Endocrinology and Metabolism

Case Report

A Case of Multiple Endocrine Neoplasia Type 1 with Papillary Thyroid Carcinoma.: Hai Jin Kim, Chul Sik Kim, Hyun Chul Je, Jina Park, Jong Suk Park, Jee Hyun Kong, Eun Seok Kang, Chul Woo Ahn, Bong Soo Cha, Sung Kil Lim, Kyung Rae Kim, Hyun Chul Lee, Hang Suk Jang, Soon Won Hong; J Korean Endocr Soc. 2006;21(1):79-84. Published online February 1, 2006; DOI: https://doi.org/10.3803/jkes.2006.21.1.79

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This is the first report of papillary thyroid carcinoma combined with multiple endocrine neoplasia type 1 (MEN 1). It is an hereditary syndrome characterized by neoplastic disorders such as pituitary adenoma, parathyroid adenoma or hyperplasia and pancreatic neuroendocrine tumor, such as gastrinoma just like in our case. But sometimes pheochromocytoma, mucosal ganglioneuromas, lipoma, forgut carcinoid and thyroid disease could be accompany the disease, but coincidental papillary thyroid carcinoma was never reported before in Korea. Herein we represent a 39-year-old woman who manifested typical features of MEN 1 with coincidental papillary thyroid carcinoma. Despite with definite family history of MEN 1, her genetic analysis of DNA had not found any germline mutation in MEN 1 gene. Unidentified culprit gene unable further genetic study of finding LOH (loss of heterogeneity) in 11q13, the possible explanation of papillary thyroid carcinoma as a new component of MEN 1. As we have experienced a case of MEN 1 combined with papillary thyroid carcinoma, we report it with the review of literature.

Citations

Citations to this article as recorded by

A Case of Multiple Endocrine Neoplasia Type I with Atypical Clinical Course
Yun Sun Choi, Youn Sun Bai, Bon Jeong Ku, Young Suk Jo, Young Kun Kim, Heung Kyu Ro, Minho Shong
Journal of Korean Endocrine Society.2008; 23(4): 266. CrossRef

Original Article

A Case of Multiple Endocrine Neoplasia Type I Presenting with a Watery Diarrhea.: Won Hyeok Choe, Yu Jeong Park, Il Chol Hong, Se Hoon Park, Sung Chul Choi, Hyo Rak Lee, In Kyung Jeong, Jae Hoon chung, yong Ki Min, Myung Shik Lee, Moon Kyu Lee, Kwang Won Kim, Won Seog Kim; J Korean Endocr Soc. 2001;16(2):231-237. Published online April 1, 2001

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Abstract PDF: MEN1 is characterized by the combined occurrence of tumors of the parathyroids, pancreatic islet cells and the anterior pituitary. Pancreatic islet cell tumors occur in 40% of MEN1 patients. Pancreatic polypeptidomas occur most commonly but they are asymptomatic. Gastrinomas are the second most common type. VIPomas are rare and there has been no report of a case in Korea so far. We encountered a case of pancreatic VIPoma in MEN Type I. A 49 year old man was referred from his local hospital presenting with a sudden onset of an explosive watery diarrhea of 3 months duration. Abnormal findings in his blood chemistry were hypercalcemia and hypokalemia. The 99mTc-sestamibi sintigraphy showed an increased uptake in right lower parathyroid gland. Abdominal CT demonstrated a mass of 6x4 cm in tail of the pancreas and multiple lesions in both hepatic lobes. Serum levels of VIP hormones were elevated. Subtotal parathyroidectomy and subtotal pancreatectomy were done. Postoperatively his symptoms were improved transiently, however the patient showed repetitive attacks of watery diarrhea. So in order to palliate his symptoms, an RF ablation of the metastatic liver masses was performed. After that therapy his clinical symptoms were reduced dramatically. Unfortunately, the patients condition worsened again. Despite of continuous octreotide therapy, interferon and two courses of combination chemotherapy, the hepatic metastases failed to regress and the patient died 10 months after the diagnosis of a metastatic VIPoma. This is the first report of pancreatic VIPoma in MEN type I in Korea.

Case Reports

A Case of Multiple Endocrine Neoplasia Type I Presented with Secondary Amenorrhea and Osteoporosis.: Sang Bum Hong, Seok Jun Hong, Young Ki Song, Ki Soo Kim, Sang Wook Kim, Ki Ub Lee, Min Kyu Kim, Seung Mo Hong, Duck Jong Han; J Korean Endocr Soc. 1998;13(4):684-689. Published online January 1, 2001

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Abstract PDF: MEN type 1 is characterized primarily by the presence of functioning and nonfunctioning tumors or hyperplasia of the pituitary gland, parathyroid glands, and pancreatic islet cells. Pancreatic islet tumors in MEN type 1 produce different kinds of hormone which were pancreatic polypeptide, gastrin, glucagon, insulin and so on. To date, ten cases had been reported in Korea. We report another case with MEN type 1 having prolatin-secreating pituitary adenoma, parathyroid hyperplasia and insulinoma. A 36-year-old woman was admitted because of long-standing amenorrhea and recently diagnosed osteoporosis. Otherwise, she had been in good health except experiencing one episode of loss of consciousness after skipped meal. The blood chemistries were normal except hypercalcemia and hypophosphatemia. Hormonal studies revealed elevated levels of intact PTH and prolactin and decreased value of estradiol with low LH and FSH. The neck CT revealed 1 cm-sized nodule at posterior portion of right thyroid gland and 99mTc-sestamibi sintigraphy showed a increased uptake in left lower and right lower parathyroid glands. The sella MRI showed 0.7 cm-sized enhanced lesion in the left pituitary gland. The ratio of immunoreactive insulin to glucose was elevated and 3-4 pancreatic masses of variable size were identified by endoscopic ultrasonography and angiography. Subtotal parathyroidectomy and pyrolus-preserving pancreaticoduodenectomy with spleen-preserving distal pancreatectomy was done. Postoperative she had been doing well with normocalcemia and normoglycemia. Transsphenoidal adenonectomy was done 5 months later. Histologic examination of removed tissues revealed a single insulinoma, prathyroid hyperplasia and prolactin-secreating pituitary adenoma.

A Case of Multiple endocrine neoplasia type 2a.: Seung Jae Hong, In Myung Yang, Jeong Taek Woo, Jin Woo Kim, Young Seol Kim, Young Kil Choi, Yong Sun Yun, Chung Hwan Lee, Seong Ho Lee, Deok Yoon Kim, Sung Weon Kim; J Korean Endocr Soc. 1997;12(2):328-337. Published online January 1, 2001

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Abstract PDF: Multiple endocrine neoplasia type2a (MEN type2a) is a dominantly inherited cancer syndrome which is characterized by medullary thyroid carcinoma, pheochromocytoma and parathyroid hyperplasia or adenoma. Recent reports show that DNA analysis will be introduced into screening of MEN type2a families. Regular prospective screening and appropriate surgical intervention can reduce the morbidity and mortality due to MEN type2a. We experienced a case of MEN type 2a in a 46-year-old female patient. She had undergone bilateral adrenalectomy due to pheochromocytoma, followed by a total radical thyroidectomy, which revealed medullary thyroid carcinoma of the both thyroid gland and parathyroid hyperplasia.

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