Endocrinology and Metabolism

Original Article

Preoperative Localization and Intraoperative Parathyroid Hormone Assay in Korean Patients with Primary Hyperparathyroidism: Eirie Cho, Jung Mi Chang, Seok Young Yoon, Gil Tae Lee, Yun Hyi Ku, Hong Il Kim, Myung-Chul Lee, Guk Haeng Lee, Min Joo Kim; Endocrinol Metab. 2014;29(4):464-469. Published online December 29, 2014; DOI: https://doi.org/10.3803/EnM.2014.29.4.464

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Background

The intraoperative parathyroid hormone (IOPTH) assay is widely used in patients with primary hyperparathyroidism (PHPT). We investigated the usefulness of the IOPTH assay in Korean patients with PHPT.

Methods

We retrospectively reviewed the data of 33 patients with PHPT who underwent parathyroidectomy. Neck ultrasonography (US) and ^99mTc-sestamibi scintigraphy (MIBI scan) were performed preoperatively and IOPTH assays were conducted.

Results

The sensitivity of neck US and MIBI scans were 91% and 94%, respectively. A 50% decrease in parathyroid hormone (PTH) levels 10 minutes after excision of the parathyroid gland was obtained in 91% (30/33) of patients and operative success was achieved in 97% (32/33) of patients. The IOPTH assay was 91% true-positive, 3% true-negative, 0% false-positive, and 6% false-negative. The overall accuracy of the IOPTH assay was 94%. In five cases with discordant neck US and MIBI scan results, a sufficient decrease in IOPTH levels helped the surgeon confirm the complete excision of the parathyroid gland with no additional neck exploration.

Conclusion

The IOPTH assay is an accurate tool for localizing hyperfunctioning parathyroid glands and is helpful for evaluating cases with discordant neck US and MIBI scan results.

Citations

Citations to this article as recorded by

Single-Center Experience of Parathyroidectomy Using Intraoperative Parathyroid Hormone Monitoring
Seong Hoon Kim, Si Yeon Lee, Eun Ah Min, Young Mi Hwang, Yun Suk Choi, Jin Wook Yi
Medicina.2022; 58(10): 1464. CrossRef
The natural history and hip geometric changes of primary hyperparathyroidism without parathyroid surgery
Kyong Yeun Jung, A. Ram Hong, Dong Hwa Lee, Jung Hee Kim, Kyoung Min Kim, Chan Soo Shin, Seong Yeon Kim, Sang Wan Kim
Journal of Bone and Mineral Metabolism.2017; 35(3): 278. CrossRef
The utility of the radionuclide probe in parathyroidectomy for primary hyperparathyroidism
MS Lim, M Jinih, CH Ngai, NM Foley, HP Redmond
The Annals of The Royal College of Surgeons of England.2017; 99(5): 369. CrossRef
Articles in 'Endocrinology and Metabolism' in 2014
Won-Young Lee
Endocrinology and Metabolism.2015; 30(1): 47. CrossRef

Case Report

Thyroid
Medullary Thyroid Carcinoma with Ectopic Adrenocorticotropic Hormone Syndrome: Hong Seok Choi, Min Joo Kim, Chae Ho Moon, Jong Ho Yoon, Ha Ra Ku, Geon Wook Kang, Im Il Na, Seung-Sook Lee, Byung-Chul Lee, Young Joo Park, Hong Il Kim, Yun Hyi Ku; Endocrinol Metab. 2014;29(1):96-100. Published online March 14, 2014; DOI: https://doi.org/10.3803/EnM.2014.29.1.96

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Abstract PDF PubReader

Ectopic adrenocorticotropic hormone (ACTH) syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC) is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. He was diagnosed with MTC 14 years ago and underwent total thyroidectomy, cervical lymph node dissection and a series of metastasectomies. MTC was confirmed by the pathological examination of the thyroid and metastatic mediastinal lymph node tissues. Two years after his last surgery, he developed Cushingoid features, such as moon face and central obesity, accompanied by uncontrolled hypertension and new-onset diabetes. The laboratory results were compatible with ectopic ACTH syndrome. A bilateral adrenalectomy improved the clinical and laboratory findings that were associated with Cushing syndrome. This is the first confirmed case of ectopic ACTH syndrome caused by MTC in Korea.

Citations

Citations to this article as recorded by

Medullary Thyroid Cancer with Ectopic Cushing's Syndrome: A Case Report and Systematic Review of Detailed Cases from the Literature
Andrea Corsello, Vittoria Ramunno, Pietro Locantore, Giovanni Pacini, Esther Diana Rossi, Francesco Torino, Alfredo Pontecorvi, Carmela De Crea, Rosa Maria Paragliola, Marco Raffaelli, Salvatore Maria Corsello
Thyroid.2022; 32(11): 1281. CrossRef
Mucosal Neuroma Cues for Endocrine Emergency Treatment
Gyu Gang Choi, Hwan Jin Lee, Hyo Jin Han, Young Beom Jeong, Heung Bum Lee, Ji Hyun Park
Endocrinology and Metabolism.2021; 36(6): 1312. CrossRef
Articles in 'Endocrinology and Metabolism' in 2014
Won-Young Lee
Endocrinology and Metabolism.2015; 30(1): 47. CrossRef
Rapid response of hypercortisolism to vandetanib treatment in a patient with advanced medullary thyroid cancer and ectopic Cushing syndrome
Fabián Pitoia, Fernanda Bueno, Angélica Schmidt, Sabrina Lucas, Graciela Cross
Archives of Endocrinology and Metabolism.2015; 59(4): 343. CrossRef

Original Article

Obesity and Metabolism
A Novel Mutation in the Von Hippel-Lindau Tumor Suppressor Gene Identified in a Patient Presenting with Gestational Diabetes Mellitus: Yun Hyi Ku, Chang Ho Ahn, Chan-Hyeon Jung, Jie Eun Lee, Lee-Kyung Kim, Soo Heon Kwak, Hye Seung Jung, Kyong Soo Park, Young Min Cho; Endocrinol Metab. 2013;28(4):320-325. Published online December 12, 2013; DOI: https://doi.org/10.3803/EnM.2013.28.4.320

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Abstract PDF PubReader

Background

Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited, multisystemic tumor syndrome caused by mutations in the VHL gene. To date, more than 1,000 germline and somatic mutations of the VHL gene have been reported. We present a novel mutation in the VHL tumor suppressor gene that presented with gestational diabetes mellitus.

Methods

A 30-year-old woman presented with gestational diabetes mellitus. She sequentially showed multiple pancreatic cysts, spinal cord hemangioblastoma, cerebellar hemangioblastoma, and clear cell type renal cell carcinomas. Also, her father and brother had brain hemangioblastomas. Each of the three exons of the VHL gene was individually amplified by polymerase chain reaction and direct sequencing was performed using an ABI 3730 DNA analyzer.

Results

DNA sequence analysis to determine the presence of VHL mutation in her family revealed del291C, a novel frameshift mutation.

Conclusion

We found a novel mutation in the VHL tumor suppressor gene that presented with gestational diabetes mellitus.

Citations

Citations to this article as recorded by

Diversities of Mechanism in Patients with VHL Syndrome and diabetes: A Report of Two Cases and Literature Review
Yanlei Wang, Zhaoxiang Liu, Wenhui Zhao, Chenxiang Cao, Luqi Xiao, Jianzhong Xiao
Diabetes, Metabolic Syndrome and Obesity.2024; Volume 17: 1611. CrossRef
Retinal hemangioblastoma in a patient with Von Hippel-Lindau disease: A case report and literature review
Yikeng Huang, Weiwen Hu, Xionggao Huang
Frontiers in Oncology.2022;[Epub] CrossRef
Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms
Claudia von Arx, Monica Capozzi, Elena López-Jiménez, Alessandro Ottaiano, Fabiana Tatangelo, Annabella Di Mauro, Guglielmo Nasti, Maria Lina Tornesello, Salvatore Tafuto
Journal of Clinical Medicine.2019; 8(9): 1277. CrossRef
Brief Review of Articles in 'Endocrinology and Metabolism' in 2013
Won-Young Lee
Endocrinology and Metabolism.2014; 29(3): 251. CrossRef

Case Reports

Mediastinal Uptake Misinterpreted as Metastasis in Papillary Thyroid Cancer.: Eun Kyung Lee, Kyung Won Kim, So Yeon Park, Young Joo Park, Young Tae Kim, June Key Chung, Hwa Young Cho, Yun Hyi Ku, Hee Suk Min, Seong Hoe Park, Do Joon Park, Bo Youn Cho; J Korean Endocr Soc. 2007;22(6):460-464. Published online December 1, 2007; DOI: https://doi.org/10.3803/jkes.2007.22.6.460

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Abstract PDF

Radioactive iodine (RAI) therapy is used for the removal of remnant thyroid tissue or metastatic thyroid cancer cells in differentiated thyroid cancer. The main mechanism of the therapy is destruction of cells by radioactive iodine that penetrates the cells though the action of the sodium-iodide symporter (NIS). We experienced a case of a 26-year-old woman with mediastinal uptake as detected on a radioiodine scan, who was previously diagnosed with papillary thyroid cancer. For diagnostic tests including chest computed tomography (CT) and a radioiodine scan, the stimulated thyroglobulin level did not show a definite cause of the mediastinal uptake. During regular follow-up, the thymus became triangular with clear margins. The patient had neither specific symptoms nor physical findings related to the presence of a thymic mass. A subsequent CT scan showed an irregular margin of the thymus, suggestive of thymic metastasis. The patient underwent a mediastinectomy. The removed specimen was composed of normal thymic tissue. Moreover, we demonstrated the presence of human NIS by immunohistochemical analysis. After thymectomy, the mediastinal uptake was markedly decreased as compared to the previous scan. This case suggests that a clinician should be suspicious for the functional uptake of thymus when metastasis is unlikely in a clinical situation.

Citations

Citations to this article as recorded by

Clinical Significance of Diffuse Intrathoracic Uptake on Post-Therapy I-131 Scans in Thyroid Cancer Patients
Hyun Su Choi, Sung Hoon Kim, Sonya Youngju Park, Hye Lim Park, Ye Young Seo, Woo Hee Choi
Nuclear Medicine and Molecular Imaging.2014; 48(1): 63. CrossRef

A Case of Adrenocortical Carcinoma showing Variable Cortisol Production during the Clinical Course.: Yun Hyi Ku, Hyung Jin Choi, Jin Taek Kim, Ji Won Yoon, Eun Kyung Lee, Hwa Young Cho, Mi Yeon Kang, Jie Seon Lee, Young Min Cho, Seong Yeon Kim; J Korean Endocr Soc. 2006;21(5):419-423. Published online October 1, 2006; DOI: https://doi.org/10.3803/jkes.2006.21.5.419

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Abstract PDF: Patients with adrenocortical carcinoma (ACC) present with evidence of excessive adrenal steroid hormone in approximately 60% of cases, in which rapidly progressing Cushing's syndrome with or without virilization is the most frequent presentation. Some patients experience an increase or a decline in cortisol production through the progression of their ACC. We report on an unusual case of a cortisol-producing ACC, and the patient presented with a decline in cortisol production, followed by an increase in cortisol production, through the progression of the tumor. A 65-year-old woman who manifested with facial edema and weight gain was diagnosed with Cushing's syndrome, caused by cortisol producing ACC. The patient was treated with adrenalectomy. However, 8 months later, a metastatic hepatic tumor of recurred ACC was detected. At that time, the hormonal evaluation revealed that the liver mass did not produce any hormones. The patient was treated with metastatectomy. Four months later, a relapsed tumor was detected. Increased cortisol production was observed at that time. We speculate there was a change in the clonal dominance within the ACC and this change might cause such a difference. This is the first case report of ACC that showed variable hormone production during progression.

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