- A Case of Multiple Endocrine Neoplasia Type I Presented with Secondary Amenorrhea and Osteoporosis.
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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
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J Korean Endocr Soc. 1998;13(4):684-689. Published online January 1, 2001
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 Abstract
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- 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.
- Piruitray Thyrotropin-Secreting Tumors in Korean.
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Chul Hee Kim, Ghi Su Kim, Hong Kyu Kim, Joong Yeol Park, Young Kee Shong, Sang Bum Hong, Jung Min Ko, Chang Jin Kim
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J Korean Endocr Soc. 1997;12(2):165-175. Published online January 1, 2001
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Abstract
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- BACKGROUND
Thyrotropin-secreting pituitary adenoma is an uncommon disease and about 150 cases has been reported in the world literature. In Korea, only seven cases were reported as yet. The authors recently experienced four cases of TSH secreting pituitary tumor and analyzed the clinical characteristics and treatment outcomes of TSH-secreting tumors in Korean. METHODS: We analyzed clinical records of the four cases who had been recently treated at Asan Medical Center and the Korean literature which deals with the previously reported seven cases of TSH-secreting pituitary tumor. RESULTS: The average age at diagnosis was 37 years (ranging from 11 to 55 years). Four were men and seven were women. After the detection of hyperthyroidism, TSH-secreting pituitary adenoma was diagnosed 3.6 years later on the average. Ten patients presented with hyperthyroidism, but one had primary hypothyroidism. Typical features of acromegaly were observed in two patients. Visual disturbance was present in three cases, and galactorrhea was present in one case. Serum TSH concentrations ranged from 1.5 to 42.5uIU/mL showing mildly elevated or unsup-pressed TSH levels despite of elevated serum thyroid hormone concentrations. Among six cases in whom a-subunit level was measured, five showed elevated a-subunit level and a-subunit/TSH molar ratio. Two of 11 cases had microadenoma and the remainder had macroadeno#ma. Immunohisto-cheical studies were done in eight cases and revealed that three were positive for TSH only and five patients were positive for multiple hormones. Eight patients underwent transsphenoidal pituitary surgery and seven (88%) of them were cured. External irradiation or octreotide was used as adjunctive treatment in three cases. After treatment, TSH levels decreased in all six patients studied, hyperthyroidism was eliminated in all eight patients studied and visual disturbance was improved in two patients. CONCLUSION: Clinical characteristics of TSH-secreting pituitary adenoma in Koreans were similar with world literature, but were more common in women, had less visual disturbance and better surgical results. Diagnosis was commonly delayed for several years. TSH-secreting pituitary adenoma may be diagnosed more frequently and earlier with widespread use of sensitive TSH assay and early and proper diagnosis would lead proper treatments with improved outcome.
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