Updates on Rare Epithelial Ovarian Carcinoma

J. Bergstrom, I. M. Shih, A. N. Fader

Research output: Chapter in Book/Report/Conference proceedingChapter


Ovarian epithelial cancer is a heterogeneous group of neoplastic diseases that can be broadly classified as type I and type II tumors. While type II ovarian cancer is composed of mostly high-grade serous carcinoma (HGSC), the most common type of ovarian cancer, type I ovarian cancers, includes several histologic subtypes and each of them only constitutes a small percentage of ovarian cancer. These rare ovarian cancers are divided into three groups: (1) endometriosis-related tumors which include endometrioid, clear cell, and seromucinous carcinomas, (2) low-grade serous carcinomas, and (3) mucinous carcinomas and malignant Brenner tumors. They usually present at early stages which are amenable for curable surgical resection. They grow slowly and are always associated with precursor lesions. Molecularly, the rare (type I) tumors are characterized by frequent somatic mutations involving PTEN, CTNNB1, ARID1A, KRAS, BRAF, ERBB2, PIK3CA, and mismatch repair genes, which are uncommon in the conventional ovarian cancer (i.e., HGSC). The presence of mutations provides an opportunity for targeted therapy using inhibitors that inactivate individual molecular pathways. In this chapter, we will briefly describe the clinicopathological features of representative type I tumors and summarize the most recent advances in elucidating their molecular pathogenesis. Lastly, we will discuss the ongoing and newly proposed therapeutic intervention to treat these rare types of ovarian cancer.

Original languageEnglish (US)
Title of host publicationTranslational Advances in Gynecologic Cancers
PublisherElsevier Inc.
Number of pages15
ISBN (Electronic)9780128037980
ISBN (Print)9780128037416
StatePublished - Feb 22 2017
Externally publishedYes


  • Ovarian cancer
  • Pathogenesis
  • Targeted therapy

ASJC Scopus subject areas

  • Medicine(all)


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