Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects

Crit Rev Oncol Hematol. 2017 Dec:120:43-51. doi: 10.1016/j.critrevonc.2017.10.007. Epub 2017 Oct 16.


Non-epithelial ovarian cancers (NEOC) constitute a group of uncommon malignancies and their treatment is still a challenging task. Collectively, these tumours account for about 10% of all ovarian cancers and occur in all age groups from childhood to old-age. They include malignancies of germ cell origin, sex cord-stromal cell origin, and a variety of extremely rare ovarian cancers, such as small-cell carcinomas and sarcomas. Each of these classifications encompasses multiple histologic subtypes. It is imperative that these rare tumours are managed with accurate diagnosis, staging, and treatment, to optimise the outcome. The aetiology and molecular origins of each sub-group of NEOC remain largely unresolved, and international cooperation to promote high quality translational research is crucial. Much effort has been made into researching the molecular mechanisms underlying epithelial ovarian cancers, but far less is known about the genetic changes in NEOC. In this article, it is provided an overview of the current knowledge on the incidence, clinical presentation, pathology, genetics, therapeutic interventions, survival and prognostic factors of adult and juvenile granulosa cell tumours (GrCT), Sertoli-Leydig Cell tumours (SLCT) and small cell carcinoma of the ovary. We also consider future potential therapeutic targets in these rare cancers.

Keywords: Granulosa cell; Non-epithelial ovarian cancers; Sertoli-Leydig; Sex cord-stromal; Small cell carcinoma hypercalcaemic type.

Publication types

  • Review

MeSH terms

  • Carcinoma, Small Cell / genetics*
  • Carcinoma, Small Cell / pathology
  • Carcinoma, Small Cell / therapy*
  • Female
  • Humans
  • Ovarian Neoplasms / genetics*
  • Ovarian Neoplasms / pathology
  • Ovarian Neoplasms / therapy*
  • Sex Cord-Gonadal Stromal Tumors / genetics*
  • Sex Cord-Gonadal Stromal Tumors / pathology
  • Sex Cord-Gonadal Stromal Tumors / therapy*