Rare Vaginal Tumors in Young Females

Endodermal sinus tumor is a rare germ cell malignancy that is usually found in the ovary. This tumor secretes alpha-fetoprotein, which often is a useful tumor marker for monitoring patients with this neoplasm. It is usually found in infants and children, and approximately 20 cases have been reported as occurring in the vaginas of infants who were under 2 years of age. Patients generally present with complaints of bleeding or spotting from the vagina.

On physical examination, there is a friable red to pinkish-white polypoid tumor. This tumor is aggressive, and most patients have died. Therapy has involved surgery, radiation, and chemotherapy.

Young and Scully reported six patients who were disease free from 2 to 9 years after local therapy with operation, irradiation, or both, followed by systemic chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC). Copeland and colleagues reported similar results using the combination of chemotherapy and excision, and Collins and colleagues recently noted the regression of tumor with chemotherapy alone. In this report, a 5-month-old patient had regression of the tumor after VAC therapy.

Another rare tumor found in the vaginas of young females is sarcoma botryoides, or embryonal rhabdomyosarcoma. This tumor is usually found in children under 8 years of age. As with endodermal sinus tumor, the most common symptom has been vaginal bleeding. In 58 cases reviewed by Hilgers, the average age at onset of symptoms was 38.3 months. This tumor resembles clusters of grapes and forms multiple polypoid masses that are believed to begin in the subepithelial layers of the vagina and to rapidly expand, filling the vagina. Histologically, these tumors are identified by the presence of rhabdomyoblasts that may contain cross-striations.

Because of infiltration of the tumor under the vaginal epithelium, there is often a distinct subepithelial zone, called the cambium layer. The 5-year survival rate of these tumors in the past has ranged from 10 to 35%, and exenterative procedures have often been used. Hilgers reviewed the literature on pelvic exenterations in 21 cases of embryonal rhabdomyosarcoma and found that this form of therapy was ineffective in curing these patients. Effective control with less radical surgery has been achieved using multimodal treatment consisting of multiagent chemotherapy, VAC, combined with operation or radiation.

{embed="cr/stat-menu-vaginal"}

Hayes and colleagues recently reported 21 patients with vaginal rhabdomyosarcoma who received chemotherapy. In their series, 7 relapsed, with 5 of these 7 having had residual disease following incomplete resection. In 17 of 21 patients who received chemotherapy before surgery, a subsequent delayed excision could be performed. Data regarding the long-term survival of a large number of patients are not available, but such a combined approach appears to result in effective therapy with less mutilating surgery.