Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: Case report and review of current literature

Bobbie S. Gostout, Noralane M. Lindor, Connie S. DiMarco, Prema P. Peethambaram, Amy C. Clayton

Research output: Contribution to journalArticle

9 Scopus citations

Abstract

Background. Peripheral primitive neuroectodermal tumor (pPNET) is aggressive and rare, comprising 1% of soft tissue sarcomas. Involvement of the reproductive tract is unusual. Case. A 35-year-old woman had a pelvic mass and omental cake. Frozen-section examination at laparotomy revealed small round cell tumor confirmed as pPNET. Chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide with mesna yielded complete response. The patient's mother died of a similar tumor at age 52 years, and the patient's husband had adult Ewing sarcoma, constituting an unusual cluster of related tumors. Conclusions. Genetic recombination resulting in a chimeric transcript of the Ewing sarcoma gene and the Friend leukemia virus integration site is characteristic of these tumors. Surgical resection and multiagent chemotherapy may enhance survival.

Original languageEnglish (US)
Pages (from-to)247-253
Number of pages7
JournalGynecologic oncology
Volume91
Issue number1
DOIs
StatePublished - Oct 1 2003

Keywords

  • Ewing sarcoma
  • Primitive neuroectodermal tumor

ASJC Scopus subject areas

  • Oncology
  • Obstetrics and Gynecology

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