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Ovarian intratumoral 21-hydroxylase deficiency in a postmenopausal hirsute woman.

Abstract
Virilising ovarian tumours are a rare cause of hyperandrogenism in women, accounting for less than 5% of all ovarian neoplasms. It occurs most often in - and postmenopausal women. We report a case of a 64 year-old woman with signs of virilisation that had started 3 years before. Blood hormone analysis revealed increased levels of testosterone, and 17-hydroxyprogesterone. The tetracosactin test revealed 21-hydroxylase deficiency. Radiological imaging demonstrated a nodule in her left ovary. The patient was submitted to bilateral laparoscopic oophorectomy, and histopathological examination revealed a luteoma of the left ovary. Postoperative serum testosterone level and 17-hydroxyprogesterone returned to normal levels in one month. Virilism regressed within six months. Our patient also showed an elevation in 17-OHP serum levels. Normalization of 17-OHP after oophorectomy suggests a case of intratumoral 21-hydroxylase deficiency. To our knowledge, this is the first description of ovarian intratumoral 21-hydroxylase deficiency in a postmenopausal woman.
AuthorsSelma B Souto, Pedro V Baptista, Filomena Barreto, Pedro F Sousa, Daniel C Braga, Davide Carvalho
JournalArquivos brasileiros de endocrinologia e metabologia (Arq Bras Endocrinol Metabol) Vol. 56 Issue 9 Pg. 672-6 (Dec 2012) ISSN: 1677-9487 [Electronic] Brazil
PMID23329192 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Cosyntropin
  • Testosterone
Topics
  • Adrenal Hyperplasia, Congenital (pathology)
  • Cosyntropin
  • Female
  • Hirsutism (etiology, pathology)
  • Humans
  • Luteoma (complications, pathology)
  • Middle Aged
  • Ovarian Neoplasms (complications, pathology)
  • Postmenopause
  • Testosterone (blood)

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