悪性胸腺腫によるCushing症候群の一剖検例

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概要

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• A 32-year-old housewife was admitted to our hospital on March 8, 1965 with the complaints of obesity, fatigability, edema, polyuria and polydipsia. Hypertention and hypokalemia were noted by a physician.<BR>Physical examination revealed a round plethoric face, truncal obesity, crevicodorsal hump, mild hirsutism and purplish striae on the hip and thigh. Biochemical examina-tions showed mild diabetes mellitus and the elevated alkaline phosphatase. Radiological studies showed slight widening of the upper mediastinum and abnormal osteoplastic shadows which were scattered in almost all bones. The skull was normal. Both adrenal glands were considered to be normal in size by retroperitoneal pneumography.<BR>Urinary 17-OHCS levels ranged from 12.2 to 14.8 mg/24 hr. and urinary 17-KS levels were 5.3-12.1 mg/24 hr. Plasma 11-OHCS levels were elevated with the absence of the normal diurnal variation (28.5-34.5 μg/dl.). ACTH test was hyperresponsive while Metopirone (SU-4885) test was normal. Dexamethasone suppression test in doses of 8 mg/day failed to suppress the urinary 17-OHCS.<BR>During her hospitalization, tremor, muscular weakness, irritability and pigmentation of skin appeared. Severe hypokalemia was always present after her admission (2.2-3.3 mEq/L.) and was not improved by treatment. On June 22, she suddenly fell into a coma associated with marked hyperglycemia and hypokalemic alkalosis. (blood sugar levels 400 mg/dl, serum potassium 2.4 mEq/L, blood pH 7.62, Pco<SUB>2</SUB> 35.0 mmHg.) After recovering from the coma high remittent fever continued without remission. She died on July 16.<BR>Autopsy revealed a malignant thymoma and bilateral adrenocortical hyperplasia. Crooke's change was found microscopically in the pituitary gland. The metastases of the thymoma were found in many bones. Miliary tuberculosis was present. ACTH activity was detected in the malignant tissue (0.45 mU/gm.) with decreased ACTH activity of pituitary gland (Lipscomb & Nelson's method).<BR>It is strongly suggested that thymoma caused Cushing's syndrome.<BR>This case seems to be the second report in Japan of Cushing's syndrome caused by nonendocrine tumors in which the bioassay of ACTH activity was performed.

著者

• 住吉 昭信

  九州大学医学部病理学教室

• 三好 甫

  九州大学医学部第二内科教室

関連論文

• 肺の原発性癌肉腫の1剖検例 : 第14回日本肺癌学会総会演題抄録
• 悪性胸腺腫によるCushing症候群の一剖検例
• 先天性免疫不全症候群の免疫学的観察 (免疫グロブリンの化学と臨床(第67回日本内科学会講演会1970年シンポジウム))

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