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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
  • 3,260 View
  • 121 Download
  • 2 Web of Science
  • 2 Crossref
AbstractAbstract PDFPubReader   ePub   
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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Citations to this article as recorded by  
  • Editorial: Tumor metabolism and programmed cell death
    Dan-Lan Pu, Qi-Nan Wu
    Frontiers in Endocrinology.2024;[Epub]     CrossRef
  • Molecular subtypes of clear cell renal carcinoma based on PCD-related long non-coding RNAs expression: insights into the underlying mechanisms and therapeutic strategies
    Han Wang, Yang Liu, Aifa Tang, Xiansheng Zhang
    European Journal of Medical Research.2024;[Epub]     CrossRef
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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
  • 4,847 View
  • 198 Download
  • 3 Web of Science
  • 4 Crossref
AbstractAbstract PDFSupplementary MaterialPubReader   ePub   
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.

Citations

Citations to this article as recorded by  
  • The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center
    Inês Costa Carvalho, Miguel V. B. Machado, João P. Morais, Filipa Carvalho, Elisabete Barbosa, José Barbosa
    Endocrine.2024;[Epub]     CrossRef
  • Laparoscopic Resection of Para-Aortic Mass at the Aortic Bifurcation: An Atypical Presentation of a Tailgut Cyst
    Andy Wang, Jiddu A Guart, Danielle Li, Trenton Taros, Hongyi Cui
    Cureus.2024;[Epub]     CrossRef
  • Biochemical Assessment of Pheochromocytoma and Paraganglioma
    Graeme Eisenhofer, Christina Pamporaki, Jacques W M Lenders
    Endocrine Reviews.2023; 44(5): 862.     CrossRef
  • Adrenal bleeding due to pheochromocytoma - A call for algorithm
    Ewelina Rzepka, Joanna Kokoszka, Anna Grochowska, Magdalena Ulatowska-Białas, Martyna Lech, Marta Opalińska, Elwira Przybylik-Mazurek, Aleksandra Gilis-Januszewska, Alicja Hubalewska-Dydejczyk
    Frontiers in Endocrinology.2022;[Epub]     CrossRef
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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
  • 8,275 View
  • 620 Download
  • 9 Web of Science
  • 11 Crossref
AbstractAbstract PDFPubReader   ePub   
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.

Citations

Citations to this article as recorded by  
  • 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
    Clinical Nuclear Medicine.2024; 49(1): 27.     CrossRef
  • Preoperative prediction of metastatic pheochromocytoma and paraganglioma using clinical, genetic, and biochemical markers: A cohort study
    Seung Shin Park, Chang Ho Ahn, Seunghoo Lee, Woochang Lee, Won Woong Kim, Yu‐Mi Lee, Su Jin Kim, Tae‐Yon Sung, Kyu Eun Lee, Jung Hee Kim, Seung Hun Lee, Jung‐Min Koh
    Journal of Internal Medicine.2024; 296(1): 68.     CrossRef
  • Evaluation and Management of Bone Health in Patients with Thyroid Diseases: A Position Statement of the Korean Thyroid Association
    A Ram Hong, Ho-Cheol Kang
    Endocrinology and Metabolism.2023; 38(2): 175.     CrossRef
  • Lesion-based indicators predict long-term outcomes of pheochromocytoma and paraganglioma– SIZEPASS
    Helena Hanschell, Salvador Diaz-Cano, Alfredo Blanes, Nadia Talat, Gabriele Galatá, Simon Aylwin, Klaus Martin Schulte
    Frontiers in Endocrinology.2023;[Epub]     CrossRef
  • Interleukin-6-producing paraganglioma as a rare cause of systemic inflammatory response syndrome: a case report
    Yin Young Lee, Seung Min Chung
    Journal of Yeungnam Medical Science.2023; 40(4): 435.     CrossRef
  • (Extremely rare intrapericardial location of paraganglioma)
    Jaroslav Zajíc, Aleš Mokráček, Ladislav Pešl, Jiří Haniš, Dita Schaffelhoferová
    Cor et Vasa.2023; 65(4): 692.     CrossRef
  • A Case of Von Hippel-Lindau Disease With Recurrence of Paraganglioma and No Other Associated Symptoms: The Importance of Genetic Testing and Establishing Follow-Up Policies
    Naoki Okada, Akihiro Shioya, Sumihito Togi, Hiroki Ura, Yo Niida
    Cureus.2023;[Epub]     CrossRef
  • KSNM60 in Nuclear Endocrinology: from the Beginning to the Future
    Chae Moon Hong, Young Jin Jeong, Hae Won Kim, Byeong-Cheol Ahn
    Nuclear Medicine and Molecular Imaging.2022; 56(1): 17.     CrossRef
  • Change of Computed Tomography-Based Body Composition after Adrenalectomy in Patients with Pheochromocytoma
    Yousun Ko, Heeryoel Jeong, Seungwoo Khang, Jeongjin Lee, Kyung Won Kim, Beom-Jun Kim
    Cancers.2022; 14(8): 1967.     CrossRef
  • Evaluation and Management of Bone Health in Patients with Thyroid Diseases: a Position Statement from the Korean Thyroid Association
    A Ram Hong, Hwa Young Ahn, Bu Kyung Kim, Seong Hee Ahn, So Young Park, Min-Hee Kim, Jeongmin Lee, Sun Wook Cho, Ho-Cheol Kang
    International Journal of Thyroidology.2022; 15(1): 1.     CrossRef
  • Pheochromocytoma with Retroperitoneal Metastasis: A Case Report
    建新 崔
    Advances in Clinical Medicine.2021; 11(05): 2239.     CrossRef
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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
  • 5,723 View
  • 161 Download
  • 10 Web of Science
  • 8 Crossref
AbstractAbstract PDFSupplementary MaterialPubReader   ePub   
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.

Citations

Citations to this article as recorded by  
  • 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
  • Case Report: Aggressive neural crest tumors in a child with familial von Hippel Lindau syndrome associated with a germline VHL mutation (c.414A>G) and a novel KIF1B gene mutation
    Lucie Landen, Anne De Leener, Manon Le Roux, Bénédicte Brichard, Selda Aydin, Dominique Maiter, Philippe A. Lysy
    Frontiers in Endocrinology.2023;[Epub]     CrossRef
  • A Case of Pheochromocytoma as a Subsequent Neoplasm in a Survivor of Childhood Embryonal Rhabdomyosarcoma
    Rozalyn L. Rodwin, Sanyukta K. Janardan, Erin W. Hofstatter, Nina S. Kadan-Lottick
    Journal of Pediatric Hematology/Oncology.2022; 44(2): e585.     CrossRef
  • Mutations of 1p genes do not consistently abrogate tumor suppressor functions in 1p-intact neuroblastoma
    Chik Hong Kuick, Jia Ying Tan, Deborah Jasmine, Tohari Sumanty, Alvin Y. J. Ng, Byrrappa Venkatesh, Huiyi Chen, Eva Loh, Sudhanshi Jain, Wan Yi Seow, Eileen H. Q. Ng, Derrick W. Q. Lian, Shui Yen Soh, Kenneth T. E. Chang, Zhi Xiong Chen, Amos H. P. Loh
    BMC Cancer.2022;[Epub]     CrossRef
  • 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
    Endocrine Journal.2022; 69(6): 705.     CrossRef
  • 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
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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
  • 4,943 View
  • 190 Download
  • 11 Web of Science
  • 11 Crossref
AbstractAbstract PDFSupplementary MaterialPubReader   ePub   
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.

Citations

Citations to this article as recorded by  
  • 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
  • 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
    Medicine.2024; 103(17): e37916.     CrossRef
  • Validation and Evaluation of 5 Scoring Systems for Predicting Metastatic Risk in Pheochromocytoma and Paraganglioma
    Qin Li, Zhigang Lan, Yong Jiang, Rui Wang, Ziyao Li, Xiaolin Jiang
    American Journal of Surgical Pathology.2024; 48(7): 855.     CrossRef
  • 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
    Biomedicines.2024; 12(6): 1352.     CrossRef
  • Novel and recurrent genetic variants of VHL, SDHB, and RET genes in Chinese pheochromocytoma and paraganglioma patients
    Chong Li, Jingyi Li, Chao Han, Ting Wang, Lixia Zhang, Zhifang Wang, Tingting Wang, Lijun Xu, Guangzhao Qi, Guijun Qin, Xialian Li, Lili Zheng
    Frontiers in Genetics.2023;[Epub]     CrossRef
  • Genetic Study in Pheochromocytoma: Is It Possible to Stratify the Risk of Hereditary Pheochromocytoma?
    Marta Araujo-Castro, César Mínguez Ojeda, Iñigo García Sanz, Maria Calatayud, Felicia Hanzu, Mireia Mora, Almudena Vicente, Concepción Blanco Carrera, Paz de Miguel Novoa, María del Carmen López García, Cristina Lamas, Laura Manjón-Miguélez, María del Cas
    Neuroendocrinology.2023; 113(6): 657.     CrossRef
  • The Role of VHL in the Development of von Hippel-Lindau Disease and Erythrocytosis
    Petra Hudler, Mojca Urbancic
    Genes.2022; 13(2): 362.     CrossRef
  • Bilateral Pheochromocytoma with Germline MAX Variant without Family History
    Shinnosuke Hata, Mai Asano, Hiroyuki Tominaga, Masahide Hamaguchi, Fumiya Hongo, Takeshi Usui, Eiichi Konishi, Michiaki Fukui
    Clinics and Practice.2022; 12(3): 299.     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
  • 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
    Cancers.2021; 13(16): 4014.     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
Close layer
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
  • 7,777 View
  • 186 Download
  • 27 Web of Science
  • 26 Crossref
AbstractAbstract PDFPubReader   ePub   
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  
  • Preoperative prediction of metastatic pheochromocytoma and paraganglioma using clinical, genetic, and biochemical markers: A cohort study
    Seung Shin Park, Chang Ho Ahn, Seunghoo Lee, Woochang Lee, Won Woong Kim, Yu‐Mi Lee, Su Jin Kim, Tae‐Yon Sung, Kyu Eun Lee, Jung Hee Kim, Seung Hun Lee, Jung‐Min Koh
    Journal of Internal Medicine.2024; 296(1): 68.     CrossRef
  • Seventy years of pheochromocytomas and paragangliomas in Argentina. The FRENAR database
    V.C. de Miguel, L.S. Aparicio, G. Sansó, A.L. Paissan, S.N. Lupi, S.H. Belli, J. Tkatch, M.J. Marín, M.B. Barontini
    Hipertensión y Riesgo Vascular.2024;[Epub]     CrossRef
  • Big Data Research in the Field of Endocrine Diseases Using the Korean National Health Information Database
    Sun Wook Cho, Jung Hee Kim, Han Seok Choi, Hwa Young Ahn, Mee Kyoung Kim, Eun Jung Rhee
    Endocrinology and Metabolism.2023; 38(1): 10.     CrossRef
  • Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician
    Sona Sharma, Lauren Fishbein
    Endocrine Practice.2023; 29(12): 999.     CrossRef
  • Pheochromocytoma: a changing perspective and current concepts
    Andreas Kiriakopoulos, Periklis Giannakis, Evangelos Menenakos
    Therapeutic Advances in Endocrinology and Metabolism.2023;[Epub]     CrossRef
  • Image-Guided Precision Medicine in the Diagnosis and Treatment of Pheochromocytomas and Paragangliomas
    Gildas Gabiache, Charline Zadro, Laura Rozenblum, Delphine Vezzosi, Céline Mouly, Matthieu Thoulouzan, Rosine Guimbaud, Philippe Otal, Lawrence Dierickx, Hervé Rousseau, Christopher Trepanier, Laurent Dercle, Fatima-Zohra Mokrane
    Cancers.2023; 15(18): 4666.     CrossRef
  • Trends in the incidence of adrenocortical carcinoma and pheochromocytoma/paraganglioma in Taiwan
    Chung-Hsin Tsai, Tun-Sung Huang, Shih-Ping Cheng
    Formosan Journal of Surgery.2023; 56(5): 147.     CrossRef
  • An open-label, single-arm, multi-center, phase II clinical trial of single-dose [131I]meta-iodobenzylguanidine therapy for patients with refractory pheochromocytoma and paraganglioma
    Anri Inaki, Tohru Shiga, Yoshito Tsushima, Megumi Jinguji, Hiroshi Wakabayashi, Daiki Kayano, Norihito Akatani, Takafumi Yamase, Yuji Kunita, Satoru Watanabe, Tomo Hiromasa, Hiroshi Mori, Kenji Hirata, Shiro Watanabe, Tetsuya Higuchi, Hiroyasu Tomonaga, S
    Annals of Nuclear Medicine.2022; 36(3): 267.     CrossRef
  • Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance
    Jan Calissendorff, Carl Christofer Juhlin, Irina Bancos, Henrik Falhammar
    Cancers.2022; 14(4): 917.     CrossRef
  • Characteristics of Intraoperative Hemodynamic Instability in Postoperatively Diagnosed Pheochromocytoma and Sympathetic Paraganglioma Patients
    Jung Hee Kim, Hyung-Chul Lee, Su-jin Kim, Kyu Eun Lee, Kyeong Cheon Jung
    Frontiers in Endocrinology.2022;[Epub]     CrossRef
  • Change of Computed Tomography-Based Body Composition after Adrenalectomy in Patients with Pheochromocytoma
    Yousun Ko, Heeryoel Jeong, Seungwoo Khang, Jeongjin Lee, Kyung Won Kim, Beom-Jun Kim
    Cancers.2022; 14(8): 1967.     CrossRef
  • Systematic Review: Incidence of Pheochromocytoma and Paraganglioma Over 70 Years
    Abdul Rahman Al Subhi, Veronica Boyle, Marianne S Elston
    Journal of the Endocrine Society.2022;[Epub]     CrossRef
  • Incidence and risk factors for myocardial injury after laparoscopic adrenalectomy for pheochromocytoma: A retrospective cohort study
    Ling Lan, Qian Shu, Chunhua Yu, Lijian Pei, Yuelun Zhang, Li Xu, Yuguang Huang
    Frontiers in Oncology.2022;[Epub]     CrossRef
  • The Clinical Characteristics of Pheochromocytomas and Paragangliomas with Negative Catecholamines
    Lin Zhao, Xiaoran Zhang, Xu Meng, Ting Zhang, Hua Fan, Qiongyu Zhang, Yecheng Liu, Xianliang Zhou, Huadong Zhu
    Journal of Clinical Medicine.2022; 11(19): 5583.     CrossRef
  • A midline ectopic paraganglioma
    Christina SANT FOURNIER, Matthias FARRUGIA, Kimberley PACE, Christian CAMENZULI, Alexander R. ATTARD
    Chirurgia.2022;[Epub]     CrossRef
  • Non-Selective Alpha-Blockers Provide More Stable Intraoperative Hemodynamic Control Compared with Selective Alpha1-Blockers in Patients with Pheochromocytoma and Paraganglioma: A Single-Center Retrospective Cohort Study with a Propensity Score-Matched Ana
    Yang Yang, Jie Zhang, Liqun Fang, Xue Jia, Wensheng Zhang
    Drug Design, Development and Therapy.2022; Volume 16: 3599.     CrossRef
  • Incidence and Clinical Presentation of Pheochromocytoma and Sympathetic Paraganglioma: A Population-based Study
    Andreas Ebbehoj, Kirstine Stochholm, Sarah Forslund Jacobsen, Christian Trolle, Peter Jepsen, Maciej Grzegorz Robaczyk, Åse Krogh Rasmussen, Ulla Feldt-Rasmussen, Reimar Wernich Thomsen, Esben Søndergaard, Per Løgstrup Poulsen
    The Journal of Clinical Endocrinology & Metabolism.2021; 106(5): e2251.     CrossRef
  • Determinants of anxiety and depression among pheochromocytoma patients
    Siming Jia, Chengbai Li, Zhuqing Lei, Qiang Xia, Yuqing Jiang
    Medicine.2021; 100(3): e24335.     CrossRef
  • Pheochromocytoma and paraganglioma with negative results for urinary metanephrines show higher risks for metastatic diseases
    Akiyuki Kawashima, Masakatsu Sone, Nobuya Inagaki, Kentaro Okamoto, Mika Tsuiki, Shoichiro Izawa, Michio Otsuki, Shintaro Okamura, Takamasa Ichijo, Takuyuki Katabami, Yoshiyu Takeda, Takanobu Yoshimoto, Mitsuhide Naruse, Akiyo Tanabe
    Endocrine.2021; 74(1): 155.     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
  • Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A 4-Decade Experience
    Thomas Uslar, Ignacio F San Francisco, Roberto Olmos, Stefano Macchiavelo, Alvaro Zuñiga, Pablo Rojas, Marcelo Garrido, Alvaro Huete, Gonzalo P Mendez, Ignacio Cortinez, José Tomás Zemelman, Joaquín Cifuentes, Fernando Castro, Daniela Olivari, José Miguel
    Journal of the Endocrine Society.2021;[Epub]     CrossRef
  • Pheochromocytoma
    Zhonghua Liu, Junsheng Ma, Camilo Jimenez, Miao Zhang
    American Journal of Surgical Pathology.2021; 45(9): 1155.     CrossRef
  • Perioperative hemodynamic instability in pheochromocytoma and sympathetic paraganglioma patients
    Jung Hee Kim, Hyung-Chul Lee, Su-jin Kim, Soo Bin Yoon, Sung Hye Kong, Hyeong Won Yu, Young Jun Chai, June Young Choi, Kyu Eun Lee, Kwang-Woong Lee, Seung-Kee Min, Chan Soo Shin, Kyu Joo Park
    Scientific Reports.2021;[Epub]     CrossRef
  • Changes in the Nociception Level Index During Surgical Resection of Paragangliomas: A Case Report
    Rita Saynhalath, Umar H. Khan, Gijo Alex, Joseph T. Murphy, Peter Szmuk
    A&A Practice.2021; 15(11): e01542.     CrossRef
  • Hypertension Cure and Reducing Pill Burden after Adrenalectomy for Endocrine Hypertension of Adrenal Origin: A Comparative Study from an Asian and UK Cohort
    Kee Y Ngiam, Ciaran Durand, Titus C Vasciuc, Chia H Tai, Raluca Orpean, Fiona Eatock, Mehak Mahipal, Tan W Boon
    World Journal of Endocrine Surgery.2021; 13(1): 9.     CrossRef
  • A Brief Overview of the Epidemiology of Pheochromocytoma and Paraganglioma in Korea
    Yun Mi Choi
    Endocrinology and Metabolism.2020; 35(1): 95.     CrossRef
Close layer
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
  • 1,547 View
  • 32 Download
AbstractAbstract 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.
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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
  • 1,085 View
  • 17 Download
AbstractAbstract 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.
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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
  • 1,295 View
  • 17 Download
AbstractAbstract 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.
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Original Article
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
  • 1,147 View
  • 20 Download
AbstractAbstract 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.
Close layer
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
  • 1,168 View
  • 29 Download
AbstractAbstract 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.
Close layer
Original Article
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
  • 1,119 View
  • 24 Download
AbstractAbstract 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.
Close layer
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
  • 5,398 View
  • 54 Download
  • 14 Web of Science
  • 12 Crossref
AbstractAbstract PDFPubReader   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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  • High Risk of Fractures Within 7 Years of Diagnosis in Asian Patients With Inflammatory Bowel Diseases
    Hyung Jin Ahn, Ye-Jee Kim, Ho-Su Lee, Jin Hwa Park, Sung Wook Hwang, Dong-Hoon Yang, Byong Duk Ye, Jeong-Sik Byeon, Seung-Jae Myung, Suk-Kyun Yang, Beom-Jun Kim, Sang Hyoung Park
    Clinical Gastroenterology and Hepatology.2022; 20(5): e1022.     CrossRef
  • Change of Computed Tomography-Based Body Composition after Adrenalectomy in Patients with Pheochromocytoma
    Yousun Ko, Heeryoel Jeong, Seungwoo Khang, Jeongjin Lee, Kyung Won Kim, Beom-Jun Kim
    Cancers.2022; 14(8): 1967.     CrossRef
  • Bone and mineral metabolism in patients with primary aldosteronism: A systematic review and meta-analysis
    Anning Wang, Yuhan Wang, Hongzhou Liu, Xiaodong Hu, Jiefei Li, Huaijin Xu, Zhimei Nie, Lingjing Zhang, Zhaohui Lyu
    Frontiers in Endocrinology.2022;[Epub]     CrossRef
  • The Entity of Connshing Syndrome: Primary Aldosteronism with Autonomous Cortisol Secretion
    Mara Carsote
    Diagnostics.2022; 12(11): 2772.     CrossRef
  • Elemental profiling of adrenal adenomas in solid tissue and blood samples by ICP-MS and ICP-OES
    Jovana Jagodić, Branislav Rovčanin, Đurđa Krstić, Ivan Paunović, Vladan Živaljević, Dragan Manojlović, Aleksandar Stojsavljević
    Microchemical Journal.2021; 165: 106194.     CrossRef
  • Aldosterone Inhibits In Vitro Myogenesis by Increasing Intracellular Oxidative Stress via Mineralocorticoid Receptor
    Jin Young Lee, Da Ae Kim, Eunah Choi, Yun Sun Lee, So Jeong Park, Beom-Jun Kim
    Endocrinology and Metabolism.2021; 36(4): 865.     CrossRef
  • 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
    Endocrinology and Metabolism.2020; 35(1): 157.     CrossRef
  • Pheochromocytoma and paraganglioma: An emerging cause of secondary osteoporosis
    Maki Yokomoto-Umakoshi, Hironobu Umakoshi, Tazuru Fukumoto, Yayoi Matsuda, Hiromi Nagata, Masatoshi Ogata, Hisaya Kawate, Takashi Miyazawa, Ryuichi Sakamoto, Yoshihiro Ogawa
    Bone.2020; 133: 115221.     CrossRef
  • Spironolactone reduces biochemical markers of bone turnover in postmenopausal women with primary aldosteronism
    Christian Adolf, Leah T. Braun, Carmina T. Fuss, Stefanie Hahner, Heike Künzel, Laura Handgriff, Lisa Sturm, Daniel A. Heinrich, Holger Schneider, Martin Bidlingmaier, Martin Reincke
    Endocrine.2020; 69(3): 625.     CrossRef
  • Primary Aldosteronism and Bone Metabolism: A Systematic Review and Meta-Analysis
    Shaomin Shi, Chunyan Lu, Haoming Tian, Yan Ren, Tao Chen
    Frontiers in Endocrinology.2020;[Epub]     CrossRef
  • Understanding and managing secondary osteoporosis
    Luciano Colangelo, Federica Biamonte, Jessica Pepe, Cristiana Cipriani, Salvatore Minisola
    Expert Review of Endocrinology & Metabolism.2019; 14(2): 111.     CrossRef
  • Evaluation of bone health in patients with adrenal tumors
    Shobana Athimulam, Irina Bancos
    Current Opinion in Endocrinology, Diabetes & Obesity.2019; 26(3): 125.     CrossRef
Close layer
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
  • 9,616 View
  • 314 Download
  • 98 Web of Science
  • 104 Crossref
AbstractAbstract PDFPubReader   ePub   

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.

Citations

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  • Three-dimensional CT cinematic rendering of adrenal masses: Role in tumor analysis and management
    Taha M. Ahmed, Steven P. Rowe, Elliot K. Fishman, Philippe Soyer, Linda C. Chu
    Diagnostic and Interventional Imaging.2024; 105(1): 5.     CrossRef
  • Bone metastasis manifested 52 years after resection of an apparently benign paraganglioma: A case report
    Run Yu, Martin S Auerbach, Nathan S Honda
    SAGE Open Medical Case Reports.2024;[Epub]     CrossRef
  • A case report on pheochromocytoma mimicking as fulminant myocarditis—a diagnostic challenge
    Yanwei Cheng, Ning Ding, Longan Wang, Lijie Qin
    Frontiers in Cardiovascular Medicine.2024;[Epub]     CrossRef
  • A Case of Pheochromocytoma With Coagulation Necrosis Due to Hypertensive Crisis Aggravated by Contrast-Enhanced CT Scan and Negative 123I-Metaiodobenzylguanidine (MIBG) Scintigraphy
    Ai Kobayashi, Yuki Ishinoda, Asuka Uto, Sho Ogata, Naoki Oshima
    Cureus.2024;[Epub]     CrossRef
  • Management of Pheochromocytomas and Paragangliomas
    Jesse E. Passman, Heather Wachtel
    Surgical Clinics of North America.2024; 104(4): 863.     CrossRef
  • Management of Abdominal Paraganglioma: A Single Center’s Experience
    Enrico Battistella, Luca Pomba, Marica Mirabella, Riccardo Toniato, Giuseppe Opocher, Antonio Toniato
    Medicina.2024; 60(4): 604.     CrossRef
  • Head and Neck Paragangliomas: Overview of Institutional Experience
    Swar N. Vimawala, Alex Z. Graboyes, Bonita Bennett, Maria Bonanni, Aleena Abbasi, Tanaya Oliphant, Michelle Alonso-Basanta, Christopher Rassekh, Debbie Cohen, Jason A. Brant, Yonghong Huan
    Cancers.2024; 16(8): 1523.     CrossRef
  • Evaluating the diagnostic efficacy of whole-body MRI versus 123I-mIBG/131I-mIBG imaging in metastatic pheochromocytoma and paraganglioma
    Hiroshi Mori, Hiroshi Wakabayashi, Shintaro Saito, Kenichi Nakajima, Kotaro Yoshida, Tomo Hiromasa, Seigo Kinuya
    Scientific Reports.2024;[Epub]     CrossRef
  • Inferior Laryngeal Nerve Paraganglioma With Norepinephrine Hypersecretion Diagnosed Shortly After Pregnancy
    David Kishlyansky, Rithvika Ramesh, Olivia Cook, Meera Luthra
    JCEM Case Reports.2024;[Epub]     CrossRef
  • A dosimetric comparison of proton versus photon irradiation for paediatric glomus tumour: a case study
    Gabriel S. Vidal, Julie Arntzen, Salahuddin Ahmad, Christina Henson
    Journal of Radiotherapy in Practice.2023;[Epub]     CrossRef
  • Clinical presentation and diagnostic evaluation of pheochromocytoma: case series and literature review
    Panagiota Anyfanti, Κonstantinos Mastrogiannis, Αntonios Lazaridis, Κonstantinos Tasios, Despoina Vasilakou, Αnastasia Kyriazidou, Fotios Aroutsidis, Olga Pavlidou, Εleni Papoutsopoulou, Athina Tiritidou, Vasileios Kotsis, Αreti Triantafyllou, Ιoannis Zar
    Clinical and Experimental Hypertension.2023;[Epub]     CrossRef
  • Case Report: Octreotide plus CVD chemotherapy for the treatment of multiple metastatic paragangliomas after double resection for functional bladder paraganglioma and urothelial papilloma
    Zilong Wang, Feifan Liu, Chao Li, Huisheng Yuan, Yuzhu Xiang, Chunxiao Wei, Dongyuan Zhu, Muwen Wang
    Frontiers in Oncology.2023;[Epub]     CrossRef
  • Response to Peptide Receptor Radionuclide Therapy in Pheocromocytomas and Paragangliomas: A Systematic Review and Meta-Analysis
    Antonella Lucia Marretta, Alessandro Ottaiano, Domenico Iervolino, Alessandra Bracigliano, Ottavia Clemente, Francesca Di Gennaro, Roberto Tafuto, Mariachiara Santorsola, Secondo Lastoria, Salvatore Tafuto
    Journal of Clinical Medicine.2023; 12(4): 1494.     CrossRef
  • Establishment of reference intervals for plasma metanephrines in seated position measured by LC-MS/MS and assessment of diagnostic performance in pheochromocytoma/paraganglioma
    Yuxin Lin, Meihua Wang, Yingping Cao
    Annals of Clinical Biochemistry: International Journal of Laboratory Medicine.2023; 60(3): 160.     CrossRef
  • Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options
    Michal Eid, Jakub Foukal, Dana Sochorová, Štěpán Tuček, Karel Starý, Zdeněk Kala, Jiří Mayer, Radim Němeček, Jan Trna, Lumír Kunovský
    Cancer Medicine.2023; 12(13): 13942.     CrossRef
  • Editorial: Predictive tools in pheochromocytoma and paraganglioma
    Filippo Ceccato, Ricardo Correa, Masha Livhits, Henrik Falhammar
    Frontiers in Endocrinology.2023;[Epub]     CrossRef
  • 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
  • Pediatric total fractionated metanephrines: age-related reference intervals in spot urine
    Laura ROLI, Agnese VERONESI, Maria C. DE SANTIS, Enrica BARALDI
    Minerva Pediatrics.2023;[Epub]     CrossRef
  • An Overview of Circulating Biomarkers in Neuroendocrine Neoplasms: A Clinical Guide
    Michele Bevere, Francesca Masetto, Maria Elena Carazzolo, Alice Bettega, Anastasios Gkountakos, Aldo Scarpa, Michele Simbolo
    Diagnostics.2023; 13(17): 2820.     CrossRef
  • Pheochromocytoma: a changing perspective and current concepts
    Andreas Kiriakopoulos, Periklis Giannakis, Evangelos Menenakos
    Therapeutic Advances in Endocrinology and Metabolism.2023;[Epub]     CrossRef
  • N-doped carbon Co/CoOx with laccase-like activity for detection of epinephrine
    Junlun Zhu, Qian Cui, Tao Long, Yijia Wang, Wei Wen, Zhengfang Tian, Xiuhua Zhang, Shengfu Wang
    Microchimica Acta.2023;[Epub]     CrossRef
  • Targeted radionuclide therapy in endocrine-related cancers: advances in the last decade
    Taymeyah Al-Toubah, Jonathan Strosberg, Julie Hallanger-Johnson, Ghassan El-Haddad
    Frontiers in Endocrinology.2023;[Epub]     CrossRef
  • An Atypical Presentation of Childhood Paraganglioma with Seizures: A Case Report and Review of the Literature
    Elizabeth Eberechi Oyenusi, Uzoamaka Felicia Nwigbo, Oluwadamilola Moromoke Oladipo, Blessing Ebele Kene-Udemezue, Kasarachi Pauline Akowundu, Khadijah Omobusola Oleolo-Ayodeji, Oluwaseun Adunni Afoke, Funmilayo Oluwatoyin Babatunde, Felix Makinde Alakalo
    Case Reports in Endocrinology.2023; 2023: 1.     CrossRef
  • Comparison of the effectiveness of various methods for determining the level of metanephrines in the diagnosis of pheochromocytomas
    Sh. Sh. Shikhmagomedov, D. V. Rebrova, L. M. Krasnov, E. A. Fedorov, I. K. Chinchuk, R. A. Chernikov, V. F. Rusakov, I. V. Slepstov, E. A. Zgoda
    Problems of Endocrinology.2023; 70(2): 46.     CrossRef
  • Addition of 3‐methoxytyramine or chromogranin A to plasma free metanephrines as the initial test for pheochromocytoma and paraganglioma: Which is the best diagnostic strategy
    Lu Liu, Wenchun Xie, Zhijing Song, Tianyu Wang, Xuesong Li, Ying Gao, Yan Li, Junqing Zhang, Xiaohui Guo
    Clinical Endocrinology.2022; 96(2): 132.     CrossRef
  • Hereditary and Sporadic Pheochromocytoma: Comparison of Imaging, Clinical, and Laboratory Features
    Ryan Chung, Aileen O'Shea, Ann T. Sweeney, Nathaniel D. Mercaldo, Shaunagh McDermott, Michael A. Blake
    American Journal of Roentgenology.2022; 219(1): 97.     CrossRef
  • Nonvestibular Schwannomas of the Head and Neck
    A.H. Duran, M.T. Starc, A. Khorsandi
    Neurographics.2022; 12(1): 25.     CrossRef
  • Preoperative Alpha-Blocker Therapy in Patients with Missed Preoperative Diagnosis of Extra-Adrenal Retroperitoneal Paraganglioma Undergoing Resection: A Retrospective Study of 167 Cases at a Single Center
    Yi Liu, Xinye Jin, Jie Gao, Shan Jiang, Lei Liu, Jingsheng Lou, Bo Wang, Hong Zhang, Qiang Fu
    Neuroendocrinology.2022; 112(5): 457.     CrossRef
  • Metastatic pheochromocytomas and paragangliomas: where are we?
    Natalie Prinzi, Francesca Corti, Martina Torchio, Monica Niger, Maria Antista, Filippo Pagani, Teresa Beninato, Iolanda Pulice, Roberta Elisa Rossi, Jorgelina Coppa, Tommaso Cascella, Luca Giacomelli, Maria Di Bartolomeo, Massimo Milione, Filippo de Braud
    Tumori Journal.2022; 108(6): 526.     CrossRef
  • Feocromocitoma adrenal. Claves para el diagnóstico radiológico
    M.A. Corral de la Calle, J. Encinas de la Iglesia, G.C. Fernández-Pérez, M. Repollés Cobaleda, A. Fraino
    Radiología.2022; 64(4): 348.     CrossRef
  • Is the Adrenal Incidentaloma Functionally Active? An Approach-To-The-Patient-Based Review
    Stella Bernardi, Veronica Calabrò, Marco Cavallaro, Sara Lovriha, Rita Eramo, Bruno Fabris, Nicolò de Manzini, Chiara Dobrinja
    Journal of Clinical Medicine.2022; 11(14): 4064.     CrossRef
  • Systematic Review: Incidence of Pheochromocytoma and Paraganglioma Over 70 Years
    Abdul Rahman Al Subhi, Veronica Boyle, Marianne S Elston
    Journal of the Endocrine Society.2022;[Epub]     CrossRef
  • Management of Patients with Treatment of Pheochromocytoma: A Critical Appraisal
    Florence Bihain, Claire Nomine-Criqui, Philippe Guerci, Stephane Gasman, Marc Klein, Laurent Brunaud
    Cancers.2022; 14(16): 3845.     CrossRef
  • Adrenal pheochromocytoma: Keys to radiologic diagnosis
    M.A. Corral de la Calle, J. Encinas de la Iglesia, G.C. Fernández-Pérez, M. Repollés Cobaleda, A. Fraino
    Radiología (English Edition).2022; 64(4): 348.     CrossRef
  • Preoperative blood pressure targets and effect on hemodynamics in pheochromocytoma and paraganglioma
    Randi Ugleholdt, Åse Krogh Rasmussen, Pernille A H Haderslev, Bjarne Kromann-Andersen, Claus Larsen Feltoft
    Endocrine Connections.2022;[Epub]     CrossRef
  • Liquid chromatography–tandem mass spectrometry for clinical diagnostics
    Stefani N. Thomas, Deborah French, Paul J. Jannetto, Brian A. Rappold, William A. Clarke
    Nature Reviews Methods Primers.2022;[Epub]     CrossRef
  • Acute Stress Cardiomyopathy: Heart of pheochromocytoma
    Erika Cornu, Justina Motiejunaite, Ines Belmihoub, Emmanuelle Vidal-Petiot, Mariana Mirabel, Laurence Amar
    Annales d'Endocrinologie.2021; 82(3-4): 201.     CrossRef
  • Endocrine causes of heart failure: A clinical primer for cardiologists
    Saurav Khatiwada, Hiya Boro, Faraz Ahmed Farooqui, Sarah Alam
    Indian Heart Journal.2021; 73(1): 14.     CrossRef
  • Recurrence Rate of Sporadic Pheochromocytomas After Curative Adrenalectomy: A Systematic Review and Meta-analysis
    Isabelle Holscher, Tijs J van den Berg, Koen M A Dreijerink, Anton F Engelsman, Els J M Nieveen van Dijkum
    The Journal of Clinical Endocrinology & Metabolism.2021; 106(2): 588.     CrossRef
  • Overview of Monogenic Forms of Hypertension Combined With Hypokalemia
    Yi-Ting Lu, Peng Fan, Di Zhang, Ying Zhang, Xu Meng, Qiong-Yu Zhang, Lin Zhao, Kun-Qi Yang, Xian-Liang Zhou
    Frontiers in Pediatrics.2021;[Epub]     CrossRef
  • Management of the patient with incidental bilateral adrenal nodules
    Ann T. Sweeney, Padmavathi Srivoleti, Michael A. Blake
    Journal of Clinical and Translational Endocrinology: Case Reports.2021; 20: 100082.     CrossRef
  • Arterial thrombosis and intracardiac thrombus as the initial presentation of a recurrent paraganglioma: case report and review of the literature
    Dania Lizet Quintanilla-Flores, Jesús Zacarías Villarreal-Pérez, Claudia Analy Vélez-Viveros, Paola Portillo-Sánchez, Adriana Josefina Ortíz-Morales, José Gerardo González-González
    Archives of Endocrinology and Metabolism.2021;[Epub]     CrossRef
  • La gestione perioperatoria delle masse surrenaliche
    Filippo Ceccato, Carla Scaroni
    L'Endocrinologo.2021; 22(2): 127.     CrossRef
  • Verifying Clinically Derived Reference Intervals for Daily Excretion Rates of Fractionated Metanephrines Using Modern Indirect Reference Interval Models
    Kelly Doyle, Elizabeth L Frank
    American Journal of Clinical Pathology.2021; 156(4): 691.     CrossRef
  • Identification of vital prognostic genes related to tumor microenvironment in pheochromocytoma and paraganglioma based on weighted gene co-expression network analysis
    Chun-Xian Chen, Dong-Ning Chen, Xiong-Lin Sun, Zhi-Bin Ke, Fei Lin, Hang Chen, Xuan Tao, Fei Huang, Yong Wei, Ning Xu
    Aging.2021; 13(7): 9976.     CrossRef
  • Pheochromocytoma in a 49-year-old woman presenting with acute myocardial infarction: A case report
    Hao-Yu Wu, Yi-Wei Cao, Tian-Jiao Gao, Jian-Li Fu, Lei Liang
    World Journal of Clinical Cases.2021; 9(15): 3752.     CrossRef
  • Mutation Profile of Aggressive Pheochromocytoma and Paraganglioma with Comparison of TCGA Data
    Yun Mi Choi, Jinyeong Lim, Min Ji Jeon, Yu-Mi Lee, Tae-Yon Sung, Eun-Gyoung Hong, Ji-Young Lee, Se Jin Jang, Won Gu Kim, Dong Eun Song, Sung-Min Chun
    Cancers.2021; 13(10): 2389.     CrossRef
  • Hypertension in Pheochromocytoma and Paraganglioma: Evaluation and Management in Pediatric Patients
    Meredith L. Seamon, Ikuyo Yamaguchi
    Current Hypertension Reports.2021;[Epub]     CrossRef
  • Endocrine hypertension secondary to adrenal tumors: clinical course and predictive factors of clinical remission
    Uriel Clemente-Gutiérrez, Rafael H. Pérez-Soto, Juan D. Hernández-Acevedo, Nicole M. Iñiguez-Ariza, Enrique Casanueva-Pérez, Juan Pablo Pantoja-Millán, Mauricio Sierra-Salazar, Miguel F. Herrera, David Velázquez-Fernández
    Langenbeck's Archives of Surgery.2021; 406(6): 2027.     CrossRef
  • A multidisciplinary approach to the management of adrenal incidentaloma
    Giacomo Voltan, Marco Boscaro, Decio Armanini, Carla Scaroni, Filippo Ceccato
    Expert Review of Endocrinology & Metabolism.2021; 16(4): 201.     CrossRef
  • Neuroendocrine and Adrenal Tumors, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology
    Manisha H. Shah, Whitney S. Goldner, Al B. Benson, Emily Bergsland, Lawrence S. Blaszkowsky, Pamela Brock, Jennifer Chan, Satya Das, Paxton V. Dickson, Paul Fanta, Thomas Giordano, Thorvardur R. Halfdanarson, Daniel Halperin, Jin He, Anthony Heaney, Marti
    Journal of the National Comprehensive Cancer Network.2021; 19(7): 839.     CrossRef
  • Plasma Metabolome Profiling for the Diagnosis of Catecholamine Producing Tumors
    Juliane März, Max Kurlbaum, Oisin Roche-Lancaster, Timo Deutschbein, Mirko Peitzsch, Cornelia Prehn, Dirk Weismann, Mercedes Robledo, Jerzy Adamski, Martin Fassnacht, Meik Kunz, Matthias Kroiss
    Frontiers in Endocrinology.2021;[Epub]     CrossRef
  • Bilateral carotid paraganglioma: A case report
    Jean Michel Nzisabira, Eugène Ndirahisha, Sébastien Manirakiza, Linda Samantha Nzisabira, Patrice Barasukana, Léopold Nzisabira
    International Journal of Case Reports and Images.2021; 12: 1.     CrossRef
  • An Overview on Pheochromocytoma Diagnosis and Management Approach, Review Article
    Yazeed Ali Aljabri, Faris Sami Alghamdi, Khaled Ahmed Almehmadi, Abdulrahman Abubaker Babkoor, Abdulhafiz Muhammad Bahalaq, Anwar Aziz Althaqfi, Abdullah Ghazi Alharbi, Rayan Saud Alhazmi, Ahmad Ali Almalki
    World Journal of Environmental Biosciences.2021; 10(3): 18.     CrossRef
  • Bladder paraganglioma: CT and MR imaging characteristics in 16 patients
    Jing Zhang, Xu Bai, Jing Yuan, Xiaojing Zhang, Wei Xu, Huiyi Ye, Haiyi Wang
    Radiology and Oncology.2021; 56(1): 46.     CrossRef
  • Laparoscopic Partial Resection of Retroperitoneal Extramedullary Plasmacytoma
    Andrzej Gasz, Jan Danko, Maros Straka, Lazo Ilic, José M. Ramia
    Cirugía Española (English Edition).2020; 98(5): 299.     CrossRef
  • 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
    Endocrinology and Metabolism.2020; 35(1): 157.     CrossRef
  • Predictors of recurrence of pheochromocytoma and paraganglioma: a multicenter study in Piedmont, Italy
    Mirko Parasiliti-Caprino, Barbara Lucatello, Chiara Lopez, Jacopo Burrello, Francesca Maletta, Marinella Mistrangelo, Enrica Migliore, Francesco Tassone, Antonio La Grotta, Anna Pia, Giuseppe Reimondo, Roberta Giordano, Giuseppe Giraudo, Alessandro Pioves
    Hypertension Research.2020; 43(6): 500.     CrossRef
  • A Brief Overview of the Epidemiology of Pheochromocytoma and Paraganglioma in Korea
    Yun Mi Choi
    Endocrinology and Metabolism.2020; 35(1): 95.     CrossRef
  • Pheochromocytoma and paraganglioma—an update on diagnosis, evaluation, and management
    Amrish Jain, Rossana Baracco, Gaurav Kapur
    Pediatric Nephrology.2020; 35(4): 581.     CrossRef
  • Short-term stability of free metanephrines in plasma and whole blood
    Elisa Danese, Martina Montagnana, Claudio Brentegani, Giuseppe Lippi
    Clinical Chemistry and Laboratory Medicine (CCLM).2020; 58(5): 753.     CrossRef
  • Meta-analysis of α-blockade versus no blockade before adrenalectomy for phaeochromocytoma
    S Schimmack, J Kaiser, P Probst, E Kalkum, M K Diener, O Strobel
    British Journal of Surgery.2020; 107(2): e102.     CrossRef
  • In Matrix Derivatization Combined with LC-MS/MS Results in Ultrasensitive Quantification of Plasma Free Metanephrines and Catecholamines
    Martijn van Faassen, Rainer Bischoff, Karin Eijkelenkamp, Wilhelmina H. A. de Jong, Claude P. van der Ley, Ido P. Kema
    Analytical Chemistry.2020; 92(13): 9072.     CrossRef
  • Resección parcial laparoscópica de plasmocitoma extramedular retroperitoneal
    Andrzej Gasz, Jan Danko, Maros Straka, Lazo Ilic, José M. Ramia
    Cirugía Española.2020; 98(5): 299.     CrossRef
  • Bladder Preservation for Patients With Bladder Paragangliomas: Case Series and Review of the Literature
    Elizabeth Y. Wang, Jamie S. Pak, Renu K. Virk, Christopher B. Anderson, Kelly A. Healy, James A. Lee, Mitchell C. Benson, James M. McKiernan
    Urology.2020; 143: 194.     CrossRef
  • Decision-Making in Artificial Intelligence: Is It Always Correct?
    Hun-Sung Kim
    Journal of Korean Medical Science.2020;[Epub]     CrossRef
  • Pheochromocytoma and Paraganglioma
    James K. Moon, Peter Mattei
    Seminars in Pediatric Surgery.2020; 29(3): 150926.     CrossRef
  • 52-Year-Old Woman With Fever, Diaphoresis, and Abdominal Pain
    Thomas J. Breen, Brenden S. Ingraham, Jacob C. Jentzer
    Mayo Clinic Proceedings.2020; 95(7): e69.     CrossRef
  • Diagnostic des phéochromocytomes et paragangliomes
    F. Castinetti, A. Barlier, F. Sebag, D. Taieb
    Oncologie.2020;[Epub]     CrossRef
  • Glucocorticoid Excess in Patients with Pheochromocytoma Compared with Paraganglioma and Other Forms of Hypertension
    Georgiana Constantinescu, Katharina Langton, Catleen Conrad, Laurence Amar, Guillaume Assié, Anne-Paule Gimenez-Roqueplo, Anne Blanchard, Casper K Larsen, Paolo Mulatero, Tracy Ann Williams, Aleksander Prejbisz, Martin Fassnacht, Stefan Bornstein, Filippo
    The Journal of Clinical Endocrinology & Metabolism.2020; 105(9): e3374.     CrossRef
  • PDK1 Inhibitor GSK-470 Exhibits Potent Anticancer Activity in a Pheochromocytoma PC12 Cell Tumor Model via Akt/mTOR Pathway
    Xiaohua Zhang, Shan Zhong
    Anti-Cancer Agents in Medicinal Chemistry.2020; 20(7): 828.     CrossRef
  • ‘Case of the month’ from UConn Health, Farmington, CT, USA: management of a giant paraganglioma
    Vikram Lyall, Michael E. Goltzman, Mina L. Boutrous, Ravali Nallu, Peter C. Albertsen, Beatriz E. Tendler, Benjamin T. Ristau
    BJU International.2020; 126(5): 551.     CrossRef
  • Perioperative management of pheocromocytoma/ paraganglioma: a comprehensive review
    Alejandro Román-González, Huber Padilla-Zambrano, Luis Felipe Vásquez Jimenez
    Colombian Journal of Anesthesiology.2020;[Epub]     CrossRef
  • Optimizing Genetic Workup in Pheochromocytoma and Paraganglioma by Integrating Diagnostic and Research Approaches
    Laura Gieldon, Doreen William, Karl Hackmann, Winnie Jahn, Arne Jahn, Johannes Wagner, Andreas Rump, Nicole Bechmann, Svenja Nölting, Thomas Knösel, Volker Gudziol, Georgiana Constantinescu, Jimmy Masjkur, Felix Beuschlein, Henri JLM Timmers, Letizia Canu
    Cancers.2019; 11(6): 809.     CrossRef
  • Blood Pressure Profile, Catecholamine Phenotype, and Target Organ Damage in Pheochromocytoma/Paraganglioma
    Ondřej Petrák, Ján Rosa, Robert Holaj, Branislav Štrauch, Zuzana Krátká, Jan Kvasnička, Judita Klímová, Petr Waldauf, Barbora Hamplová, Alice Markvartová, Květoslav Novák, David Michalský, Jiří Widimský, Tomáš Zelinka
    The Journal of Clinical Endocrinology & Metabolism.2019; 104(11): 5170.     CrossRef
  • Expression of PDK1 in malignant pheochromocytoma as a new promising potential therapeutic target
    X. Zhang, Z. Yu
    Clinical and Translational Oncology.2019; 21(10): 1312.     CrossRef
  • Prospective evaluation of 68Ga-DOTANOC positron emission tomography/computed tomography and 131I-meta-iodobenzylguanidine single-photon emission computed tomography/computed tomography in extra-adrenal paragangliomas, including uncommon primary sites and
    Saurabh Arora, Rakesh Kumar, Averilicia Passah, Madhavi Tripathi, Sandeep Agarwala, Rajesh Khadgawat, Chandrasekhar Bal
    Nuclear Medicine Communications.2019; 40(12): 1230.     CrossRef
  • Combinatorial Inhibition of mTORC2 and Hsp90 Leads to a Distinctly Effective Therapeutic Strategy in Malignant Pheochromocytoma
    Xiaohua Zhang, Fengbin Gao, Shan Zhong
    Current Cancer Drug Targets.2019; 19(9): 698.     CrossRef
  • Ischémie aiguë du membre inférieur et acidocétose diabétique révélant un phéochromocytome
    Kamal Ahsayan, Amine Ghalem, Mohammed Bouziane, Noha El Ouafi, Brahim Housni
    Anesthésie & Réanimation.2019; 5(4): 337.     CrossRef
  • Enfoque diagnóstico de feocromocitomas y paragangliomas
    R.M. Gómez, M. Hernaiz, V. de Miguel, L.S. Aparicio, M.J. Marín, S. Lupi, M. Barontini
    Hipertensión y Riesgo Vascular.2019; 36(1): 34.     CrossRef
  • Phéochromocytome et paragangliome
    E. Cornu, I. Belmihoub, N. Burnichon, C. Grataloup, F. Zinzindohoué, S. Baron, E. Billaud, M. Azizi, A.-P. Gimenez-Roqueplo, L. Amar
    La Revue de Médecine Interne.2019; 40(11): 733.     CrossRef
  • Rendimiento de las metanefrinas libres plasmáticas en el diagnóstico de los feocromocitomas y paragangliomas en la población asturiana
    Eduardo Martínez-Morillo, Nuria Valdés Gallego, Edwin Eguia Ángeles, Juan Carlos Fernández Fernández, Belén Prieto García, Francisco V. Álvarez
    Endocrinología, Diabetes y Nutrición.2019; 66(5): 312.     CrossRef
  • The Identification of Differentially Expressed Genes Showing Aberrant Methylation Patterns in Pheochromocytoma by Integrated Bioinformatics Analysis
    Dengqiang Lin, Jinglai Lin, Xiaoxia Li, Jianping Zhang, Peng Lai, Zhifeng Mao, Li Zhang, Yu Zhu, Yujun Liu
    Frontiers in Genetics.2019;[Epub]     CrossRef
  • Pharmacological and analytical interference in hormone assays for diagnosis of adrenal incidentaloma
    Antoine-Guy Lopez, François Fraissinet, Herve Lefebvre, Valéry Brunel, Frédéric Ziegler
    Annales d'Endocrinologie.2019; 80(4): 250.     CrossRef
  • Analysis of Short-term Blood Pressure Variability in Pheochromocytoma/Paraganglioma Patients
    Valeria Bisogni, Luigi Petramala, Gaia Oliviero, Maria Bonvicini, Martina Mezzadri, Federica Olmati, Antonio Concistrè, Vincenza Saracino, Monia Celi, Gianfranco Tonnarini, Gino Iannucci, Giorgio De Toma, Antonio Ciardi, Giuseppe La Torre, Claudio Letizia
    Cancers.2019; 11(5): 658.     CrossRef
  • Imaging Features of Succinate Dehydrogenase–deficient Pheochromocytoma-Paraganglioma Syndromes
    Samuel Joseph Withey, Stephen Perrio, Dimitra Christodoulou, Louise Izatt, Paul Carroll, Anand Velusamy, Rupert Obholzer, Valerie Lewington, Audrey Eleanor Therese Jacques
    RadioGraphics.2019; 39(5): 1393.     CrossRef
  • A Clinical Efficacy of PRRT in Patients with Advanced, Nonresectable, Paraganglioma-Pheochromocytoma, Related to SDHx Gene Mutation
    Agnieszka Kolasinska-Ćwikła, Mariola Pęczkowska, Jarosław Ćwikła, Ilona Michałowska, Jakub Pałucki, Lisa Bodei, Anna Lewczuk-Myślicka, Andrzej Januszewicz
    Journal of Clinical Medicine.2019; 8(7): 952.     CrossRef
  • A Branching Algorithm
    Elisabeth Pedersen, Gurpreet Dhaliwal, Ashwin Gupta, Tobias Else, Robert Chang, Nathan Houchens
    Journal of Hospital Medicine.2019; 14(11): 707.     CrossRef
  • Liquid chromatography tandem mass spectrometry for plasma metadrenalines
    S.L. Davies, A.S. Davison
    Clinica Chimica Acta.2019; 495: 512.     CrossRef
  • Addressing delays in the diagnosis of pheochromocytoma/paraganglioma
    Ioannis Ilias, Costas Thomopoulos
    Expert Review of Endocrinology & Metabolism.2019; 14(5): 359.     CrossRef
  • Performance of plasma free metanephrines in diagnosis of pheochromocytomas and paragangliomas in the population of Asturias
    Eduardo Martínez-Morillo, Nuria Valdés Gallego, Edwin Eguia Ángeles, Juan Carlos Fernández Fernández, Belén Prieto García, Francisco V. Álvarez
    Endocrinología, Diabetes y Nutrición (English ed.).2019; 66(5): 312.     CrossRef
  • A Case of Sudden Death: Subarachnoid Hemorrhage, Pheochromocytoma, Berry Aneurysm
    Ana B. Arevalo, Jacxelyn Moran, Stephen Zink, Binny Khandakar
    Case Reports in Pathology.2019; 2019: 1.     CrossRef
  • Pheochromocytoma
    Frederick-Anthony Farrugia, Anestis Charalampopoulos
    Endocrine Regulations.2019; 53(3): 191.     CrossRef
  • What's New in Endocrinology: The Chromaffin Cell
    Lee E. Eiden, Sunny Zhihong Jiang
    Frontiers in Endocrinology.2018;[Epub]     CrossRef
  • Pheochromocytoma: A Genetic And Diagnostic Update
    Leilani B. Mercado-Asis, Katherine I. Wolf, Ivana Jochmanova, David Taïeb
    Endocrine Practice.2018; 24(1): 78.     CrossRef
  • Retroperitoneal Paraganglioma Involving the Renal Hilum: A Case Report and Literature Review
    Peter Yincheng Cai, Ron Golan, Brent Yanke
    Urology.2018; 122: 24.     CrossRef
  • Fluorescent ternary complexes of some biogenic amines and their metabolites with europium and oxytetracycline for applications in the chemical analysis
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    Mendeleev Communications.2018; 28(5): 553.     CrossRef
  • Phéochromocytomes et paragangliomes génétiquement déterminés: quelle conduite à tenir chez les apparentés ?
    Marie Vermalle, Antoine Tabarin, Frederic Castinetti
    Annales d'Endocrinologie.2018; 79: S10.     CrossRef
  • Exosomal double-stranded DNA as a biomarker for the diagnosis and preoperative assessment of pheochromocytoma and paraganglioma
    Liang Wang, Ying Li, Xin Guan, Jingyuan Zhao, Liming Shen, Jing Liu
    Molecular Cancer.2018;[Epub]     CrossRef
  • Strain-specific metastatic phenotypes in pheochromocytoma allograft mice
    Martin Ullrich, Josephine Liers, Mirko Peitzsch, Anja Feldmann, Ralf Bergmann, Ulrich Sommer, Susan Richter, Stefan R Bornstein, Michael Bachmann, Graeme Eisenhofer, Christian G Ziegler, Jens Pietzsch
    Endocrine-Related Cancer.2018; 25(12): 993.     CrossRef
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    High Blood Pressure & Cardiovascular Prevention.2018; 25(4): 345.     CrossRef
  • Amperometric Determination of Catecholamines by Enzymatic Biosensors in Flow Systems
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    Pharmacological Reviews.2018; 70(3): 549.     CrossRef
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    Internal Medicine.2018; 15(2): 31.     CrossRef
Close layer
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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AbstractAbstract PDFPubReader   
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.

Citations

Citations to this article as recorded by  
  • European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European Network for the Study of Adrenal Tumors
    Martin Fassnacht, Stylianos Tsagarakis, Massimo Terzolo, Antoine Tabarin, Anju Sahdev, John Newell-Price, Iris Pelsma, Ljiljana Marina, Kerstin Lorenz, Irina Bancos, Wiebke Arlt, Olaf M Dekkers
    European Journal of Endocrinology.2023; 189(1): G1.     CrossRef
  • Diagnostic Accuracy of CT Texture Analysis in Adrenal Masses: A Systematic Review
    Filippo Crimì, Emilio Quaia, Giulio Cabrelle, Chiara Zanon, Alessia Pepe, Daniela Regazzo, Irene Tizianel, Carla Scaroni, Filippo Ceccato
    International Journal of Molecular Sciences.2022; 23(2): 637.     CrossRef
  • Susceptibility and characteristics of infections in patients with glucocorticoid excess or insufficiency: the ICARO tool
    Marianna Minnetti, Valeria Hasenmajer, Emilia Sbardella, Francesco Angelini, Chiara Simeoli, Nicola Di Paola, Alessia Cozzolino, Claudia Pivonello, Dario De Alcubierre, Sabrina Chiloiro, Roberto Baldelli, Laura De Marinis, Rosario Pivonello, Riccardo Pofi
    European Journal of Endocrinology.2022; 187(5): 719.     CrossRef
  • Approach to large adrenal tumors
    Urszula Ambroziak
    Current Opinion in Endocrinology, Diabetes & Obesity.2021; 28(3): 271.     CrossRef
  • Frequently asked questions and answers (if any) in patients with adrenal incidentaloma
    F. Ceccato, M. Barbot, C. Scaroni, M. Boscaro
    Journal of Endocrinological Investigation.2021; 44(12): 2749.     CrossRef
  • Draft of the clinical practice guidelines “Adrenal incidentaloma”
    D. G. Beltsevich, E. A. Troshina, G. A. Melnichenko, N. M. Platonova, D. O. Ladygina, A. Chevais
    Endocrine Surgery.2021; 15(1): 4.     CrossRef
  • Attenuation Value in Adrenal Incidentalomas: A Longitudinal Study
    Filippo Ceccato, Irene Tizianel, Giacomo Voltan, Gianmarco Maggetto, Isabella Merante Boschin, Emilio Quaia, Filippo Crimì, Carla Scaroni
    Frontiers in Endocrinology.2021;[Epub]     CrossRef
  • Guía práctica sobre la evaluación inicial, seguimiento y tratamiento de los incidentalomas adrenales. Grupo de patología adrenal de la Sociedad Española de Endocrinología y Nutrición
    Marta Araujo-Castro, Marta Iturregui Guevara, María Calatayud Gutiérrez, Paola Parra Ramírez, Paola Gracia Gimeno, Felicia Alexandra Hanzu, Cristina Lamas Oliveira
    Endocrinología, Diabetes y Nutrición.2020; 67(6): 408.     CrossRef
  • Practical guide on the initial evaluation, follow-up, and treatment of adrenal incidentalomas. Adrenal Diseases Group of the Spanish Society of Endocrinology and Nutrition
    Marta Araujo-Castro, Marta Iturregui Guevara, María Calatayud Gutiérrez, Paola Parra Ramírez, Paola Gracia Gimeno, Felicia Alexandra Hanzu, Cristina Lamas Oliveira
    Endocrinología, Diabetes y Nutrición (English ed.).2020; 67(6): 408.     CrossRef
  • Endocrine Causes of Hypertension
    Taniya de Silva, Gina Cosentino, Suneeta Ganji, Alejandra Riera-Gonzalez, Daniel S. Hsia
    Current Hypertension Reports.2020;[Epub]     CrossRef
  • Adrenocortical carcinoma: presentation and outcome of a contemporary patient series
    Iiro Kostiainen, Liisa Hakaste, Pekka Kejo, Helka Parviainen, Tiina Laine, Eliisa Löyttyniemi, Mirkka Pennanen, Johanna Arola, Caj Haglund, Ilkka Heiskanen, Camilla Schalin-Jäntti
    Endocrine.2019; 65(1): 166.     CrossRef
  • CT Characteristics of Pheochromocytoma: Relevance for the Evaluation of Adrenal Incidentaloma
    Letizia Canu, Janna A W Van Hemert, Michiel N Kerstens, Robert P Hartman, Aakanksha Khanna, Ivana Kraljevic, Darko Kastelan, Corin Badiu, Urszula Ambroziak, Antoine Tabarin, Magalie Haissaguerre, Edward Buitenwerf, Anneke Visser, Massimo Mannelli, Wiebke
    The Journal of Clinical Endocrinology & Metabolism.2019; 104(2): 312.     CrossRef
  • The Evaluation of Incidentally Discovered Adrenal Masses
    Anand Vaidya, Amir Hamrahian, Irina Bancos, Maria Fleseriu, Hans K. Ghayee
    Endocrine Practice.2019; 25(2): 178.     CrossRef
  • Evaluation of quantitative parameters for distinguishing pheochromocytoma from other adrenal tumors
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    Hypertension Research.2018; 41(3): 165.     CrossRef
  • Long term follow-up of non functional adrenal incidentalomas in size: a single center experience
    Güven Barış Cansu, Bengür Taşkıran, Eylem Bahadır
    Ortadoğu Tıp Dergisi.2018; 10(4): 506.     CrossRef
  • Unenhanced CT imaging is highly sensitive to exclude pheochromocytoma: a multicenter study
    Edward Buitenwerf, Tijmen Korteweg, Anneke Visser, Charlotte M S C Haag, Richard A Feelders, Henri J L M Timmers, Letizia Canu, Harm R Haak, Peter H L T Bisschop, Elisabeth M W Eekhoff, Eleonora P M Corssmit, Nanda C Krak, Elise Rasenberg, Janneke van den
    European Journal of Endocrinology.2018; 178(5): 431.     CrossRef
  • The impact of an adrenal incidentaloma algorithm on the evaluation of adrenal nodules
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    Journal of Clinical & Translational Endocrinology.2018; 13: 39.     CrossRef
  • Adrenal incidentaloma: differential diagnosis and management strategies
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    Minerva Endocrinologica.2018;[Epub]     CrossRef
  • Adrenal Imaging
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  • Update on the risks of benign adrenocortical incidentalomas
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    Current Opinion in Endocrinology, Diabetes & Obesity.2017; 24(3): 193.     CrossRef
  • Management Scheme for Adrenal Incidentalomas: Who Gets What?
    Glenda G. Callender, Robert Udelsman
    Current Surgery Reports.2016;[Epub]     CrossRef
  • Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors
    Martin Fassnacht, Wiebke Arlt, Irina Bancos, Henning Dralle, John Newell-Price, Anju Sahdev, Antoine Tabarin, Massimo Terzolo, Stylianos Tsagarakis, Olaf M Dekkers
    European Journal of Endocrinology.2016; 175(2): G1.     CrossRef
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