathologists, and the Korean Society of Laboratory Medicine has developed this position statement focusing on the comprehensive and updated diagnosis for PPGLs.

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Successful Laparoscopic Resection of a Giant Para-aortic Pheochromocytoma: A Case Report
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A Prospective Comparative Study of 18F-FDOPA PET/CT Versus 123I-MIBG Scintigraphy With SPECT/CT for the Diagnosis of Pheochromocytoma and Paraganglioma
Changhwan Sung, Hyo Sang Lee, Dong Yun Lee, Yong-il Kim, Jae Eun Kim, Sang Ju Lee, Seung Jun Oh, Tae-Yon Sung, Yu-Mi Lee, Young Hoon Kim, Beom-Jun Kim, Jung-Min Koh, Seung Hun Lee, Jin-Sook Ryu
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Germline Mutations and Phenotypic Associations in Korean Patients With Pheochromocytoma and Paraganglioma: A Multicenter Study and Literature Review
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Original Articles

Clinical Study
Whole Exome Sequencing Identifies Novel Genetic Alterations in Patients with Pheochromocytoma/Paraganglioma: Soo Hyun Seo, Jung Hee Kim, Man Jin Kim, Sung Im Cho, Su Jin Kim, Hyein Kang, Chan Soo Shin, Sung Sup Park, Kyu Eun Lee, Moon-Woo Seong; Endocrinol Metab. 2020;35(4):909-917. Published online December 23, 2020; DOI: https://doi.org/10.3803/EnM.2020.756

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Background
Pheochromocytoma and paragangliomas (PPGL) are known as tumors with the highest level of heritability, approximately 30% of all cases. Clinical practice guidelines of PPGL recommend genetic testing for germline variants in all patients. In this study, we used whole exome sequencing to identify novel causative variants associated with PPGL to improve the detection of rare genetic variants in our cohort.
Methods
Thirty-six tested negative for pathogenic variants in previous Sanger sequencing or targeted gene panel testing for PPGL underwent whole exome sequencing. Whole exome sequencing was performed using DNA samples enriched using TruSeq Custom Enrichment Kit and sequenced with MiSeq (Illumina Inc.). Sequencing alignment and variant calling were performed using SAMtools.
Results
Among previously mutation undetected 36 patients, two likely pathogenic variants and 13 variants of uncertain significance (VUS) were detected in 32 pheochromocytoma-related genes. SDHA c.778G>A (p.Gly260Arg) was detected in a patient with head and neck paraganglioma, and KIF1B c.2787-2A>C in a patient with a bladder paraganglioma. Additionally, a likely pathogenic variant in BRCA2, VUS in TP53, and VUS in NFU1 were detected.
Conclusion
Exome sequencing further identified genetic alterations by 5.6% in previously mutation undetected patients in PPGL. Implementation of targeted gene sequencing consisted of extended genes of PPGL in routine clinical screening can support the level of comprehensive patient assessment.

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Metastatic Pheochromocytoma in a Patient With Li-Fraumeni Syndrome
Sotiris Loizidis, Christiana Matthaiou, Efrosini Iacovou, Karel Pacak, Ashley Grossman
JCEM Case Reports.2025;[Epub] CrossRef
Bilateral Pheochromocytoma in a BRCA2 Carrier: Coincidence or Emerging Association?
Poovarasan S, Shahrukh Memon, Rudrakshi Mahaldar, Saurabh Raj
Case Reports in Oncology.2025; 18(1): 1247. CrossRef
Patient Sex and Origin Influence Distribution of Driver Genes and Clinical Presentation of Paraganglioma
Susan Richter, Nicole Bechmann
Journal of the Endocrine Society.2024;[Epub] CrossRef
Germline Mutations and Phenotypic Associations in Korean Patients With Pheochromocytoma and Paraganglioma: A Multicenter Study and Literature Review
Kwan Hoon Jo, Jaewoong Lee, Jaeeun Yoo, Hoon Seok Kim, Eun Sook Kim, Je Ho Han, Yi Sun Jang, Jae-Seung Yun, Jang Won Son, Soon Jib Yoo, Seung Hwan Lee, Dong Jun Lim, Hyuk-Sang Kwon, Seungok Lee, Sungdae Moon, Myungshin Kim
Annals of Laboratory Medicine.2024; 44(6): 591. CrossRef
Clinical exome next‑generation sequencing panel for hereditary pheochromocytoma and paraganglioma diagnosis
Beatrice Melli, Vincenza Cusenza, Sandra Martinelli, Federica Castiglione, Loretta Fornaciari, Andrea Palicelli, Luca Braglia, Enrico Farnetti, Aurelio Negro, Simonetta Rosato, Andrea Frasoldati, Maicol Baldini, Chiara Grasselli, Davide Nicoli
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Lucie Landen, Anne De Leener, Manon Le Roux, Bénédicte Brichard, Selda Aydin, Dominique Maiter, Philippe A. Lysy
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Rozalyn L. Rodwin, Sanyukta K. Janardan, Erin W. Hofstatter, Nina S. Kadan-Lottick
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A case of juvenile-onset pheochromocytoma with KIF1B p.V1529M germline mutation
Masahiro Nezu, Yosuke Hirotsu, Kenji Amemiya, Miho Katsumata, Tomomi Watanabe, Soichi Takizawa, Masaharu Inoue, Hitoshi Mochizuki, Kyoko Hosaka, Toshio Oyama, Masao Omata
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Systematic analysis of expression profiles and prognostic significance for MMDS-related iron–sulfur proteins in renal clear cell carcinoma
Ling Yang, Yu-Xin Chen, Ying-Ying Li, Xiao-Juan Liu, Yong-Mei Jiang, Jia Mai
Scientific Reports.2022;[Epub] CrossRef
Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force
Eu Jeong Ku, Kyoung Jin Kim, Jung Hee Kim, Mi Kyung Kim, Chang Ho Ahn, Kyung Ae Lee, Seung Hun Lee, You-Bin Lee, Kyeong Hye Park, Yun Mi Choi, Namki Hong, A Ram Hong, Sang-Wook Kang, Byung Kwan Park, Moon-Woo Seong, Myungshin Kim, Kyeong Cheon Jung, Chan
Endocrinology and Metabolism.2021; 36(2): 322. CrossRef
Recurrent Germline Mutations of CHEK2 as a New Susceptibility Gene in Patients with Pheochromocytomas and Paragangliomas
Yinjie Gao, Chao Ling, Xiaosen Ma, Huiping Wang, Yunying Cui, Min Nie, Anli Tong, Dario De Biase
International Journal of Endocrinology.2021; 2021: 1. CrossRef

Clinical Study
Genetic Analysis and Clinical Characteristics of Hereditary Pheochromocytoma and Paraganglioma Syndrome in Korean Population: Heewon Choi, Kyoung Jin Kim, Namki Hong, Saeam Shin, Jong-Rak Choi, Sang Wook Kang, Seung Tae Lee, Yumie Rhee; Endocrinol Metab. 2020;35(4):858-872. Published online December 23, 2020; DOI: https://doi.org/10.3803/EnM.2020.683

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Background
Pheochromocytoma and paragangliomas (PPGL) are hereditary in approximately 30% to 40% cases. With the advancement of genetic analysis techniques, including next-generation sequencing (NGS), there were attempts to classify PPGL into molecular clusters. With NGS being applied to clinical settings recently, we aimed to review the results of genetic analysis, including NGS, and investigate the association with clinical characteristics in Korean PPGL patients.
Methods
We reviewed the medical records of PPGL patients who visited Severance hospital from 2006 to 2019. We documented the clinical phenotype of those who underwent targeted NGS or had known germline mutations of related genes.
Results
Among 57 PPGL patients, we found 28 pathogenic germline mutations of susceptibility genes. Before the targeted NGS was implemented, only obvious syndromic feature lead to the Sanger sequencing for the specific genes. Therefore, for the exact prevalence, only patients after the year 2017, when targeted NGS was added, were included (n=43). The positive germline mutations were found in 14 patients; thus, the incidence rate is 32.6%. Patients with germline mutations had a higher likelihood of family history. There were significant differences in the type of PPGLs, percentage of family history, metastasis rate, presence of other tumors, and biochemical profile among three molecular clusters: pseudohypoxic tricarboxylic acid cycle-related, pseudohypoxic von Hippel-Lindau (VHL)/endothelial PAS domain-containing protein 1-related, and kinase-signaling group. Germline mutations were identified in seven PPGL-related genes (SDHB, RET, VHL, NF1, MAX, SDHA, and SDHD).
Conclusion
We report the expected prevalence of germline mutations in Korean PPGL patients. NGS is a useful and accessible tool for genetic analysis in patients with PPGLs, and further research on molecular classification is needed for precise management.

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Merve Korkmaz Yilmaz, Ozlem Kandemir Alibakan, Aydeniz Aydin Gumus, Alper Gezdirici, Huseyin Karatay, Serkan Sari, Tugba Matlim Ozel, Mutlu Niyazoglu, Esra Hatipoglu
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R. A. Atanesyan, L. Y. Klimov, T. M. Vdovina, G. A. Saneeva, E. I. Andreeva, I. K. Gasparian
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Susan Richter, Nicole Bechmann
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Main mechanisms and clinical implications of alterations in energy expenditure state among patients with pheochromocytoma and paraganglioma: A review
Yuqi Yang, Tong Zhou, Xue Zhao, Yunjia Cai, Yao Xu, Xiaokun Gang, Guixia Wang
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Validation and Evaluation of 5 Scoring Systems for Predicting Metastatic Risk in Pheochromocytoma and Paraganglioma
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Differences between Patients with Sporadic and Familial Pheochromocytoma—Is It Possible to Avoid Genetic Testing in Certain Patients?
María Consuelo Muñoz, Beatriz Febrero, Miriam Abellán, Antonio Miguel Hernández, José Manuel Rodríguez
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Germline Mutations and Phenotypic Associations in Korean Patients With Pheochromocytoma and Paraganglioma: A Multicenter Study and Literature Review
Kwan Hoon Jo, Jaewoong Lee, Jaeeun Yoo, Hoon Seok Kim, Eun Sook Kim, Je Ho Han, Yi Sun Jang, Jae-Seung Yun, Jang Won Son, Soon Jib Yoo, Seung Hwan Lee, Dong Jun Lim, Hyuk-Sang Kwon, Seungok Lee, Sungdae Moon, Myungshin Kim
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Genotype and clinical phenotype characteristics of MAX germline mutation–associated pheochromocytoma/paraganglioma syndrome
Bijun Lian, Jun Lu, Xudong Fang, Yiming Zhang, Wei Wang, Yi He, Hongyuan Yu, Feiping Li, Junwei Wang, Weiying Chen, Xiaoping Qi
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Novel and recurrent genetic variants of VHL, SDHB, and RET genes in Chinese pheochromocytoma and paraganglioma patients
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Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force
Eu Jeong Ku, Kyoung Jin Kim, Jung Hee Kim, Mi Kyung Kim, Chang Ho Ahn, Kyung Ae Lee, Seung Hun Lee, You-Bin Lee, Kyeong Hye Park, Yun Mi Choi, Namki Hong, A Ram Hong, Sang-Wook Kang, Byung Kwan Park, Moon-Woo Seong, Myungshin Kim, Kyeong Cheon Jung, Chan
Endocrinology and Metabolism.2021; 36(2): 322. CrossRef
Prevalence of Germline Variants in a Large Cohort of Japanese Patients with Pheochromocytoma and/or Paraganglioma
Masato Yonamine, Koichiro Wasano, Yuichi Aita, Takehito Sugasawa, Katsutoshi Takahashi, Yasushi Kawakami, Hitoshi Shimano, Hiroyuki Nishiyama, Hisato Hara, Mitsuhide Naruse, Takahiro Okamoto, Tadashi Matsuda, Shinji Kosugi, Kazuhiko Horiguchi, Akiyo Tanab
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Recurrent Germline Mutations of CHEK2 as a New Susceptibility Gene in Patients with Pheochromocytomas and Paragangliomas
Yinjie Gao, Chao Ling, Xiaosen Ma, Huiping Wang, Yunying Cui, Min Nie, Anli Tong, Dario De Biase
International Journal of Endocrinology.2021; 2021: 1. CrossRef

Clinical Study
Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service: Jung Hee Kim, Hyemi Moon, Junghyun Noh, Juneyoung Lee, Sin Gon Kim; Endocrinol Metab. 2020;35(1):157-164. Published online March 19, 2020; DOI: https://doi.org/10.3803/EnM.2020.35.1.157

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Background

Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors originating from chromaffin cells. PPGLs are associated with a high mortality rate and several complications. To date, no epidemiological studies have been conducted on PPGLs in Asia. This study aimed to investigate the epidemiology and prognosis of PPGLs in Korea using nationwide data.

Methods

Using the National Health Insurance Service Database, subjects with a principal diagnosis of PPGLs on two or more occasions between 2003 and 2014 who satisfied the operational definition of PPGLs were included. Incidence, prevalence, complications, metastasis, and mortality were investigated.

Results

In total, 1048 subjects with a mean age of 47.6±16.1 years were included. There was no sex preponderance. The overall prevalence of PPGLs was 2.13 per 100,000 persons, and the overall age-standardized incidence rate was 0.18 per 100,000 person-years. Malignant PPGLs accounted for 17.7% (185 of 1,048) of cases, and 94 subjects exhibited metastasis at the time of diagnosis. Among initially non-metastatic PPGLs, 9.5% (nine of 954) eventually metastasized after a mean duration of 78.1±41.4 months. The 5-year survival rates for non-metastatic and metastatic PPGLs at diagnosis were 97% and 84%, respectively. Multivariable Cox regression models adjusted for covariates showed that metastatic PPGLs were associated with a 2.40-fold higher risk of mortality than non-metastatic PPGLs (95% confidence interval, 1.38 to 4.17; P=0.002).

Conclusion

PPGLs are rare in Korea, and the prognosis of these endocrine tumors varies depending on whether they are benign or malignant. This epidemiological study paves the way for further research on PPGLs.

Citations

Citations to this article as recorded by

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Case Reports

A Case of Calcitonin Secreting Pheochromocytoma.: Joo Won Byun, Young Goo Shin, Choon Hee Chung, Young Jun Won, Yoon Jong Choi, Eui Ryun Park, Mi Duck Lee, Chang Ho Song, Mi Youn Cho, Sung Jun Kang; J Korean Endocr Soc. 1996;11(3):343-347. Published online November 7, 2019

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Abstract PDF: Pheochromocytoma is a catecholamine producing turnor and raise with less than 0.1% of hypertensive patients. It is developed, most commonly, in sporadic pheochromocytoma or multiple endocrine neoplasia type 2. Therefore, when hypercalcitoninemia is found in a patient with pheochromocytoma, the possibility of multiple endocrine neoplasia type 2 or the ectopic secretion of calcitonin must be considered. Recently we experienced a 45 year old male patient with sporadic pheochrornocytoma. He also had hypercalcitoninemia and normocalcemia. After the removal of pheochromocytoma, serum calcitnnin level returned to normal. Secretion of calcitonin was confirmed by immunohisto- chemical stain.

Nerepinephrine Secreting Pheochromocytoma in the Organ of Zuckerkandl With Epinephrine Granules in Cytoplasm.: Eui Suk Kim, Gang Ryong Lee, Won Seok Lee, Jeong Aa Lee, Jie Jeong Jang, In Cheol Sunwoo, Dae Ha Kim, Gwang Seob Lee; J Korean Endocr Soc. 1996;11(2):233-239. Published online November 7, 2019

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Abstract PDF: Extraadrenal pheochromocytomas developed from the paraganglia other than adrenal medulla are uncommon tumors. Such lesions have been reported that as much as 10% of retroperitoneal paragliomas arise outside the adrenal gland. But their incidence and prognosis are changing partly as a result of progression in developmental mechanism, diagnostic tools and concepts including ultrastructure, immunohistochemistry. The ultrastructure of chromaffine neoplasm from adrenal and extraadrenal tissues has been studied with chrarnaffine granule specificity and type of catecholamine content. With rare exceptions, extraadrenal chrornaffine tumor mainly has high concentration of norepinephrine which is suggested as dict relationship to numoruos electrone dense granular bodies in the cell cytoplasrn. High or intermediate level of epinephrine in tissue is associated with cytoplasmic granular bodies of much less density, Recently we experienced the exception, a case of norepinephrine secreting pheochrom-ocytoma in the organ of Zuckerkandl with epinephrine granules in cytoplasm and this discrepancy probably due to the plasticity - the differences in developmental process and local factors between adrenal medulla and extraadrenal tissues.

A Case of Multiple Extraadrenal Pheochromocytoma Iocalized by 131 I-MIBG Scan.: Sung Hee Ihm, Jae Myung Yoo, Moon Gi Choi, Hyung Joon Yoo, Seok Boo Yoon, Jin Bong Kim, Doo Man Kim, Jin Seon Cho, Sang Kon Lee, Sung Woo Park; J Korean Endocr Soc. 1995;10(4):439-444. Published online November 7, 2019

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Abstract PDF: Peroperative localization of pheochromocytoma is the very crucial step for the successful removal of tumors. Computed tomography(CT) and magnetic resonance(MR) imaging have been commonly used for tumor localization, but in some cases of pheochromocytoma, such as extraadrenal location or distant metastasis of malignant tumor, their localization is somewhat difficult. Recently ^131 I-metaiodobenzylguanidine(MIBG) scintigraphy has been developed and increasingly used for the localization of pheochromocytoma and reported to be more sensitive and specific than CT or MR imaging in the cases of extraadrenal tumor location.We report a case of multiple extraadrenal pheochromocytoma in which ^131 I-MIBG scintigraphy clearly localized two intraabdominal and one bladder tumors, after failure of localization with conventional CT and MR imaging.

Original Articles

A Clinical Observation of Endocrine Adrenal Tumors.: Hye Young Park, Tae Suk Kim, Soo Jin Lee, Dong Gu Choi, Moon Ho Kang; J Korean Endocr Soc. 1994;9(3):228-238. Published online November 6, 2019

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Abstract PDF: Endocrine adrenal tumors are uncommon but clinically significant because they can be managed successfully by surgical therapy. During pregnancy, adrenal tumors can be a cause of death for both mother and baby, unless they diagnoses antenatally and managed properly. To investigate clinical characteristics of endocrine adrenal tumors, we analyzed 26 cases of adrenal tumor diagnosed and managed in Chung Ang Gil Hospital including cases associated with pregnancy and also a rare case of paraganglioma in liver. 1) There were 7 cases of Cushing's syndrome among which two cases were due to Cushing's disease and five cases were due to adrenal adenoma. Six cases were managed successfully by appropriate surgery. Another one case was associated with pregnancy but the patient expired due to sudden development of pulmonary edema and hypotension during preoperative preparation.2) Among 9 cases of primary aldosteronism, 8 cases were aldosterone-producing adenoma(APA) and 1 case was idiopathic hyperaldosteronism(IHA). In differential diagnosis between APA and IHA, diagnostic accuracy of postural study and adrenal CT was 86%, 78% respectively. In each one case of APA and IHA, adrenal venous aldosterone sampling was used for the differential diagnosis. Six cases of APA were managed by appropriate surgery. One patient with APA who refuses surgery and one patient with IHA were placed on spironolactone with good response.3) Among 10 cases of pheochromocytoma, seven cases were from adrenal gland and three cases were paragangliomas including one case originating from liver and two cases from abdominal ganglia. One patient with pheochromocytoma was diagnosed in 1st trimester of pregnancy and she was successfully treated by surgery.We observed 26 cases of endocrine adrenal tumors which were successfully managed except one case. We think that it is important to pay attention to adrenal tumor for early diagnosis particulary when associated with pregnancy. Paraganglioma in liver, although very rare, should be included in the differential diagnosis of space occupying lesion in liver.

Immunohistochemical Study of c - Myc, c - Fos and c - Jun Oncoprotein Expression in the Human Pheochromocytoma.: Jae Wha Jo, Eun Jig Lee, Moon Suk Nam, Kyung Rae Kim, Su Youn Nam, Young Duk Song, Sung Kil Lim, Hyun Chul Lee, Kap Bum Huh, Yong Hye Lee, Tae Seung Kim, Kwan Woo Lee; J Korean Endocr Soc. 1995;10(1):26-34. Published online November 6, 2019

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Abstract PDF: A large number of studies for genes involved in oncogenesis have been done during last decade. Over 20 oncogenes have been isolated characterized, and the oncogene expressions in human tumors have been examined. The proto-oncogenes of c-Myc, c-Fos and c-Jun, which modulate the transcription factors, have overexpressed in a variety of human cancers. Immunohistochemical method was used in this study to examine c-Myc, c-Fos and c-Jun oncoprotein expression in 31 patients with human pheochromocytoma 28(90.0%) were benign and 3(10.0%) malignant. C-Myc oncoprotein immunoreactivity was found in 24 cases(77.4%), c-Fos in 29(93.5%), and c-Jun in 25(80.6%). Twenty-one(67.7%) showed positive immunoreactivity for all these oncoproteins, six(19.4%) for 2 oncoproteins, 3 for one oncoprotein. Only 1 case showed negative immunoreactivity for all 3 oncoproteins. The oncoprotein immunoreactivity did not correlate with the amount of 24 hour urinary catecholamine excretion. Although the number of malignant pheochromocytomsa was not so many, most of them showed that the immunoreactivity for oncoprotein was more than 30 percent of tumor cells.The expression of c-Myc, c-Fos and c-Jun oncoprotein were frequently found in human pheochromocytoma. These results suggest that the oncoprotein expression may play an important role in tumorogenesis and proliferation of human pheochromocytoma.

Case Report

A Case of Pheochromocytoma Diagnosed as Preeclampsia during Prenancy.: Bong Yun Cha, Ho Young Son, Soon Jip Yoo, Ki Hyun Baik, Myung Hoon Kim, Moo Il Kang, E Jin Choi, Kwang Woo Lee, Sung Koo Kang, Uk Kim, Jong Man Won, Jin Young Yoo; J Korean Endocr Soc. 1994;10(3):295-299. Published online November 6, 2019

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Abstract PDF: A case of a pregnant woman with an undiagnosed pheochromocytoma is presented. Gestational hypertension was developed at 33 weeks' gestation. Also she experienced intermittent chest discomfort, headache and irritability during those period. Normal vaginal delivery was successfully performed with close observation. Even though experiencing same symptoms for two years after delivery, she had been treated as neurosis. Uncatched pheochromocytoma was detected and the tumor was resected successfully. It seems to be that many cases of pheochromocytoma with pregnancy still go unrecognized because of close similarity between pheochromocytoma and toxemia. High index of suspicion should be maintained to achieve early diagnosis because antenatal diagnosis can lower the mortality in both mother and fetus.

Namgok Lecture 2017

Adrenal gland
Bone Health in Adrenal Disorders: Beom-Jun Kim, Seung Hun Lee, Jung-Min Koh; Endocrinol Metab. 2018;33(1):1-8. Published online March 21, 2018; DOI: https://doi.org/10.3803/EnM.2018.33.1.1

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Abstract PDF PubReader ePub

Secondary osteoporosis resulting from specific clinical disorders may be potentially reversible, and thus continuous efforts to find and adequately treat the secondary causes of skeletal fragility are critical to ameliorate fracture risk and to avoid unnecessary treatment with anti-osteoporotic drugs. Among the hyperfunctional adrenal masses, Cushing's syndrome, pheochromocytoma, and primary aldosteronism are receiving particularly great attention due to their high morbidity and mortality mainly by increasing cardiovascular risk. Interestingly, there is accumulating experimental and clinical evidence that adrenal hormones may have direct detrimental effects on bone metabolism as well. Thus, the present review discusses the possibility of adrenal disorders, especially focusing on pheochromocytoma and primary aldosteronism, as secondary causes of osteoporosis.

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Review Article

Update on Modern Management of Pheochromocytoma and Paraganglioma: Jacques W. M. Lenders, Graeme Eisenhofer; Endocrinol Metab. 2017;32(2):152-161. Published online June 23, 2017; DOI: https://doi.org/10.3803/EnM.2017.32.2.152

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Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT) or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as ⁶⁸Ga-DOTATATE (⁶⁸Ga-labeled DOTA(0)-Tyr(3)-octreotide) will probably replace ¹²³I-MIBG (iodine-123-metaiodobenzylguanidine) in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background) should be followed up lifelong.

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Original Article

Clinical Study
A 5-Year Prospective Follow-Up Study of Lipid-Rich Adrenal Incidentalomas: No Tumor Growth or Development of Hormonal Hypersecretion: Camilla Schalin-Jäntti, Merja Raade, Esa Hämäläinen, Timo Sane; Endocrinol Metab. 2015;30(4):481-487. Published online December 31, 2015; DOI: https://doi.org/10.3803/EnM.2015.30.4.481

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Background

Current guidelines for follow-up of adrenal incidentalomas are extensive and hampered by lack of follow-up studies. We tested the hypothesis that small lipid-rich adrenal incidentalomas, initially characterized by tumor size <40 mm and <10 Hounsfield units (HUs) on unenhanced computed tomography (CT) may not demonstrate excessive growth/hormonal hypersecretion on follow-up.

Methods

Sixty-nine incidentalomas in 56 patients were restudied with unenhanced CT and screening for hypercortisolism (dexamethasone suppression test [DST], plasma adrenocorticotropic hormone) and pheochromocytoma (24-hour urinary metanephrines and normetanephrines) 5 years later. Primary hyperaldosteronism was excluded at base-line.

Results

Tumor (n=69) size was similar before and after 5 years follow-up (19±6 mm vs. 20±7 mm). Mean tumor growth was 1±2 mm. Largest increase in tumor size was 8 mm, this tumor was surgically removed and histopathology confirmed cortical adenoma. DST was normal in 54 patients and two patients (3.6%) were still characterized by subclinical hypercortisolism. Initial tumor size was >20 mm for the patient with largest tumor growth and those with subclinical hypercortisolism. All patients had normal 24-hour urinary metanephrines and normetanephrines. Low attenuation (<10 HU) was demonstrated in 97% of 67 masses re-evaluated with unenhanced CT.

Conclusion

None of the patients developed clinically relevant tumor growth or new subclinical hypercortisolism. Biochemical screening for pheochromocytoma in incidentalomas demonstrating <10 HU on unenhanced CT is not needed. For such incidentalomas <40 mm, it seems sufficient to perform control CT and screen for hypercortisolism after 5 years.

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Case Reports

Adrenal gland
Incidentally Detected Inoperable Malignant Pheochromocytoma with Hepatic Metastasis Treated by Transcatheter Arterial Chemoembolization: Joong Keun Kim, Bo Hyun Kim, Sung Min Baek, Dong Hun Shin, Won Jin Kim, Yun Kyoung Jeon, Sang Soo Kim, In Joo Kim; Endocrinol Metab. 2014;29(4):584-589. Published online December 29, 2014; DOI: https://doi.org/10.3803/EnM.2014.29.4.584

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Malignant pheochromocytoma (PCC) is a rare condition. Although the liver is the second most frequent site of metastasis in malignant PCC, no definite treatments have been established. Herein, we report a case of liver metastasis of PCC that was successfully treated by transcatheter arterial chemoembolization (TACE). A 69-year-old man was admitted to the Department of Gastroenterology for evaluation of an incidental hepatic mass in August 2013. He had undergone right adrenalectomy in May 2005 and PCC had been confirmed on the basis of histopathological findings. Liver biopsy was performed, and metastatic PCC was diagnosed. The lesion appeared inoperable because of invasion of the portal vein and metastases in the lymph nodes along the hepatoduodenal ligament. Thus, TACE was performed instead. After TACE, symptoms including dizziness and cold sweating improved, and the patient's serum catecholamine levels decreased. On the basis of this case, we believe that TACE may be a useful treatment for liver metastasis in malignant PCC.

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Adrenal gland
Pheochromocytoma with Markedly Abnormal Liver Function Tests and Severe Leukocytosis: Chai Ryoung Eun, Jae Hee Ahn, Ji A Seo, Nan Hee Kim; Endocrinol Metab. 2014;29(1):83-90. Published online March 14, 2014; DOI: https://doi.org/10.3803/EnM.2014.29.1.83

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Pheochromocytoma is a rare neuroendocrine tumor arising from the medulla of the adrenal glands, which causes an overproduction of catecholamines. The common symptoms are headache, palpitations, and sweating; however, various other clinical manifestations might also be present. Accurate diagnosis of pheochromocytoma is important because surgical treatment is usually successful, and associated clinical problems are reversible if treated early. A 49-year-old man with a history of uncontrolled hypertension and diabetes mellitus presented with chest pain, fever, and sweating. His liver function tests and white blood cell counts were markedly increased and his echocardiography results suggested stress-induced cardiomyopathy. His abdominal computed tomography showed a 5×5-cm-sized tumor in the left adrenal gland, and laboratory tests confirmed catecholamine overproduction. After surgical resection of the left adrenal gland, his liver function tests and white blood cell counts normalized, and echocardiography showed normal cardiac function. Moreover, his previous antihypertensive regimen was deescalated, and his previously uncontrolled blood glucose levels normalized without medication.

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Original Article

Adrenal gland
Clinical Characteristics for 348 Patients with Adrenal Incidentaloma: Jongho Kim, Kwi Hyun Bae, Yeon Kyung Choi, Ji Yun Jeong, Keun Gyu Park, Jung Guk Kim, In Kyu Lee; Endocrinol Metab. 2013;28(1):20-25. Published online March 25, 2013; DOI: https://doi.org/10.3803/EnM.2013.28.1.20

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Background

Adrenal incidentaloma is an adrenal neoplasm frequently encountered in clinical practice for which detection rates have recently increased. We describe here the clinical characteristics of adrenal incidentalomas.

Methods

A retrospective study was performed examining the age, sex, location, size, function, and the histological findings for 348 patients with an adrenal mass discovered incidentally on computed tomography (CT) undertaken for health examination or nonadrenal disease from August 2005 to May 2012.

Results

Patients consisted of 156 males (44.8%) and 192 females (55.2%), aged between 20 and 86. Adrenal masses were most commonly found in patients in their sixth decade (32.5%). Regarding the location of the masses, 62.0% were found in the left adrenal gland, 30.2% were found in the right, and 7.8% were found bilaterally. Of all of the masses analyzed, 87.1% were 1 to 4 cm in size, and an adenoma-like appearance was the most common finding (75.3%) seen on CT scans. Hormonal analysis showed that 82.2% of the masses were nonfunctioning, 6.0% were diagnosed as subclinical Cushing's syndrome, 4.6% were aldosterone-producing adenomas, and 7.2% were pheochromocytomas. Adrenalectomy was performed in a total of 69 patients having adenoma (50.7%), pheochromocytoma (24.6%), and carcinoma (4.3%).

Conclusion

The characteristics of benign, malignant, nonfunctional, and functional adrenal masses that were incidentally found at our hospital were similar to those presented in other studies.

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Yonsei Medical Journal.2018; 59(4): 501. CrossRef
Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature
Jenan N Gabi, Maali M Milhem, Yara E Tovar, Emhemmid S Karem, Alaa Y Gabi, Rodhan A Khthir
Journal of the Endocrine Society.2018; 2(7): 621. CrossRef
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Case Reports

A Case of Adrenal Cystic Pheochromocytoma with Contralateral Adrenocortical Adenoma Causing Subclinical Cushing's Syndrome.: Chang Jun Park, Joo Wan Seo, Hyeog Gyu Seoung, Jung Hee Koh, Yong Jae Lee, Bo Hyun Kim, In Ju Kim; Endocrinol Metab. 2012;27(4):323-328. Published online December 20, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.4.323

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Abstract PDF

Bilateral adrenal neoplasms are associated with metastatic cancer, pheochromocytoma and lymphoma. The coexistence of a unilateral functioning adrenocortical adenoma with contralateral pheochromocytoma is extremely rare. A 52-year-old woman complained of fatigue, headache, palpitation, and progressive weight gain. Hormonal assessment demonstrated high 24 hours urine epinephrine, norepinephrine, and free cortisol. A dexamethasone suppression test (overnight 1 mg, low dose 2 mg) showed insuppressible cortisol. Computerized tomographic scanning revealed a bilateral adrenal tumor. To preserve adrenal function, right adrenalectomy along with left adrenal tumorectomy was performed. Histological finding of the right adrenal tumor was pheochromocytoma and the left adrenal tumor was adrenocortical adenoma. This patient was the first case of a functional adrenocortical adenoma with contralateral cystic pheochromocytoma in Korea. We report the case with a review of the literature.

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A Case of Bilateral ACTH-independent Adrenal Adenomas with Cushing's Syndrome Treated by Ipsilateral Total and Contralateral Partial Laparoscopic Adrenalectomy
Seung Ah Park, Dong min Jung, Soon young Kim, Nan Young Choi, Tae-jun Kim, Yong kyun Kim, Seong kyun Na, Chul Sik Kim, Seong Jin Lee, Sung-Hee Ihm, Jun Goo Kang
The Korean Journal of Obesity.2013; 22(4): 254. CrossRef

A Case of Dopamine-Secreting Pheochromocytoma.: Jung Kyu Park, Hoon Kyu Oh, Moo Hyun Shon, Hyun Hee Kim, Eon Ju Jeon, Eui Dal Jung; Endocrinol Metab. 2012;27(2):159-162. Published online June 20, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.2.159

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Abstract PDF

A pheochromocytoma is a neuroectodermal tumor that originates from the chromaffin cells of the sympathetic system. It has typical symptoms or signs, such as periodic attacks of paroxysmal hypertension, palpitation, headache, and sweating, related to an increased catecholamine secretion. Types of catecholamine secreted from tumors are usually norepinephrine and epinerphrine. There are a few reports of dopamine-secreting pheochromocytoma with absence of other catecholamines secretion. Here, we report the case of a 59-year-old man with dopamine-secreting pheochromocytoma, with no typical symptoms or signs.

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Metanephrine negative pheochromocytoma: a rare case report of dopamine-secreting tumor in an adolescent neurofibromatosis type 1 patient
Mi-Seon Lee, Rosie Lee, Sook-Hyun Park, Soon Hak Kwon, Jin-Young Park, Sang-Woo Lee, So-Mi Lee, Jung-Eun Moon
Annals of Pediatric Endocrinology & Metabolism.2023; 28(4): 302. CrossRef

Original Article

Characterization of Incidentally Detected Adrenal Pheochromocytoma.: Ye An Kim, Yul Hwangbo, Min Joo Kim, Hyung Jin Choi, Je Hyun Seo, Yenna Lee, Soo Heun Kwak, Eu Jeong Ku, Tae Jung Oh, Eun Roh, Jae Hyun Bae, Jung Hee Kim, Kyoung Soo Park, Seong Yeon Kim; Endocrinol Metab. 2012;27(2):132-137. Published online June 20, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.2.132

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BACKGROUND
In approach to an adrenal incidentaloma, early exclusion of pheochromocytoma is clinically important, due to the risk of catecholamine crisis. The aims of this study are to investigate the characteristics of incidentally detected pheochromocytomas, compared with that of the other adrenal incidentalomas, and to compare these characteristics with those of symptomatic pheochromocytomas. METHODS: In this retrospective study, we reviewed the medical records of 198 patients with adrenal incidentaloma from 2001 to 2010. We analyzed the clinical, laboratory and radiological data of pheochromocytomas, in comparison with those of the other adrenal incidentalomas. We also compared the characteristics of these incidentally detected pheochromocytomas with the medical records of 28 pathologically proven pheochromocytomas, diagnosed based on typical symptoms. RESULTS: Among the 198 patients with adrenal incidentaloma, nineteen patients were diagnosed with pheochromocytoma. Pheochromocytomas showed larger size and higher Hounsfield unit at precontrast computed tomography (CT) than did non-pheochromocytomas. All pheochromocytomas were larger than 2.0 cm, and the Hounsfield units were 19 or higher in precontrast CT. When both criteria of size > 2.0 cm and Hounsfield unit > 19 were met, the sensitivity and specificity for the diagnosis of pheochromocytoma were 100% and 79.3%, respectively. Compared with patients with pheochromocytoma, diagnosed based on typical symptoms, patients with incidentally detected pheochromocytoma were older, presented less often with hypertension, and showed lower levels of 24-hour urine metanephrine. CONCLUSION: Adrenal incidentaloma with < 2.0 cm in size or < or = 19 Hounsfield units in precontrast CT imaging was less likely to be a pheochromocytoma. Patients with incidentally discovered pheochromocytoma showed lower catecholamine metabolites, compared with those patients with symptomatic pheochromocytoma.

Citations

Citations to this article as recorded by

Guidelines for the Management of Adrenal Incidentaloma: the Korean Endocrine Society, Committee of Clinical Practice Guidelines
Jung-Min Lee, Mee Kyoung Kim, Seung-Hyun Ko, Jung-Min Koh, Bo-Yeon Kim, Sang-Wan Kim, Soo-Kyung Kim, Hae-Jin Kim, Ohk-Hyun Ryu, Juri Park, Jung-Soo Lim, Seong Yeon Kim, Young Kee Shong, Soon Jib Yoo
The Korean Journal of Medicine.2017; 92(1): 4. CrossRef
Clinical Guidelines for the Management of Adrenal Incidentaloma
Jung-Min Lee, Mee Kyoung Kim, Seung-Hyun Ko, Jung-Min Koh, Bo-Yeon Kim, Sang Wan Kim, Soo-Kyung Kim, Hae Jin Kim, Ohk-Hyun Ryu, Juri Park, Jung Soo Lim, Seong Yeon Kim, Young Kee Shong, Soon Jib Yoo
Endocrinology and Metabolism.2017; 32(2): 200. CrossRef
Characterization of Incidentally Detected Adrenal Pheochromocytoma
Soon Jib Yoo, Woohyeon Kim
Endocrinology and Metabolism.2012; 27(2): 116. CrossRef

Case Reports

A Case of Malignant Pheochromocytoma Presenting as Inverted Takotsubo-Like Cardiomyopathy.: Jung Eun Jang, Hyuk Hee Kwon, Min Jung Lee, Chang Hee Jung, Sung Jin Bae, Hong Kyu Kim, Woo Je Lee; Endocrinol Metab. 2012;27(1):98-104. Published online March 1, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.1.98

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Abstract PDF: Takotsubo cardiomyopathy or stress induced cardiomyopathy is characterized by acute transient left ventricular apical ballooning without significant coronary artery disease. The pathophysiology of Takotsubo cardiomyopathy remains unclear, but it has been suggested that the stress related neurohumoral factors, especially catecholamines, play an important role. Recently, several reports have described an inverted Takotsubo cardiomyopathy, which is characterized by the dysfunction of the basal and mid-ventricular segments sparing the apex of the heart. In this report, we present a case of a 50-year-old female with a transient left ventricular dysfunction in an inverted Takotsubo pattern, that later was diagnosed as a malignant pheochromocytoma.

Laparoscopically Resected Composite Pheochromocytoma-Ganglioneuroma.: Na Rae Kim, Taeeun Kim, Jeong Nam Lee, Young Sil Eom, Dong Hae Chung, Sanghui Park, Hyun Yee Cho; Endocrinol Metab. 2011;26(4):340-344. Published online December 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.4.340

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Abstract PDF

Composite pheochromocytoma-ganglioneuroma is one of the mixed neuroendocrine-neural tumors composed of pheochromocytoma and other neural crest derivatives. To date, less than 50 cases of composite pheochromocytoma have been reported, and about 70% of the accompanying tumors were ganglioneuromas. Here, we describe six cases of composite pheochromocytoma-ganglioneuromas in five men and one woman, aged 33 to 64. The size of the tumors ranged from 3.0 to 11.0 cm, and four out of the six presented with intermittent onset of hypertension, palpitation, or dizziness. Microscopically, each tumor was composed of large pleomorphic shaped chromaffin cells arranged in the Zellballen patterns characteristic of pheochromocytoma, and they were mixed with clusters of mature ganglion cells and bundles of spindle-shaped Schwann cells characteristic of ganglioneuroma of variable proportions. All were successfully treated laparoscopically, and none were associated with multiple endocrine neoplasm syndrome or neurofibromatosis. Preoperative diagnosis of a composite pheochromocytoma-ganglioneuroma is impossible because of the low incidence rate, and the radiological findings and symptoms are similar to those of typical pheochromocytomas. Although the significance of microscopic detection of the nonpheochromocytoma component from pheochromocytoma has not yet been clarified, microscopic identification of the composite pheochromocytoma-ganglioneuroma is important because cumulative cases are used in an effort to predict the behavior of this composite tumor.

Citations

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A rare case of a composite phaeochromocytoma-ganglioneuroma in Australia
Peter Pham, Benedict Kakala, Amanda Love, Archana M Sudarsan, Clement Wong
Journal of Surgical Case Reports.2025;[Epub] CrossRef
Imaging of rare medullary adrenal tumours in adults
C.A. Maciel, Y.Z. Tang, G. Coniglio, A. Sahdev
Clinical Radiology.2016; 71(5): 484. CrossRef

Case Reports and Estimated Prevalence of Adrenal Pheochromocytoma in Patients with Neurofibromatosis Type I in Korea.: Ju Yang Jung, Seun Joo Ahn, Hyung Young Yoon, Jung Min Ko, Yoon Sok Chung; Endocrinol Metab. 2011;26(3):258-262. Published online September 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.3.258

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Abstract PDF: We report three patients with adrenal pheochromocytoma who were associated with type I neurofibromatosis. Two of them were asymptomatic, but one case involved hypertension. We reviewed medical records and adrenal imaging, and estimated the prevalence of adrenal pheochromocytoma among neurofibromatosis type I patients in one university hospital in Korea. A total of 658 patients were coded for neurofibromatosis type I (Q85.0 with International Classification of Diseases 10 version) with clinical impression, but only 371 were confirmed via 1997 National Institute of Health criteria. Adrenal images were generated in 203 patients, and 3 of them were diagnosed with pheochromocytoma. According to the results of this study, the estimated prevalence of adrenal pheochromocytoma in type I neurofibromatosis was 0.30-1.48%.

Mutational Analysis of the NF1 Gene in Two Families with Neurofibromatosis 1 Accompanied by Pheochromocytoma.: Hyon Seung Yi, Sei Hyun Kim, Jihoon Kim, Eun Jin Bae, Suntaek Hong, Ie Byung Park, Yu Jin Kim, Sihoon Lee; Endocrinol Metab. 2011;26(2):177-184. Published online June 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.2.177

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Neurofibromatosis type 1 (NF1) is one of the most common autosomal dominant inherited disorders affecting the nervous system. NF1 is associated with mutations in the NF1 gene, which is located on chromosome sub-band 17q11.2 and contains 57 exons spanning approximately 300 kb of genomic DNA. NF1 is caused by a loss of function mutation of the NF1 gene, a tumor suppressor gene, which encodes for neurofibromin, a GTPase-activating protein (GAP) involved in the negative regulation of Ras activity. The GAP-related domain, which is encoded for by exons 20-27a, is one of the most important functional domains in neurofibromin. The cysteine-serine-rich domain has been recognized as an important functional domain in NF1-related pheochromocytomas. As the result of many genetic analyses of NF1-related pheochromocytomas, pheochromocytoma has generally been recognized as a true component of NF1. We recently experienced two families with NF1 accompanied by pheochromocytoma. The proband of family 1 is a 31-year-old female diagnosed with NF1 and pheochromocytoma. Gene analysis of the proband and her sister showed that the mutation of the NF1 gene (c.7907+1G>A) led to the skipping of exon 53 during NF1 mRNA splicing. The proband of family 2 is a 48-year-old male who was diagnosed with the same condition. Gene analysis demonstrated the mutation of the NF1 gene (c.5206-8C>G) with missplicing of exon 37. These novel germline mutations did not fall into the GAP-related nor the cysteine-serine-rich domains, but into the C-terminal area of the NF1 gene. This suggests that the correlation between the genotype and phenotype of NF1-related pheochromocytoma is somewhat difficult to characterize. Further studies will be necessary to confirm the function of the C-terminal area of the NF1 gene and its contribution to the development of NF1 and pheochromocytoma.

Citations

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Mutation Spectrum of NF1 and Clinical Characteristics in 78 Korean Patients With Neurofibromatosis Type 1
Jung Min Ko, Young Bae Sohn, Seon Yong Jeong, Hyon-Ju Kim, Ludwine M. Messiaen
Pediatric Neurology.2013; 48(6): 447. CrossRef
Oncologic manifestations of neurofibromatosis type 1 in Korea
Eui Tae Kim, Hwan Namgung, Hyun Deok Shin, Soon Il Lee, Jee Eun Kwon, Myung Chul Chang, Dong Guk Park
Journal of the Korean Surgical Society.2012; 82(4): 205. CrossRef

A Case of Pheochromocytoma Presenting as Syncope Due to Orthostatic Hypotension.: Ji Yeun Kim, Sung Woo Kim, Seung Jun Lee, Hyun Sook Kim, Eui Dal Jung, Yun Seop Kum; Endocrinol Metab. 2011;26(2):155-159. Published online June 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.2.155

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Abstract PDF: Patients with pheochromocytoma manifest with headache, perspiration, and palpitation. Although most patients have either sustained or paroxysmal hypertension, some patients present with hypotension. However, severe orthostatic hypotension is relatively rare in patients with pheochromocytoma. We report here on a 72-year-old woman with pheochromocytoma and she presented with recurrent syncope due to hypotension and blood pressure fluctuation. Syncope due to hypotension is unusual in patients with pheochromocytoma and only a few such cases have been reported. The present case serves to illustrate an unexpected presentation of this tumor.

A Case of Pheochromocytoma Crisis with Acute Myocardial Infarction Induced by Glucocorticoids Administration.: Woo Sun Rou, Sang Kyung Jung, Sung Yun Lee, Yun Jeong Lee, Dong Jun Kim, Young Doo Kim, Hyung Yoon Kim, Sunhee Chang, Jung Hyun Noh; Endocrinol Metab. 2010;25(3):240-244. Published online September 1, 2010; DOI: https://doi.org/10.3803/EnM.2010.25.3.240

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Abstract PDF: The most common symptoms of pheochromocytoma are paroxysmal or sustained hypertension, or symptoms of paroxysmal adrenergic stimulation such as palpitation, headache, and diaphoresis. These patients can on rare occasion reveal or be complicated with cardiovascular symptoms such as arrhythmia, cardiomyopathy, acute coronary syndrome and cardiogenic shock. These cardiac manifestations of pheochromocytoma may delay the diagnosis, which can cause a catastrophic outcome. A pheochromocytoma crisis is provoked by surgery, anesthesia, exercise and, several drugs and it is known to be an endocrine emergency with mortality as high as 85%. Many classes of drugs are well known to precipitate adverse reactions, but the presentation of pheochromocytoma after the administration of steroid has rarely been reported. We report here on a case of pheochromocytoma crisis with acute myocardial infarction after the patient took prednisolone. Furthermore, we discuss the mechanism of glucocorticoid induced crisis and myocardial infarction in pheochromocytoma patients.

A Case of Pheochromocytoma Presented with Cardiogenic Shock and Followed by Spontaneous Remission.: Jae Wook Kwak, Jong Sang Kim, Yun Jong Seo, Jae Hui Jang, Sun Hui Park, Hyo Heon Kim; Endocrinol Metab. 2010;25(3):236-239. Published online September 1, 2010; DOI: https://doi.org/10.3803/EnM.2010.25.3.236

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Abstract PDF

Pheochromocytoma is derived from the chromaffin cells and patients with pheochromocytoma present with several signs and symptoms by producing, storing and secreting catecholamine. Spontaneous rupture or necrosis of pheochromocytoma is extremely rare, but it can be lethal because of the dramatic change in the circulation such as an acute abdominal emergency or shock. Spontaneous remission of the clinical symptoms due to necrosis of the pheochromocytoma is rare. We describe such a case that presented with cardiogenic shock due to extensive necrosis of the pheochromocytoma and this was followed by spontaneous remission of the clinical symptoms without removal of the pheochromocytoma.

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Pheochromocytoma-induced cardiogenic shock successfully treated by extracorporeal circulation
Min Young Lee, Sang Bae Lee, Hyun Seo Cha, Ji Hong You, Eui Young Choi, Jong Suk Park
Yeungnam University Journal of Medicine.2017; 34(2): 285. CrossRef

A Family Presenting with Catastrophic Features due to Pheochromocytoma Associated with Multiple Endocrine Neoplasia 2A.: Yun Jung Lee, Suk Chon, Sang Ho Lee, Tae Won Lee, Chun Gyoo Ihm, Tae June Noh, Seungjoon Oh, Jeong Taek Woo, Sung Woon Kim, Jin Woo Kim, Young Seol Kim; Endocrinol Metab. 2010;25(2):135-141. Published online June 1, 2010; DOI: https://doi.org/10.3803/EnM.2010.25.2.135

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Abstract PDF: Multiple endocrine neoplasia 2A (MEN 2A) is an autosomal dominant disease that consists of medullary thyroid carcinoma (MTC), pheochromocytoma and parathyroid hyperplasia. The activation of germ-line mutations in the RET proto-oncogene are responsible for MEN 2A. We describe here a rare case of MEN 2A in a patient who presented with an acute catecholamine-induced cardiomyopathy with cardiogenic shock and acute renal failure. The patient was diagnosed with pheochromocytoma and MTC associated with MEN 2A, which was confirmed by the detection of a RET proto-oncogene mutation at exon 11 on codon 634 (Cys634Arg). During familial screening, the patient's younger sister was found to have a benign thyroid nodule. Re-evaluation of this thyroid nodule revealed MTC with the same gene mutation. We also provide a review of the relevant literature.

Ectopic ACTH Syndrome with Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A.: Ji Mi Moon, Yoon Jung Kim, Young Jin Seo, Hye Yoon Choi, Joo Hyong Kim, Ju Ri Park, Yun Jeong Lee, Hee Young Kim, Sin Gon Kim, Dong Seop Choi; J Korean Endocr Soc. 2009;24(4):265-271. Published online December 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.4.265

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Abstract PDF

Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN 2A arises due to a germline missense mutation of the RET proto-oncogene. Specific RET mutation analysis has revolutionized the diagnosis and therapy of this disorder, and early thyroidectomy may have lowered the morbidity and mortality associated with these diseases. Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of ACTH outside of the pituitary gland; the most common causes are malignancies, but rarely adrenal pheochromocytoma may be the cause.

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Presentation of multiple endocrine neoplasia type 2A-associated ectopic cushing’s syndrome: case report and a systematic review
Wei Wang, Wei-Ying Chen, Mei-Xian Zhang, Zhen-Yu Chen, Zhi-Lie Cao, Jun-Wei Wang, Wu-Gen Yao, Jian-Qiang Zhao, Fei-Ping Li, Hong-Yuan Yu, Jun Lu, Xiao-Ping Qi
Frontiers in Endocrinology.2025;[Epub] CrossRef
Pheochromocytoma With High Adrenocorticotropic Hormone Production Capacity Without Pigmentation and Cushingoid Symptoms: A Case Report With a Literature Review
Gen Mizutani, Masashi Isshiki, Eisuke Shimizu, Daigo Saito, Akira Shimada
Cureus.2024;[Epub] CrossRef
Ectopic ACTH- and/or CRH-Producing Pheochromocytomas
Patrick F Elliott, Thomas Berhane, Oskar Ragnarsson, Henrik Falhammar
The Journal of Clinical Endocrinology & Metabolism.2021; 106(2): 598. CrossRef
Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature
Jenan N Gabi, Maali M Milhem, Yara E Tovar, Emhemmid S Karem, Alaa Y Gabi, Rodhan A Khthir
Journal of the Endocrine Society.2018; 2(7): 621. CrossRef
Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis
Jae Ho Cho, Da Eun Jeong, Jae Young Lee, Jong Geol Jang, Jun Sung Moon, Mi Jin Kim, Ji Sung Yoon, Kyu Chang Won, Hyoung Woo Lee
Yeungnam University Journal of Medicine.2015; 32(2): 132. CrossRef

A Case of Improved Diabetes Mellitus After Removal of Nonfunctioning Adrenal Incidentaloma Diagnosed as Pheochromocytoma.: Se Kyung Park, Duk Su Kim, Mi Oh Roh, Min Soo Song, Chan Hee Jung, Jung Hwa Jung, Hyeong Kyu Park, Yeo Ju Kim, Ji Oh Mok, Sang Jin Kim, Chul Hee Kim, Dong Won Kim, Dong Won Byun, Kyo Il Suh, Myung Hi Yoo; J Korean Endocr Soc. 2009;24(3):189-194. Published online September 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.3.189

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Abstract PDF: Pheochromocytoma not only decreases insulin secretion but also increases insulin resistance. One third of patients with pheochromocytoma have diabetes mellitus and their clinical prognosis showed improvement after surgery. Until now, those patients whose prognosis for diabetes mellitus improved after the operation of pheochromocytoma had typical characteristics such as hypertension, palpitation, headache and elevated hormones such as Vanillylmandelic acid, metanephrine, epinephrine and norepinephrine. We present the case of a 75-year-old woman with asymptomatic adrenal incidentaloma identified as pheochromocytoma which exhibited normal biochemical test results, and after removal of the abdominal mass, her severe hyperglycemia improved.

Graves' Disease Accompanied by Pheochromocytoma: Report of a Case.: Jin Hwa Kim, Sang Jun Lee, Ji Hye Shin, Mi Ra You, Jae Sik Jung, Sang Yong Kim, Hak Yeon Bae; J Korean Endocr Soc. 2009;24(2):126-131. Published online June 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.2.126

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Abstract PDF

We present here a rare case of Graves' disease accompanied by pheochromocytoma, and the patient showed normal urine and serum levels of catecholamines and their metabolites. A 45-year-old woman was referred to our hospital for the evaluation of a right adrenal incidentaloma that was detected by chest computed tomography. She had been diagnosed with Graves' disease 1 month previously. She had no symptoms of pheochromocytoma such as hypertension or a history of hypertension attack. Two consecutive 24-hour urine samples were sent to the lab for measurement of the catecholamines, and both samples showed normal levels of metanephrine and vanillylmandelic acid (VMA). After right adrenalectomy was performed, the final pathological diagnosis was adrenal pheochromocytoma. This case suggests that the onset of Graves' disease may be associated with excess catecholamine secreted by a pheochromocytoma. In addition, although the conventional method for detecting pheochromocytoma is to identify an increase of the urine catecholamines, physicians should be aware of the possibility of false negativity on this test.

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Functional retroperitoneal paraganglioma invading the inferior vena cava in the elderly, a case report and literature review
Mohamed Aymane Loukili, Imane Assarrar, Nada El Yamani, Anass Haloui, Siham Rouf, Hanane Latrech
International Journal of Surgery Case Reports.2023; 109: 108547. CrossRef

A Case of Pheochromocytoma That Presented as Inverted Takotsubo Cardiomyopathy.: Meyoung Cho, Ik Sang Shin, Ae Ryoung Jin, Jong Bin Park, Hye Jung Noh, Hun Soo Kim, Ha Young Kim, Byoung Hyun Park, Chung Gu Cho, Jin Won Jeong; J Korean Endocr Soc. 2009;24(1):47-53. Published online March 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.1.47

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Abstract PDF

A 52-year-old female was admitted to the hospital with abdominal pain. Her electrocardiogram revealed ST depressions in leads II, III, aVF and V2-5. The echocardiography showed transient cardiomyopathy with akinesia of the basal and mid portions of the left ventricle and hyperkinesia of the apex. There was no evidence of any vascular lesion on the emergency coronary angiography. She was diagnosed with pheochromocytoma by abdominal computed tomography and the post-operative pathologic examinations. These findings led us to a diagnosis of inverted Takotsubo cardiomyopathy related with pheochromocytoma. The recognition of such a rare cardiac manifestation should be considered in the diagnosis of pheochromocytoma, and especially in the circumstances of acute heart failure.

Citations

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A Case of Malignant Pheochromocytoma Presenting as Inverted Takotsubo-Like Cardiomyopathy
Jung Eun Jang, Hyuk Hee Kwon, Min Jung Lee, Chang Hee Jung, Sung Jin Bae, Hong Kyu Kim, Woo Je Lee
Endocrinology and Metabolism.2012; 27(1): 98. CrossRef

A Case of Graves' Disease with Pheochromocytoma.: Hye Sook Kim, Hyung Joon Joo, Yoon Seok Choi, Eun Sun Kim, Soo Yeon Park, Yun Jeong Lee, Hee Young Kim, Ji A Seo, Sin Gon Kim, Dong Seop Choi; J Korean Endocr Soc. 2007;22(6):465-469. Published online December 1, 2007; DOI: https://doi.org/10.3803/jkes.2007.22.6.465

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Abstract PDF

Graves' disease is an autoimmune disorder in which thyrotropin-stimulating hormone receptor antibodies stimulate the thyroid gland. Stress hormones such as catecholamine are known to play important roles in the pathogenesis of Graves' disease. Pheochromocytoma with Graves' disease is extremely rare, and no case has been reported within the Republic of Korea. However, according to previous studies conducted abroad, pheochromocytoma influences the pathogenesis of Graves' disease by producing excessive cathecholamine. In the present report, we describe a 65-year-old female patient with paroxysmal hypertension and rapidly progressive body weight loss who was diagnosed as having Graves'disease with pheochromocytoma.

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Graves' Disease Accompanied by Pheochromocytoma: Report of a Case
Jin-Hwa Kim, Sang-Jun Lee, Ji-Hye Shin, Mi-Ra You, Jae-Sik Jung, Sang-Yong Kim, Hak-Yeon Bae
Journal of Korean Endocrine Society.2009; 24(2): 126. CrossRef

A Case of Spontaneous Rupture of Adrenal Pheochromocytoma.: Eun Joo Kim, Mie Jin Lim, Byoung Wook Bang, Hyun Joo Park, Seong Bin Hong, Moon Suk Nam, Yong Seong Kim, Suk Jin Choi; J Korean Endocr Soc. 2006;21(5):424-427. Published online October 1, 2006; DOI: https://doi.org/10.3803/jkes.2006.21.5.424

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Abstract PDF

Although the spontaneous rupture of a pheochromocytoma is rare, it can be fatal if not promptly diagnosed. Hemorrhagic necrosis of the tumor will require an emergency operation, as this causes intraperitoneal hemorrhaging. Excessive vasoconstriction, tachycardia and labile blood pressure are the signs of a ruptured pheochromocytoma. The standard diagnostic tool of such a condition is an abdominal computed tomographic scan. Although the exact mechanism of the rupture is still unknown, a hemorrhage inside the tumor or a high intra-cortical pressure, due to rapid growth of the tumor, is known to play a major role in the pathogenesis. Herein is reported the case of a 53-year old male patient with a pheochromocytoma and an acute abdomen. The CT scan showed a left adrenal gland mass, with a hemorrhage in the left perirenal space. The patient underwent an emergency adrenalectomy, with the final histopathological diagnosis being that of a pheochromocytoma. Prompt recognition and early surgical intervention can improve outcome.

Citations

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Pheochromocytoma manifested as a surgical emergency
Eugenia Yiannakopoulou
European Journal of Surgical Oncology.2025; 51(11): 110439. CrossRef
Ruptured functioning adrenal tumour, atypical presentation with renal colic and hypertension
Amr Elmoheen, Mohamed Yousry, Ahmed Elmesery, Khalid Bashir
BMJ Case Reports.2020; 13(12): e236050. CrossRef
Spontaneous ruptured pheochromocytoma: an unusual case report and literature review
Ye Seob Jee
Annals of Surgical Treatment and Research.2017; 93(3): 170. CrossRef

Urinary Bladder Pheochromocytoma with a Long Asymptomatic Period.: Soon Hong Park, Jung Eun Lee, Sung Soo Moon, Joo Young Lee, Eui Dal Jung, Jung Guk Kim, In Kyu Lee, Bo Wan Kim; J Korean Endocr Soc. 2006;21(5):402-407. Published online October 1, 2006; DOI: https://doi.org/10.3803/jkes.2006.21.5.402

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Abstract PDF: Pheochromocytoma arises from chromaffin cells of the paraganglionic system. Urinary bladder pheochromocytoma is very rare tumor that accounts for less than 0.06% of all urinary bladder tumors and it is less than 1% of all pheochromocytoma. Urinary bladder pheochromocytoma usually arises from the sympathetic nervous system of the bladder wall. Paroxysmal headache and palpitation precipitated by micturition are common specific symptoms of this tumor. The paroxysm commonly persists or the patients relapse in a few days or months. Herein, we report a case of urinary bladder pheochromocytoma with a long asymptomatic period.

A Case Report of von Hippel-Lindau Disease Manifested in a Monozygous Twin.: Jin Woo Kim, Sang Jo Choi, Yeon Kyeong Kim, Sang Mi Ahn, Kyoung Eun Song, Sun Hye Jung, Dae Jung Kim, Yoon Sok Chung, Kwan Woo Lee, Il Jin Kim, Hio Chung Kang, Jae Gahb Park; J Korean Endocr Soc. 2005;20(4):395-400. Published online August 1, 2005; DOI: https://doi.org/10.3803/jkes.2005.20.4.395

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Abstract PDF

Von Hippel-Lindau (VHL) disease is an autosomal dominant disease, which forms hypervascular tumors in multiple organs, such as hemangioblastomas in the retina and central nervous system, renal cell carcinomas, pheochromocytomas and cysts in various organs. Recent advances in gene testing have made it possible to screen family members for VHL disease. We experienced a 28 year-old male, who was diagnosed with bilateral pheochromocytomas through a family screening test when his elder monozygous twin brother was diagnosed with a pheochromocytoma. He received no treatment until December, 2004, when he visited the Emergency room due to a headache. A hemangioma of the cerebellum was seen in the brain MR study, leading to the diagnosis of type 2A VHL disease. An abdominal CT scan revealed no lesions of the pancreas or kidney. There was no evidence of a hemangioma in the retinal scan. The subsequent gene testing showed a germline mutation in exon 3 codon 167 of the VHL gene. The mother of the patient was revealed to have the same mutation of the VHL gene, but the elder brother of the patient did not.

Citations

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Germline mutation of Glu70Lys is highly frequent in Korean patients with von Hippel–Lindau (VHL) disease
Sena Hwang, Cheol Ryong Ku, Ji In Lee, Kyu Yeon Hur, Myung-Shik Lee, Chul-Ho Lee, Kyo Yeon Koo, Jin-Sung Lee, Yumie Rhee
Journal of Human Genetics.2014; 59(9): 488. CrossRef
A Case of Type 1 von Hippel-Lindau (VHL) Disease associated with VHL Germline Mutation
Jeong Hoon Seo, Jae Hong Yang, Pyoung Lak Choi, Yu Lee Kim, Young Sik Choi, Yo Han Park, Ji Ho Ko, Hio Chung Kang, IL Jin Kim, Jae Gahb Park
Journal of Korean Endocrine Society.2006; 21(3): 239. CrossRef

A Case of Pheochromocytoma Presented with Cardiogenic Shock.: Mi Young Do, Hee Man Kim, Young Guk Ko, Sung Kil Lim, Jae Hun Jung, Namsik Chung, Yeon A Kim, Shin Ae Kang, Jae Hoon Moon, Jin Hyung Lee, Sang Tae Choi; J Korean Endocr Soc. 2005;20(3):283-288. Published online June 1, 2005; DOI: https://doi.org/10.3803/jkes.2005.20.3.283

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Abstract PDF

A pheochromocytoma is a catecholamine secreting tumor, which is often overlooked when cardiovascular complications, such as acute heart failure, myocardial infarction, angina pectoris, arrhythmias, and dilated cardiomyopathy, presented as the initial clinical manifestations. Failure to identify a pheochromocytoma in these situations may be fatal. We report the case of 32-year-old female, who presented with cardiogenic shock. Echocardiography revealed severe global hypokinesia of the dilated left ventricle, with the exception of the apex. Computed tomography of the aorta showed a well-enhanced left adrenal mass, 3.5cm in diameter. A 24 hour urine collection study for catecholamines and a 131I-metaiodobenzylguanidine(MIBG) scan were suggestive of the diagnosis of a single adrenal pheochromocytoma. The patient stabilized after shock management, and recovered with intensive medical treatment. Follow-up echocardiography revealed normalized cardiac function and chamber dimensions. Thereafter, the adrenal mass was successfully removed using laparaoscopic surgery, without complications

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A Case of Pheochromocytoma Crisis with Acute Myocardial Infarction Induced by Glucocorticoids Administration
Woo Sun Rou, Sang Kyung Jung, Sung Yun Lee, Yun Jeong Lee, Dong Jun Kim, Young Doo Kim, Hyung Yoon Kim, Sunhee Chang, Jung Hyun Noh
Endocrinology and Metabolism.2010; 25(3): 240. CrossRef

Original Article

The Efficacy of MIBG Scan as a Diagnostic and Docalization Test for Pheochromocytoma.: Cheol Ku Park, Kyeong Won Kim, Do Hee Kim, Jae Hyeon Kim, Jun Gu Kang, San Wan Kim, Young Min Cho, Do Joon Park, Chan Soo Shin, Kyong Soo Park, Bo Youn Cho, Hong Kyu Lee, Seong Yeon Kim; J Korean Endocr Soc. 2005;20(1):21-28. Published online February 1, 2005; DOI: https://doi.org/10.3803/jkes.2005.20.1.21

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Abstract PDF: BACKGROUND
Computed tomography(CT) is currently considered as the initial imaging procedure of choice for the localization of pheochromocytomas in most of the cases. 131I-or 123I-Metaiodobenzylguanidine scintigraphy(MIBG scan) was proven to be a highly specific tool for the detection of adrenal and extra-adrenal pheochromocytomas, but was less sensitive than CT. The present study is aimed to evaluate the usefulness of a MIBG scan in diagnosis and localization of pheochromocytoma when compared to CT. METHODS: We retrospectively evaluated 27 patients who underwent a MIBG scan for a pheochromocytoma at the Seoul National University Hospital from the year 2000 and 2002. According to the pathological and clinical findings, in 16 the patients pheochromocytoma was confirmed to be positive and the rest 11 of the patients were excluded from the study. RESULTS: Pheochromocytomas was identified in 16 patients. Eleven of them were localized in adrenal gland and 5 were extra-adrenal lesions. The sensitivity to MIBG scan in adrenal lesions and extra-adrenal lesions, was 72%(8/11) and 40%(2/5) respectively. In our study, the overall sensitivity to MIBG scan was 62%(10/16), and overall specificity was 90.9%(10/11). By CT four were identified to have equivocal biochemical abnormalities, but were definite and extraadrenal tumors by MIBG scan showed abnormal uptakes in two of them. CONCLUSION: The MIBG scan was especially useful in 2 of the 27 patients but we had no experienced about the additional benefits of a MIBG scan in the other 25 cases. Our results reveal that a MIBG scan should be performed carefully for the diagnosis and localization of a pheochromocytoma, while considering cost and time of operation.

Evaluation Studies

Diagnostic Value of 24-hours Urinary Total Metanephrine As a Screening Test of Patients with Suspected Pheochromocytoma.: Sang Wan Kim, Kyung Won Kim, Do Joon Park, Chan Soo Shin, Kyung Soo Park, Seong Yeon Kim, Bo Youn Cho, Hong Kyu Lee; J Korean Endocr Soc. 2005;20(1):12-20. Published online February 1, 2005; DOI: https://doi.org/10.3803/jkes.2005.20.1.12

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Abstract PDF: BACKGROUND
A pheochromocytoma is a rare cause of secondary hypertension. Its diagnosis is important as the hypertension is usually curable by resection of the tumor, whereas the condition is potentially lethal if undetected. Biochemical confirmation of excessive catecholamine production is a prerequisite to a definitive diagnosis. Various studies from other countries have indicated that measuring of the urinary metanephrine, using a specific procedure, is the single most reliable screening test for all patients suspected of having a pheochromocytoma. However, the diagnostic value of urinary metanephrine has never been reported in Korea. We investigated the diagnostic value of 24-hours urinary metanephrine in patients with a suspected pheochromocytoma. METHODS: This was a retrospective evaluation study, which included 95 patients with sustained hypertension and paroxysmal symptoms, and 38 patients with asymptomatic adrenal incidentaloma at Seoul National University Hospital, between July 2000 and October 2002. We performed the 24- hour urinary total metanephrine test on all patients. The diagnosis of a pheochromocytoma was applied only when confirmed by pathological analysis of a resected specimen. The possibility of a pheochromocytoma was ruled out when all biochemical tests were normal, which were performed at least in duplicate, or there was no evidence of a mass in abdominal radiological studies or histological verification. We determined the upper reference limit for urinary metanephrine as 1.3mg/day and calculated the sensitivity and specificity of the 24-hour urinary metanephrine test. RESULTS: Seventeen patients were diagnosed with a pheochromocytoma. The total metanephrine measurement had sensitivities and specificities of 82.4 and 73.3% in all the patients, 90.9 and 66.7% in patients with hypertension and paroxysmal symptoms, and 66.7 and 90.6% in patients with adrenal incidentaloma, respectively. CONCLUSION: The urinary total metanephrine measurement had relatively lower sensitivities and specificities than in other countries(sensitivity: 83~100%, specificity: 80~98%). The sensitivity of urinary metanephrine was relatively high in patients with hypertension and paroxysmal symptoms, and the specificity was high in patients with an adrenal incidentaloma. We suggest that normetanephrine and metanephrine should be separately measured, and a reasonable upper reference limit be used. It may also be necessary to measure urinary metanephrine together with urinary catecholamine or VMA to improve the diagnostic value of the urinary metanephrine test.

Case Report

A Case of Apical Hypertrohic Cardiomyopathy Associated with Pheochromocytoma.: Joon Ho Moon, Sung Woo Park, Sung Hee Ihm, Cheol Young Park, Ki Won Oh, Cheol Soo Choi, Seong Jin Lee, In Kyung Jung, Eun Gyung Hong, Hyeon Kyu Kim, Doo Man Kim, Jae Myung Yoo, Moon Gi Choi, Hyung Joon Yoo, So Young Ku, Soo Kee Min; J Korean Endocr Soc. 2004;19(5):522-527. Published online October 1, 2004

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Abstract PDF: Pheochromocytomas often present with cardiovascular manifestations, such as arrhythmia, angina pectoris and acute myocardial infarction and so on. Both dilated and nonobstructive hypertrophic cardiomyopathies are also rare complications of pheochromocytomas. In hypertrophic cardiomyopathy, an apical variant form constitutes about 25% of cases in Japan, but only 1 to 2% of those in non-Japanese populations, including Korea. The cause of apical hypertrophic cardiomyopathy (AHC) remains unknown. Recently, some cases of AHC associated with pheochromocytomas have been reported, with catecholamine thought to be an important cause. AHC associated with a pheochromocytoma has never been previously reported in Korea. Herein is reported our experience of a case of apical hypertrophic cardiomyopathy associated a pheochromocytoma with a review of the literature

Original Article

A Case of Multiple Endocrine Neoplasia 2A with Germ Line Mutation of RET Gene.: Hee Young Kim, Ji Yeon Lee, Sung Bum Kim, Kye Won Lee, Ji A Seo, Jeong Heon Oh, Sin Gon Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi, Nan Hee Kim; J Korean Endocr Soc. 2003;18(5):481-488. Published online October 1, 2003

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Abstract PDF: Multiple endocrine neoplasia 2A (MEN 2A) is an autosomal dominantly inherited disease, composed of medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. The activation of germ-line mutations in the RET proto-oncogene are responsible for MEN 2. The analysis of the RET mutations has replaced the measurement of the calcitonin level in the diagnosis of the MEN carrier state. Specific RET codon mutations correlate with the MEN 2 syndromic variant, the age at onset of the medullary thyroid carcinoma (MTC) and the aggressiveness of the MTC. Herein, our experience of a 47-year-old woman, who had a bilateral pheochromocytoma and MTC, and MEN 2A confirmed by the detection of an RET proto-oncogene mutation at axon 10 on codon 618, is reported. Her sister was found to have the same mutant gene. After a total thyroidectomy and bilateral adrenalectomy, the calcitonin and catecholamine levels were normalized, and the patient discharged without problems. This case is reported, with a review of the literature.

Case Reports

A Case of Pheochromocytoma with Ganglioneuronal Differentiation Producing Vasoactive Intestinal Peptide.: Min Ah Na, Sang Soo Kim, Dong Uk Kim, Jae Hoon Choi, Tae Kun Lee, Ok Nyu Kong, Jun Hyeop An, Jee Yeon Kim, Han Dong Sung, Chul Ho Joung, Seok Man Son, In Joo Kim, Yong Ki Kim; J Korean Endocr Soc. 2003;18(2):227-231. Published online April 1, 2003

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Abstract PDF: Vasoactive intestinal peptide(VIP) is a 28-amino acid peptide, which may cause secretory diarrhea by stimulating the production of adenylate cyclase. Neuroendocrine tumors, secreting vasoactive intestinal peptide (VIP), are almost always of a pancreatic in origin. However, a pheochromocytoma may produce several neuropeptides, containing VIP, as they are considered to be neuroendocrine tumors. A 57-year-old woman, who presented with chronic watery diarrhea, hypokalemia, weight loss and a left adrenal mass, is described. Histologically, the tumor was diagnosed as a pheoch-romocytoma, with ganglioneuronal differentiation, and was histochemically confirmed to produce a vasoactive intestinal polypeptide. A left adrenal VIP-producing pheochromocytoma was successfully resected. After surgery, her diarrhea subsided and the electrolytes, affected neuroendocrine hormone levels, blood pressure and blood sugar level were normalized.

A Case of Multiple Endocrine Neoplasia Type 2B associated with a M918T Mutation in RET Proto-Oncogene.: Tae Yong Kim, Jae Kyung Hwang, Min Kyong Moon, Young Joo Park, Do Joon Park, Seong Yeon Kim, Hong Kyu Lee, Yo Kyu Yoon, Bo Youn Cho; J Korean Endocr Soc. 2003;18(1):85-93. Published online February 1, 2003

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Abstract PDF: A multiple endocrine neoplasia type 2B(MEN2B) is the most distinct and aggressive form of the MEN type 2 variants. We report a case of a 24-years-old woman with MEN2B. The patient had previously undergone a Duhamel's operation due to a megacolon at 6 years old, minor surgery to remove small tumors on the lip at 8 years old, and a bilateral osteotomy of the femur, due to coxa valga, at 15 years old. She underwent a total thyroidectomy and neck dissection, due to a growing thyroid nodule, despite thyroxine treatment, at 19 years old. The pathology revealed a medullary thyroid carcinoma. There was no history of MEN 2B in her family. She had prominent lips, multiple oral mucosal masses, and marfanoid habitus. During the subsequent follow-up, a positron emission tomogram was taken due to a persistently high level of serum calcitonin, despite repeated neck dissections, which revealed a mass in the right adrenal gland. Adrenomedullary function tests showed high levels of urinary catecholamine metabolites, and a genetic analysis of the peripheral leukocyte showed a codon 918 mutation (Met918Thr) at exon 16 of the RET proto-oncogene. The patient underwent a right adrenalectomy and the pathology revealed a pheoch-romocytoma.

Original Article

Clinical Study of the Pheochromocytoma.: Dong Hyun Shin, Sin Gon Kim, Dong Rim Kim, Nan Hee Kim, Kyung Mook Choi, Saih Yun Baik, Dong Seop Choi, Sung Ock Suh; J Korean Endocr Soc. 2002;17(4):554-563. Published online August 1, 2002

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Abstract PDF: BACKGROUND
Pheochromocytoma may arise within the adrenal medulla, or in other locations where sympathetic ganglia or chromaffin tissues are known to exist. Approximately 0.1% of hypertensive patients have pheochromocytoma. Most of this hypertension can be cured by surgical removal of the tumor, but lethal complication can develop if proper treatment is not prepared. Therefore, accurate diagnosis and preoperative preparation is very important. The objective of this study was to evaluate the clinical characteristics of pheochromocytoma and the blood pressure change following tumor removal. METHOD: The medical records of 45 patients [29 cases of intra-adrenal, 16 cases of extra-adrenal (paraganglioma)] diagnosed with of pheochromocytoma at Korea University Medical Center between 1991 and 2001 were reviewed. RESULTS: Twenty of the cases were male and 25 were female. The mean age of these patients was 43 years old. Hypertension, headaches, palpitations and impaired glucose tolerance were observed more frequently in cases of intra-adrenal tumor than in those of extra-adrenal. In the extra-adrenal group, abdominal pain, hypertension and nausea were more frequently observed. A biochemical study showed that the sensitivity of the test for catecholamines and metabolites in 24 hours urine was over 80%. For the localization of tumors we used abdominal CT and MIBG. The sensitivities of the CT and MIBG for the tumor localization were 97 and 91%, respectively. Blood pressures during the operations were effectively controlled by preoperative treatment with phenoxybenzamine (non-competitive, non-selective -adrenoreceptor antagonists). CONCLUSION: Surgical treatment cured 23 of the 30 cases of hypertension with pheochromocytoma. We have to take careful approaches in the care of the patient who may have pheochromocytoma, due to the various clinical signs and symptoms.

Case Reports

A Case of Cured Diabetes Mellitus after Pheochromocytoma Removal.: Chang Kyun Hong, Yu Bae Ahn, Sul Hye Kim, Young Sik Woo, Seoung Goo Lee, Seung Hyun Ko, Ho Ki Song, Kun Ho Yoon, Moo Il Kang, Bong Yeon Cha, Kwang Woo Lee, Ho Young Son, Sung Koo Kang; J Korean Endocr Soc. 2001;16(4-5):502-507. Published online October 1, 2001

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Abstract PDF: Pheochromocytoma is usually associated with a combination of various clinical manifestations caused by the overproduction of catecholamines. It is frequently accompanied by impaired glucose tolerance operating through 2-and-adrenergic mechanisms. A 41-year-old-woman was admitted to the hospital because of poorly-controlled diabetes mellitus and hypertension. She had suffered intermittent paroxysmal attacks of headache and chest discomfort and had been treated intermittently over a 2 year period for diabetes mellitus and hypertension. At admission, the levels of serum epinephrine, norepinephrine urinary excretion of total metanephrine, and VMA were all abnormally elevated. Adrenal CT showed a well-defined, homogenous mass in the right adrenal region and the tumor was diagnosed as pheochromocytoma. After tumor resection, the increased blood level of catecholamines, the urinary excretion of total metanephrine, and VMA were normalized, as was the hyperglycemia state. Diabetes mellitus of the patient was considered permanently resolved after tumor removal by the result of glucose tolerance in 75g oral glucose tolerance test.

A Case of Pheochromocytoma with Acute Myocardial Infarction.: Yoo Bae Ahn, Moo Il Kang, Kwang Woo Lee, Ho Young Son, Sung Koo Kang, Bong Yeon Cha, Baek Jong Seo, Ki Yook Jang, In Jae Yoon, Sang Jun Lee, Sun Sook Park, Yong Seok Oh; J Korean Endocr Soc. 1997;12(4):655-660. Published online January 1, 2001

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Abstract PDF: Pheochromocytoma, the catecholamine-producing tumor of chromaffin tissue, is associated with a curable form of hypertension. Recently we report the case of a 59 year-old male admitted for an acute myocardial infarction and who subsequently developed late recurrent severe ventricular arrhythmia coincident with transient hypertensive episodes. A pheochromocytoma was diagnosed on the basis of the urinary concentration of catecholamines and computerized tomography of the adrenal glands. After stabilization of his cardiac rhythm and blood pressure with alpha adrenergic blockade, the left adrenal gland, which contained the tumor, was subsequently resected. The diagnosis of a pheochromocytoma should be considered when recurrent ventricular arrhythmia are associated with intermittent hypertension after acute myocardial infarction.

A Case of Neurofibromatosis with Bilateral Pheochromocytoma.: Hong Seung Kim, Young Gu Shin, Il Hoi Kim, Yun Mee Kim, Mee Yeon Cho; J Korean Endocr Soc. 1997;12(3):478-484. Published online January 1, 2001

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Abstract PDF: Pheochromocytoma is originated from chromaffin cell of sympathetic nervous system and associated with other disease, such as neurofibromatosis, duodenal carcinoid, medullary thyroid cancer and parathyroid adenoma. Especially, pheochromocytoma is developed more than 50% in neurofibromatosis associated with hypertension. In such cases, several clinical features documented as more frequent bilateral phochromocytoma, more associated with other neuroendocrine tumors and thus more poor prognosis. We can observe the sustained hypertension despite of surgical resection of tumors in pheochromocytoma cases. One of the possible reason of post operative sustained hypertension is the pheochromocytoma originated from minor organ of Zukerkandl that was not resected during operation. Untreated or delayed treated cases with pheochromocytoma were often expired by complication of hypertension such as cerebrovascular hemorrhage, myocardial infarction, etc. Thus, in neurofibromatosis with hypertension, screening of pheochromocytorna is very important for the early detection of tumor and more favorable prognosis. Recently, We experienced a case of neurofibromatosis associated with bilateral pheochromocy-toma expired by cerebral hemorrhage during operation, so we report the case with literature review.

A Case of Insulinoma Combined with Pheochromocytoma.: Si Hoon Lee, Young Hoon Youn, Sung Kil Lim, Bong Soo Cha, Hyun Chul Lee, Tae Woong Noh, Kap Bum Huh; J Korean Endocr Soc. 2000;15(4-5):640-647. Published online January 1, 2001

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Abstract PDF: Multiple endocrine neoplasia (MEN) is a combination of endocrine tumor associations and consists of 3 types: 1, 2A and 2B. Herein, we report a case of insulinoma which is combined with pheochromocytoma discovered at the left adrenal gland which was treated by surgical resection. We think there is a possibility of a new type of multiple endocrine neoplasia (MEN).

A Case of Pheochromocytoma Manifested by Generalized Seizure.: Young Mee Ahn, Young Ah Choi, Kyoung Won Park, Chung Hyun Chun, Chang Bai Lee, Eun Sun Hong, Yeon Ah Sung, Nan Ho Kyung; J Korean Endocr Soc. 2000;15(4-5):622-626. Published online January 1, 2001

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Abstract PDF: Pheochromocytoma is originated from chromaffin cells of sympathetic nervous system and clinical symptoms are caused by catecholamine released from tumor. In typical cases, periodic attacks of hypertension, palpitation, headache, and sweating are main symptoms. But, the diagnosis of pheochromocytoma is sometimes difficult since its clinical presentation is quite variable. We evaluated a 25-year-old woman who was admitted because of left flank pain and seizure. She had a history of hemoptysis and toxemia. During the hospitalization, she experienced generalized seizure once more. The clinical diagnosis of pheochromocytoma was proven by highly elevated urinary catecholamines and confirmed histologically after operation. We think it is the first case report of pheochromocytoma manifested by seizure in korea, so we report this case with literature review.

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