Practical Review of Ovarian Sex Cord-Stromal Tumors

Surg Pathol Clin. 2019 Jun;12(2):587-620. doi: 10.1016/j.path.2019.02.005.

Abstract

Ovarian sex cord-stromal tumors are uncommon tumors and clinically differ from epithelial tumors. They occur across a wide age range and patients often present with hormone-related symptoms. Most are associated with an indolent clinical course. Sex cord-stromal tumors are classified into 3 main categories: pure stromal tumors, pure sex cord tumors, and mixed sex cord-stromal tumors. The rarity, overlapping histomorphology and immunoprofile of various sex cord-stromal tumors often contributes to diagnostic difficulties. This article describes the various types of ovarian sex cord-stromal tumors and includes practical approaches to differential diagnoses and updates in classification.

Keywords: DICER1 syndrome; Histomorphology; Immunohistochemistry; Sex cord–stromal tumor.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / metabolism
  • Diagnosis, Differential
  • Female
  • Granulosa Cell Tumor / diagnosis
  • Granulosa Cell Tumor / pathology
  • Humans
  • Leydig Cell Tumor / diagnosis
  • Leydig Cell Tumor / pathology
  • Ovarian Neoplasms / diagnosis
  • Ovarian Neoplasms / pathology*
  • Sertoli Cell Tumor / diagnosis
  • Sertoli Cell Tumor / pathology
  • Sex Cord-Gonadal Stromal Tumors / diagnosis
  • Sex Cord-Gonadal Stromal Tumors / pathology*
  • Thecoma / diagnosis
  • Thecoma / pathology

Substances

  • Biomarkers, Tumor

Supplementary concepts

  • Granulosa cell tumor of the ovary