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Rare ovarian tumors: an update on diagnosis and treatment
  1. Charlotte Debuquoy1,
  2. Clemence Romeo2,
  3. Helene Vanacker2 and
  4. Isabelle Ray-Coquard2,3
  1. 1Gynecology Surgical Department, Centre Leon Berard, Lyon, Rhône-Alpes, France
  2. 2Medical Oncology, Centre Leon Berard, Lyon, Rhône-Alpes, France
  3. 3University Claude Bernard, Laboratoire HESPER, Lyon, France
  1. Correspondence to Dr Isabelle Ray-Coquard, Centre Leon Berard, Lyon 69373, France; isabelle.ray-coquard{at}


Rare ovarian cancers occur frequently. Almost half of ovarian malignancies relate to several different ‘rare’ histotypes, according to the World Health Organization. The most common tumors are epithelial tumors, including high grade serous carcinomas, the presumed ‘frequent ovarian cancers’, together with low grade serous, mucinous, endometrioid, clear cell, and carcinosarcomas. Sex cord stromal tumors and germ cell carcinomas define two other groups of different subtypes, and small cell carcinomas are an independent high grade subtype closely related to the family of rhabdoid tumors. All of these cancers are primary ovarian cancers, classified by the International Federation of Gynecology and Obstetrics. However, the tumor subtypes display various epidemiologic, clinical, pathological, prognostic, and therapeutic characteristics. Because of the scarcity of data, current understanding of each subtype is limited and treatment has generally been derived from the more common tumor types. The aim of this article is to review the current literature on rare ovarian malignancies.

  • Ovarian cancer

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  • CD and CR contributed equally.

  • Contributors All authors contributed to the editing of the manuscript. The two first authors contributed equally to this work.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial, or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.