Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review.
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.
Gonçalves
Fabrícia Torres
FT
Hospital de Clínicas, Universidade Federal de Uberlândia, Uberlândia, Minas Gerais, Brazil, CEP 38400-020.
Feibelmann
Taciana Carla Maia
TC
Mendes
Cínthia Monteiro
CM
Fernandes
Maria Luiza Mendonça Pereira
ML
Miranda
Geraldo Henrique Gouvêa de
GH
Gouvêa
Agostinho Pinto
AP
Jorge
Paulo Tannús
PT
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