Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature.
BACKGROUND
Peripheral primitive neuroectodermal tumor (pPNET) is aggressive and rare, comprising 1% of soft tissue sarcomas. Involvement of the reproductive tract is unusual.


METHODS
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.
Gostout
Bobbie S
BS
Section of Gynecologic Surgery, Mayo Clinic, Rochester, MN 55905, USA.
Lindor
Noralane M
NM
DiMarco
Connie S
CS
Peethambaram
Prema P
PP
Clayton
Amy C
AC
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018288:Carcinoma, Small Cell / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D010534:Peritoneal Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D014625:Vaginal Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery