ACTH非依存性両側副腎皮質大結節性過形成(AIMAH)によるCushing症候群の1例

Abstract

49歳男にみられたACTH非依存性両側副腎皮質大結節性過形成により, クッシング症候群を呈した症例.治療は両側副腎摘出術を行い, 術後ハイドロコルチゾール補充療法を施行し順調に経過している

We treated a case of ACTH-independent bilateral adrenocortical macronodular hyperplasia (AIMAH), a rare disease. The patient was a 49 year-old man having chief complaints of facial edema, muscle wasting and typical Cushing's syndrome symptoms. He was diagnosed with AIMAH by specific hormonal tests for Cushing's syndrome and CT scan. Bilateral total adrenalectomy was performed in a two-stage operation for bilateral macronodular adrenocortical hyperplasia. The resected adrenal tumor weighed 57 g on the right side and 78 g on the left, and both had a yellowish nodular surface. The histological appearance was typical AIMAH. A total of 23 AIMAH reported cases was reviewed.