Ovarian steroid cell tumor, not otherwise specified, associated with congenital adrenal hyperplasia: rare tumors of an endocrine disease

J Pediatr Surg. 2013 Jun;48(6):E23-7. doi: 10.1016/j.jpedsurg.2013.04.006.

Abstract

Ovarian steroid cell tumors, not otherwise specified (OSCTs), are extremely rare and present a diagnostic challenge when evaluating an ovarian mass. We present a case of such a tumor in a patient with known Congenital Adrenal Hyperplasia (CAH), secondary to 21-hydroxylase deficiency, who was noncompliant with her medications. The workup, diagnosis, and treatment of this rare condition are described.

Keywords: Congenital adrenal hyperplasia; Endocrine tumors; Ovarian adrenal rest tumors; Ovarian steroid cell tumors; Ovarian tumor.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adrenal Hyperplasia, Congenital / complications*
  • Adrenal Rest Tumor / diagnosis*
  • Adrenal Rest Tumor / etiology
  • Female
  • Humans
  • Ovarian Neoplasms / diagnosis*
  • Ovarian Neoplasms / etiology

Supplementary concepts

  • Congenital adrenal hyperplasia due to 21 hydroxylase deficiency