Para-ovarian adrenal rest tumors: gynecologic manifestations of untreated congenital adrenal hyperplasia.
Gynecol Endocrinol
; 34(8): 644-646, 2018 Aug.
Article
em En
| MEDLINE
| ID: mdl-29460643
ABSTRACT
Congenital adrenal hyperplasia (CAH) is an inherited disorder of adrenal steroidogenesis often diagnosed in infancy. Gynecologists may encounter adult patients with CAH due to the clinical effects of increased androgens, e.g. hirsutism, clitoromegaly, oligomenorrhea, or, rarely, pelvic masses. This case report reviews the association of para-ovarian adrenal rest tumors with CAH, and the role of gynecologists in their evaluation and treatment. A 23-year-old woman with CAH (21-hydroxyase deficiency) untreated for the past 5 years presented with a pelvic mass and elevated serum testosterone (1433 ng/dL) and plasma ACTH (1117 pg/mL). Intraoperative findings revealed multiple retroperitoneal masses. Final pathology demonstrated adrenal rest tissue. Para-ovarian and ovarian adrenal rest tumors may present as a rare gynecologic manifestation in patients with untreated CAH.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Retroperitoneais
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Anexos Uterinos
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Doenças dos Anexos
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Hiperplasia Suprarrenal Congênita
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Tumor de Resto Suprarrenal
Tipo de estudo:
Etiology_studies
Limite:
Adult
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Female
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Humans
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article