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

• Autor(a) principal: Gonçalves,Fabrícia Torres
• Data de Publicação: 2006
• Outros Autores: Feibelmann,Taciana Carla Maia, Mendes,Cínthia Monteiro, Fernandes,Maria Luiza Mendonça Pereira, Miranda,Geraldo Henrique Gouvêa de, Gouvêa,Agostinho Pinto, Jorge,Paulo Tannús
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: São Paulo medical journal (Online)
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802006000600007
• topic: Cushing's syndrome; Hyperplasia; Adrenalectomy; Adrenal cortex diseases; Lentigo

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.