title: Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
authors.affiliation: Hospital de Clínicas, Universidade Federal de Uberlândia, Uberlândia, Minas Gerais, Brazil, CEP 38400-020.
authors.forename: Fabrícia Torres
authors.initials: FT
authors.lastname: Gonçalves
authors.forename: Taciana Carla Maia
authors.initials: TC
authors.lastname: Feibelmann
authors.forename: Cínthia Monteiro
authors.initials: CM
authors.lastname: Mendes
authors.forename: Maria Luiza Mendonça Pereira
authors.initials: ML
authors.lastname: Fernandes
authors.forename: Geraldo Henrique Gouvêa de
authors.initials: GH
authors.lastname: Miranda
authors.forename: Agostinho Pinto
authors.initials: AP
authors.lastname: Gouvêa
authors.forename: Paulo Tannús
authors.initials: PT
authors.lastname: Jorge
mesh_terms: D000293:Adolescent; D000303:Adrenal Cortex Diseases / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology*; D000315:Adrenalectomy; D003480:Cushing Syndrome / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D005260:Female; D006801:Humans; D007118:Immunoassay; D007911:Lentigo / Q000150:complications* / Q000235:genetics; D008163:Luminescent Measurements; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis* / Q000235:genetics; D013577:Syndrome; D014057:Tomography, X-Ray Computed