Borderline with Bad Behavior: An Unusual Low-grade Serous Carcinoma with Dedifferentiation from a Serous Borderline Tumor

Amanda L. Strickland*, Kruti P Maniar, Edward Tanner, Elisheva Shanes, Lawrence J Jennings, Jian Jun Wei

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

The pathogenesis of serous ovarian tumors has been extensively investigated, with a dualistic model dividing these cancers into 2 groups. Type I tumors, including low-grade serous carcinoma, is characteristic for concurrent presence of borderline tumors, less atypical cytology, relatively indolent biologic behavior, and molecular aberrations related to the MAPK pathway with chromosomal stability. Meanwhile, type II tumors, such as high-grade serous carcinoma, are notable for no significant association with borderline tumors, higher grade cytology, more aggressive biologic behavior, and TP53 mutations along with chromosomal instability. We describe a case of morphologic low-grade serous carcinoma with focally increased cytologic atypia arising in serous borderline tumors involving both ovaries, which demonstrated highly aggressive behavior despite several years of surgical and chemotherapeutic management. Each recurrent specimen contained more uniform higher grade morphology than what was seen in the original specimen. Immunohistochemical and molecular studies in both the original tumor and the most recent recurrence demonstrate identical mutations in the MAPK genes, but with additional mutations in the latter, notably an acquisition of a variant of possible clinical significance in the SMARCA4 gene, which is associated with dedifferentiation and aggressive biologic behavior. This case challenges our current and still evolving understanding of the pathogenesis, biologic behavior, and expected clinical outcome of low-grade serous ovarian carcinomas. It also underscores the need for further investigation into this complicated tumor.

Original languageEnglish (US)
Pages (from-to)241-246
Number of pages6
JournalInternational Journal of Gynecological Pathology
Volume42
Issue number3
DOIs
StatePublished - May 1 2023

Keywords

  • Dedifferentiation
  • Low-grade serous carcinoma
  • MAPK
  • SMARCA4
  • Serous borderline tumor

ASJC Scopus subject areas

  • Obstetrics and Gynecology
  • Pathology and Forensic Medicine

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