Endocrinology and Metabolism

Case Reports

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; J Korean Endocr Soc. 2008;23(4):266-271. Published online August 1, 2008; DOI: https://doi.org/10.3803/jkes.2008.23.4.266

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Multiple endocrine neoplasia type 1 (MEN 1) is characterized by the combined occurrence of primary hyperparathyroidism, enteropancreatic tumors and anterior pituitary adenoma. Yet carcinoid tumors, adrenal adenoma and lipoma might exist simultaneously. Thymic carcinoid tumors, which are recognized as one of the causes of death for patients with MEN 1, are uncommon and their natural history has barely been investigated.

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A rare case of multiple endocrine neoplasia type 1 initially presenting as an asymptomatic, huge mediastinal mass: case report
Ji Eun Jun, You-Cheol Hwang, Kyu Jeong Ahn, Ho Yeon Chung, In-Kyung Jeong
BMC Endocrine Disorders.2021;[Epub] CrossRef
A Case of Asymptomatic Multiple Endocrine Neoplasia Type I with Thymic Carcinoid
Suk Ki Park, Moon Won Lee, In Sub Han, Young Joo Park, Sung Yong Han, Joon Woo Park, Bong Eun Lee, Gwang Ha Kim, Sang Soo Kim
The Korean Journal of Helicobacter and Upper Gastrointestinal Research.2019; 19(1): 65. CrossRef
Incidental metastatic mediastinal atypical carcinoid in a patient with parathyroid adenoma: a case report
Zareen Kiran, Asma Ahmed, Owais Rashid, Saira Fatima, Faizan Malik, Saulat Fatimi, Mubassher Ikram
Journal of Medical Case Reports.2017;[Epub] CrossRef
Multiple Endocrine Neoplasia Type 1 Presenting with an Invasive Giant Prolactinoma
Jinhoon Cha, Jin Seo Kim, Jung Suk Han, Yeon Won Park, Min Joo Kim, Yun Hyi Ku, Hong Il Kim
The Korean Journal of Medicine.2016; 91(3): 300. CrossRef
Genetic and Epigenetic Analysis in Korean Patients with Multiple Endocrine Neoplasia Type 1
Yoon Jung Chung, Sena Hwang, Jong Ju Jeong, Sun Yong Song, Se Hoon Kim, Yumie Rhee
Endocrinology and Metabolism.2014; 29(3): 270. CrossRef
A Case of Asymptomatic Multiple Endocrine Neoplasia Type 1 Detected Incidentally on Health Screening
Pyung-San Cho, Hoon Park, Guk-Haeng Lee, Myung-Chul Lee
Korean Journal of Otorhinolaryngology-Head and Neck Surgery.2012; 55(6): 373. CrossRef

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.

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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

A Case of Multiple Endocrine Neoplasia Type 1 with Mutation in MENIN Gene.: Se Eun Park, Eun Seok Kang, Hyun Joo Lee, So Hun Kim, Mi Young Do, Shin Ae Kang, Seung Jin Han, Hyeong Jin Kim, Chul Woo Ahn, Bong Soo Cha, Sung Kil Lim, Kyung Rae Kim, Il Jin Kim, Hyun Chul Lee; J Korean Endocr Soc. 2005;20(1):71-77. Published online February 1, 2005; DOI: https://doi.org/10.3803/jkes.2005.20.1.71

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Abstract PDF

Multiple endocrine neoplasia type 1(MEN 1) is an autosomal dominantly inherited syndrome, characterized by the combined occurrence of tumors of the parathyroid glands, endocrine pancreas, and anterior pituitary gland. The MENIN gene, which is a kind of tumor suppressor gene, is located at the chromosomal locus 11q13. It consists of one untranslated exon and nine exons encoding the menin protein. We report a case of a 22-yearss-old woman with MEN type 1, who was proven to have a mutation in the MENIN gene. The patient was admitted because of repeated hypoglycemia. The fasting plasma glucose level was 32mg/dL. Seventy two hours fasting test showed an the insulin/glucose ratio as 0.33. Endoscopic ultrasonography detected multiple masses on the pancreas. The arterial -stimulated venous sampling(ASVS) with calcium showed sudden step up of insulin at the head and tail portions of the pancreas. The sellar MRI showed a pituitary mass that produced prolactin. Instead of a pathologic diagnosis from operational specimen, the genetic analysis revealed a mutation in the MENIN 1 gene(exon 2, 200~201insAGCCC).

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Genetic and Epigenetic Analysis in Korean Patients with Multiple Endocrine Neoplasia Type 1
Yoon Jung Chung, Sena Hwang, Jong Ju Jeong, Sun Yong Song, Se Hoon Kim, Yumie Rhee
Endocrinology and Metabolism.2014; 29(3): 270. CrossRef
A Case of Familial Multiple Endocrine Neoplasia Type 1 with a Novel Mutation in theMEN1Gene
Min Jung Kim, Eun Hee Kim, Mi-Seon Shin, Joo Hui Kim, Hee Kyung Na, Seong Joon Park, Sang Ah Lee, Eun Hee Koh, Woo Je Lee, Ki Ho Song, Joong-Yeol Park, Ki-Up Lee, Gu-Hwan Kim, Han-Wook Yoo, Min-Seon Kim
Endocrinology and Metabolism.2011; 26(2): 171. CrossRef
Somatic Mutational Analysis of MEN1 and Phenotypic Correlation in Sporadic Parathyroid Tumors
Young Su Chae, Hee Jin Kim, Sun Wook Kim, Myung-Chul Chang
Journal of the Korean Surgical Society.2009; 76(1): 15. CrossRef
Multiple Endocrine Neoplasia Type 1 with Multiple Leiomyomas Linked to a Novel Mutation in the MEN1 Gene
Heekyoung Choi, Sehyun Kim, Jae-Hoon Moon, Yoon Hee Lee, Yumie Rhee, Eun Seok Kang, Chul Woo Ahn, Bong Soo Cha, Eun Jig Lee, Kyung Rae Kim, Hyun Chul Lee, Seon Yong Jeong, Hyun Ju Kim, Sung-Kil Lim
Yonsei Medical Journal.2008; 49(4): 655. CrossRef
A Case of Familial Multiple Endocrine Neoplasia Type 1 with MEN1 Gene Mutation
Young Eun Jo, Yong-Jun Choi, Yun Kyung Kim, Sang Mi Ahn, Sun Hye Jung, Hae Jin Kim, Dae Jung Kim, Kwan Woo Lee, Ji-Hee Hong, Seon-Yong Jeong, Hyon J Kim, Yoon-Sok Chung
Journal of Korean Endocrine Society.2007; 22(1): 68. CrossRef
A Case of Familial Multiple Endocrine Neoplasia with MEN1 Gene Mutation
Hye-Young Sung, Yeon-Joo Chun, Hyeug Lee, Bum Jun Kwon, Kun Woo Park, Jung Min Lee, Sung Dae Moon, Sang Ah Chang, Je-Ho Han
Journal of Korean Endocrine Society.2006; 21(6): 560. CrossRef

A Case of Multiple Endocrine Neoplasia Type 1.: Soon Jib Yoo, Bong Yun Cha, Kwang Woo Lee, Ho Young Son, Sung Koo Kang, Jong Man Won, Young Seok Cho, Yong Jik Sung, Kyung Sub Song, Jin Young Yoo, Chul Woo Lee; J Korean Endocr Soc. 1997;12(1):111-119. Published online January 1, 2001

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Abstract PDF: Multiple endocrine neoplasia type 1 (MEN 1) is the association of neoplastic transformation of parathyroid, pituitary, and pancreatic islet cells. This syndrome is inherited as an autosomal dominant trait. A 38-year-old woman presented with general weakness and mental changes. She had experienced same symptoms 7 years ago, after then her weight gradually increased. Insulinoma was suspected by markedly decreased blood glucose level (20mg/dL) and the increased insulin/glucose ratio (0.43) that sampled in emergency room at the time of mental change. Unusually large pancreatic tail mass and Lt, adrenal gland mass were detected by abdominal CT. Percutaneous transhepatic portal vein catheterization with insulin sampling showed sudden step up of insulin/glucose ratio at the middle portion of pancreas. Measuring of basal pituitary hormones as a screening procedure of MEN showed increased basal prolactin level. Combined pituitary stimulation test showed blunted response of prolactin to TRH and sellar magnetic resonance imaging showed intrasellar mass. Thyroid nodule was palpated on her anterior neck. Thyroid scintigram showed cold nodule, and there was no lymphadenopathy around the nodule by the thyroid sonogram. Fine needle aspiration cytology showed benign hyperplastic follicular cells only. Serum ionized calcium and parathyroid hormone level were normal. Under the impression of MEN type I, the distal pancreatectomy, splenectomy, Lt. thyroid lobectomy and Lt. adrenalectomy was performed at the same time. Histologic examination of the surgically removed tissues revealed pancreas islet cell tumor, adrenal cortical adenoma and thyroid adenoma. Transient hyperglycemia was developed after surgical intervention, but thereafter she never felt any symptoms of hypoglycemia till now and her blood glucose showed completely normal level with oral glucose tolerance tests. We present this case with a review of literature.

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