Rhabdomyosarcomas are the most common soft tissue sarcomas in childhood, and 20% involve the pelvis or genitourinary tract (227). Dramatic gains were made in the treatment of these tumors during the past 20 years. Previously, radical pelvic surgery was the standard approach, but results were poor. A multimodal approach evolved, and survival rates improved significantly, with a corresponding decrease in morbidity. In a report of the experience of the Intergroup Rhabdomyosarcoma Study I and II (1972 to 1984) with primary tumors of the female genital tract, nine patients aged 1 to 19 years had primary vulvar tumors, and these tumors were often regarded as a form of Bartholin gland infection before biopsy (228). They were all managed with chemotherapy (vincristine,or actinomycin D and cyclophosphamide and doxorubicin), with or without radiotherapy. Wide local excision of the tumor, with or without inguinal-femoral lymphadenectomy, was carried out before or after the chemotherapy. Seven of the nine patients were free of disease 4 or more years from diagnosis, one patient was free of disease when lost to follow-up at 5 years, and one patient was alive with disease.
In addition to the previously mentioned tumors, a number of malignancies more commonly seen in other areas of the body may rarely occur as isolated vulvar tumors.
Lymphomas The genital tract may be involved primarily by malignant lymphomas but, more commonly, involvement is a manifestation of systemic disease. In the lower genital tract, the cervix is most often involved, followed by the vulva and the vagina (229). Most patients are in their third to sixth decades of life, and about three-fourths of the cases involve diffuse large cell or histiocytic non-Hodgkin’s lymphomas. The remainder are nodular or Burkitt’s lymphomas. Treatment is by surgical excision followed by chemotherapy and radiation or both, and the overall 5-year survival rate is about 70% (229).
Metastatic Tumors of the Vulva
Eight percent of vulvar tumors are metastatic. The most common primary site is the cervix, followed by the endometrium, kidney, and urethra. Most patients in whom vulvar metastases develop have advanced primary tumors when diagnosed, and in about one-fourth of the patients, the primary lesion and the vulvar metastasis are diagnosed simultaneously (235).
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