Lower genital tract rhabdomyosarcoma: case series and literature review.

摘要

OBJECTIVE: Rhabdomyosarcoma (RMS) of the lower genital tract is a rare tumor. It tends to occur in childhood in the vagina and in rare cases, RMS can originate in the uterine cervix, with a peak incidence in the 2nd decade. METHODS: A hospital-based tumor registry was searched to find all patients with female genital tract RMS, which were treated between 1999 and 2004. The medical records of all patients were reviewed. A single pathologist reviewed all pathologic specimens. RESULTS: Among the 1,528 patients with genital tract malignancies, six RMS were found: three vaginas, three cervixes. All patients presented with vaginal bleeding. Mean age of patients was 16(13-30). The lesions in vagina were clinically staged as stage I (2), stage II (1) and in cervix were stage I (2) and stage III(1). All of the patients were treated with surgery and adjuvant chemotherapy. One patient with cervical RMS was treated with adjuvant chemo radiation. Two patients with cervical RMS died from the large size and extent ofthe disease 9 and 11 months after diagnosis, but all patients with vaginal RMS remain alive after a mean follow-up of 38 months. CONCLUSION: Most patients present with vaginal bleeding and a palpable cervical or vaginal mass. While the optimal management of these tumors is uncertain, primary therapy with wide local excision and chemotherapy can result in prolonged survival and cure in patients with early stage RMS. Vaginal lesions have a better prognosis than cervical lesions. In patients with un-embrional RMS, large size of lesion, cervical origin and extent of disease, survival rates was decreased.