Sao Paulo medical journal = Revista paulista de medicina · 2006
Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review.
Клинический случайСтатьяОбзорНазвание и аннотация: Перевод в очереди
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Gonçalves Fabrícia Torres, Feibelmann Taciana Carla Maia, Mendes Cínthia Monteiro, Fernandes Maria Luiza Mendonça Pereira, Miranda Geraldo Henrique Gouvêa de, Gouvêa Agostinho Pinto, Jorge Paulo Tannús
Аннотация
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.
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.