Abstract
Non-epithelial ovarian cancers (NEOC) are a group of uncommon malignancies that mainly includes germ cell tumours (GCT), sex cord-stromal tumours (SCST), and some extremely rare tumours, such as small cell carcinomas and sarcomas. Each of these classifications encompasses multiple histologic subtypes. The aetiology and molecular origins of each sub-group of NEOC require further investigation, and our understanding on the genetic changes should be optimised. In this article, we provide an update on the clinical presentation, pathology, genetics, treatment and survival of the main histological subtypes of the GCT and the SCST, as well as of ovarian small cell carcinomas. We also discuss miRNA expression profiles of NEOC and report the currently active clinical trials that include NEOC.
Highlights
Epithelial ovarian cancer (EOC) is the most lethal gynaecologic malignancy, as it is commonly diagnosed at an advanced stage
This review summarises the published literature on the clinical, histological and therapeutic aspects of the main subtypes of Non-epithelial ovarian cancers (NEOC), along with miRNA expression profiles and clinical trials that currently recruit patients
NEOC represents a group of rare ovarian cancers, which mainly affects young women and adolescents
Summary
Epithelial ovarian cancer (EOC) is the most lethal gynaecologic malignancy, as it is commonly diagnosed at an advanced stage. Non-epithelial ovarian cancers (NEOC) are rare, accounting for approximately 10% of all ovarian cancers and include mainly germ cell tumours (GCT), sex cord-stromal tumours (SCST), and some extremely rare tumours [1,2,3]. SCST arise from the sex cord and ovarian stroma and comprise a heterogeneous group of tumours, of which granulosa cell tumour (GrCT) is the most frequent type. Serum anti-Müllerian hormone (AMH) may be a marker of ovarian reserve and GrCT in postmenopausal or post-oophorectomy women. While these markers are nonspecific, they can provide prognostic information, and as such quantitative hCG, AFP, LDH and cancer antigen 125 (CA-125) should be measured preoperatively [2]. This review summarises the published literature on the clinical, histological and therapeutic aspects of the main subtypes of NEOC, along with miRNA expression profiles and clinical trials that currently recruit patients
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