Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review

Sao Paulo Medical Journal

Fabrícia Torres Gonçalves¹  Taciana Carla Maia Feibelmann¹  Cínthia Monteiro Mendes¹  Maria Luiza Mendonça Pereira Fernandes¹  Geraldo Henrique Gouvêa De Miranda¹  Agostinho Pinto Gouvêa¹  Paulo Tannús Jorge¹ 

[1] ,Universidade Federal de Uberlândia Hospital de Clínicas Uberlândia Minas Gerais ,Brazil

关键词: Cushing's syndrome;    Hyperplasia;    Adrenalectomy;    Adrenal cortex diseases;    Lentigo;    Síndrome de Cushing;    Hiperplasia;    Adrenalectomia;    Doenças do córtex supra-renal;    Lentigo;   

DOI  :  10.1590/S1516-31802006000600007

来源: SciELO

【 摘 要 】

CONTEXT: Carney complex (CNC), a familial multiple neoplasm syndrome with dominant autosomal transmission, is characterized by tumors of the heart, skin, endocrine and peripheral nervous system, and also cutaneous lentiginosis. This is a rare syndrome and its main endocrine manifestation, primary pigmented nodular adrenal disease (PPNAD), is an uncommon cause of adrenocorticotropic hormone-independent Cushing's syndrome. CASE REPORT: We report the case of a 20-year-old patient with a history of weight gain, hirsutism, acne, secondary amenorrhea and facial lentiginosis. Following the diagnosing of CNC and PPNAD, the patient underwent laparoscopic bilateral adrenalectomy, and she evolved with decreasing hypercortisolism. Screening was also performed for other tumors related to this syndrome. The diagnostic criteria, screening and follow-up for patients and affected family members are discussed.

【 授权许可】

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