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


METHODS
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.


CONCLUSIONS
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.
Argenta
Peter A
PA
Division of Gynecologic Oncology, Department of Obstetrics, Gynecology, and Women's Health, University of Minnesota, MN, USA. argenta@umn.edu
Thomas
Sajeena
S
Chura
Justin C
JC
D000328:Adult; D005260:Female; D006801:Humans; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D018335:Rhabdoid Tumor / Q000473:pathology / Q000628:therapy*; D012509:Sarcoma / Q000473:pathology / Q000628:therapy*; D014846:Vulvar Neoplasms / Q000473:pathology / Q000628:therapy*