- A Case of Multiple Endocrine Neoplasia Type 1.
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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
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J Korean Endocr Soc. 1997;12(1):111-119. Published online January 1, 2001
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- 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.
- A Case of Cushing's syndrome due to Primary Pimary Pigmented Nodular Adrenal Dysplasia ( PPNAD ): A Case of Carney's Complex.
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Soon Jib Yoo, Bong Yun Cha, Kwang Woo Lee, Ho Young Son, Sung Koo Kang, Youn Sik Kim, Jong Min Lee, Jong Man Won
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J Korean Endocr Soc. 1997;12(1):90-98. Published online January 1, 2001
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- Primary Pigmented Nodular Adrenal Dysplasia (PPNAD) is a rare cause of Cushing's syndrome in infants and young adults. The familial occurrence, it may be variably associated with a complex of other pathologic characteristics that manifests extraadrenal disorders (includes cardiac myxomas, lentigines, mammary myxoid lesions, testicular tumors, pituitary adenomas, and neuroectodermal tumors) was considered indicative of Carneys complex. This was based on the failure of cortisol suppression by high-dose dexamethasone, either normal or suppressed basal adrenocorticotropic hormone (ACTH) levels, and normal radiographic studies of the sellar turcica, and adrenals glands is almost normal or slightlg eulaged.. Bilateral adrenalectomy has thus the only effective means of cure. The disease may be a component of a rare, but potentially dangerous complex of abnormalities that follow an autosomal-dominant mode of inheritance. Recently we experienced a case of Carney's complex composed by Cushings syndrome due to PPNAD with familial purple colored lentigines on their lips and report it with reviews of the literatures.
- A Case of Pheochromocytoma Diagnosed as Preeclampsia during Prenancy.
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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
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J Korean Endocr Soc. 1994;10(3):295-299. Published online November 6, 2019
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- 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.
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