巨大な両側性副腎皮質多発結節性過形成によるクッシング症候群の一例

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Adrenal nodular hyperplasia is a rare clinical condition, and its pathogenesis is still under debate. Recently we encountered a patient with Cushing syndrome due to huge bilateral adrenal nodular hyperplasia. A 51 y.o. male, pilot, had been healthy and rather fat (body weight 75 kg) until 1971 when he was noticed to have glycosuria and hypertension. Since then, despite antidiabetic therapy, he lost weight gradually reaching 61 kg. Since August 1975 general lassitude and hypodynamia have been associated. Because of recently onsetted severe anorexia and continuous fever of 38°C he was admitted to our hospital on Nov. 7, 1975. On his admission, mild buffalo hump, muscular atrophy of extremities, left pleural effusion, facial edema and abdominal distension were observed. However, plethoric moon face and cutaneous striae were not seen. Laboratory findings demonstrated some inflammatory changes of unknown origin, and moderate hyperglycemia and hypertension. Serum K was 3.4 mEq/l. Loss of weight, bloody pleural effusion and elevated LDH (640 Wr U) were suggestive of malignancy, however, no malignant lesion was found. Treatment by insulin, antibiotics and aldactone were started, and after 4 weeks he become afebrile and pleural effusion also disappeared completely. After cessation of aldactone, hypopotassemia (2.2 mEq/l) and clinical manifestations of Cushing syndrome became evident. Endocrinological examinations revealed increased urinary 17-OHCS excretion (17.5-32.1 mg/day) and hypercortisolism with no diurnal change, while urinary 17-KS remained within normal limits. His adrenals did not respond to metopyrone and 8 mg dexamethasone, but an exaggerated response was seen by ACTH administration. Plasma ACTH. which was reported after surgery, was 10 pg/ml. Adrenal scintigraphy by 131-I adosterol showed typical head light sign. Through drip infusion pyelography and retroperitoneal pneumoroentogenogram, a tumor-like shadow in the left suprarenal region and another round shadow in the right infrarenal region were observed. There was some suspicious shadow in the right suprarenal region also, but this was not certain. From these observations, ectopic ACTH secretion from a right lower abdominal tumor or adrenal nodular hyperplasia were considered. On February 18, 1976, bilateral adrenalectomy was performed. Both adrenals were greatly enlarged but well capsulated and had multinodular appearances. They weighed 141 g (l) and 85 g (r), respectively. The tumor-like mass in the right lower abdomen contained yellow pus and was an abscess. Histologically, a tumor-like hyperplasia of the adrenal cortex was confirmed. Major cells had bright cytoplasma and were rich in lipid granules which are typical properties of glucocorticoid secreting cells. Besides, sporadic distributions of reticular and glomerular zone derived cells were also observed. The postoperative course has been quite good, and 37.5 mg of cortisone acetate and 0.25 mg of decadron have maintained his health until Dec. 1976. No apparent rise of plasma ACTH or beta-MSH has been noticed. Such a huge nodular hyperplasia exceeding 200 g has never been reported before, and this case seemed to represent advanced features of this condition. Compared with the data of 19 previously reported cases, adrenal nodular hyperplasia appears to have the following characteristics : long duration of the disease (1-45 years), increased urinary 17-OHCS but rather normal 17-KS, unresponsiveness to metopyrone (6/9) and 8 mg dexamethasone (12/14), responsive to exogenous ACTH (11/17), and low plasma ACTH (7/8). These characteristics are commonly seen in cases with large tumors, including this case. However, in cases with small tumor effectiveness of metopyrone and 8 mg dexamethasone, and sometimes elevated plasma ACTH are reported, suggesting the likelihood of simple adrenal hyperplasia. In a middle-sized case reported by Choi (15 g),
日本内分泌学会の論文

巨大な両側性副腎皮質多発結節性過形成によるクッシング症候群の一例

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