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Review Case Reports
Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review.
- Fabrícia Torres Gonçalves, Taciana Carla Maia Feibelmann, Cínthia Monteiro Mendes, Maria Luiza Mendonça Pereira Fernandes, Geraldo Henrique Gouvêa de Miranda, Agostinho Pinto Gouvêa, and Paulo Tannús Jorge.
- Hospital de Clínicas, Universidade Federal de Uberlândia, Uberlândia, Minas Gerais, Brazil, CEP 38400-020.
- Sao Paulo Med J. 2006 Nov 7; 124 (6): 336339336-9.
ContextCarney 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 ReportWe 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.
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