"Proximal-type" epithelioid sarcoma vs. malignant rhabdoid tumor of the vulva: A case report, review of the literature, and an argument for consolidation

Peter A Argenta, Sajeena Thomas, Justin C. Chura

Research output: Contribution to journalReview articlepeer-review

34 Scopus citations

Abstract

Background.: Vulvar sarcomas, which comprise less than 2% of vulvar malignancies, are a heterogeneous group of tumors. Sub-categorization of these tumors makes treatment strategies difficult to evaluate. "Proximal" type epithelioid sarcoma (PES) and malignant rhabdoid tumor (MRT) describe a rare subset of vulvar malignancies which share histologic features and an aggressive clinical course. Case.: We describe a case of vulvar PES/MRT in a 35 year old female treated with radical excision, inguinal-femoral lymphadenectomy, and adjuvant radiation who remains without evidence of disease 40 months after surgery. We review all English language reports of vulvar PES or MRT, and compare treatments and outcomes. Conclusion.: Vulvar PES/MRT is a rare disease attended by high rates of local and distant relapse. Radical excision remains the foundation of treatment. The role of adjuvant therapy, and particularly radiation, remains unclear but merits consideration.

Original languageEnglish (US)
Pages (from-to)130-135
Number of pages6
JournalGynecologic oncology
Volume107
Issue number1
DOIs
StatePublished - Oct 2007

Keywords

  • Malignant rhabdoid tumor
  • Proximal epithelioid sarcoma
  • Radiation
  • Sarcoma
  • Treatment
  • Vulvar

Fingerprint Dive into the research topics of '"Proximal-type" epithelioid sarcoma vs. malignant rhabdoid tumor of the vulva: A case report, review of the literature, and an argument for consolidation'. Together they form a unique fingerprint.

Cite this