Carney Complex Complicated with Primary Pigmented Nodular Adrenocortical Disease without Cushing's Syndrome Recurrence for Five Years after Unilateral Adrenalectomy

被引：4

作者：

Tsurutani, Yuya ^{[1
]}

Kiriyama, Kanako ^{[1
]}

Kondo, Mai ^{[1
]}

Hasebe, Masanori ^{[2
]}

Sata, Akira ^{[2
]}

Mizuno, Yuzo ^{[2
]}

Sugisawa, Chiho ^{[1
]}

Saito, Jun ^{[1
]}

Nishikawa, Tetsuo ^{[1
]}

机构：

[1] Yokohama Rosai Hosp, Endocrinol & Diabet Ctr, Yokohama, Kanagawa, Japan

[2] Kanto Cent Hosp, Div Endocrinol & Metab, Tokyo, Japan

来源：

INTERNAL MEDICINE | 2022年 / 61卷 / 02期

关键词：

Carney complex; primary pigmented nodular adrenocortical disease; unilateral adrenalectomy; Cushing's syndrome; ALPHA REGULATORY SUBUNIT; MUTATIONS; GENETICS; PRKAR1A;

D O I：

10.2169/internalmedicine.7418-21

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

We herein report a case of Carney complex (CNC) complicated with primary pigmented nodular adrenocortical disease (PPNAD) after unilateral adrenalectomy. A 44-year-old woman was admitted to our hospital for PPNAD surgery. She had previously undergone surgery for cardiac myxoma and had a PRKAR1A mutation with no family history of CNC. She had Cushing's signs, but her metabolic abnormalities were mild. Adrenal insufficiency due to poor medication adherence was a concern, so she underwent unilateral adrenalectomy. Cushing's signs improved postoperatively and without recurrence for five years. Treatment plans for PPNAD should be determined based on the patient's condition, medication adherence, and wishes.

引用

页码：205 / 211

页数：7

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