The most common benign and malignant mesenchymal tumors of the vagina in adult women are smooth muscle tumors (229). Vaginal sarcomas are usually fibrosarcomas or leiomyosarcomas and are extremely rare. Radical local excision, followed by adjuvant chemotherapy or radiation therapy, is the indicated treatment.
The most common malignant mesenchymal tumor of the vagina in children and infants is botryoid rhabdomyosarcoma (Fig. 36.11). Botryoid sarcoma is usually found in the vagina during infancy and early childhood, in the cervix during the reproductive years, and in the corpus uteri during postmenopausal years. Preoperative chemotherapy with vincristine, actinomycin D, and cyclophosphamide, followed by conservative surgery or radiation, has improved survival.
Figure 36.11 Embryonal rhabdomyosarcoma of the vagina (botryoid sarcoma). This lesion consists of primitive mesenchymal cells and rhabdomyoblasts, which have abundant eosinophilic cytoplasm. With further differentiation, cross striations may become evident.
Treatment Treatment selection is based on the clinical examination, CT scan results, chest radiography results, age, and condition of the patient. PET scans may give more accurate information about disease spread than MRI or CT scan alone (230). Most tumors are treated by radiation therapy. Surgery is limited to selected cases. These are as follows: 1.
Women with stage I disease involving the upper posterior vagina may be treated by radical vaginectomy and pelvic lymphadenectomy. If the uterus is in situ,itis removed as a radical hysterectomy specimen. When margins are clear and lymph nodes are negative, no additional therapy is necessary.
2.
Patients with stage IV disease with either rectovaginal or vesicovaginal fistula may be candidates for primary pelvic exenteration with pelvic and para-aortic lymphadenectomy (225). Low rectal anastomosis, continent urinary diversion, and vaginal reconstruction are indicated and are more successful in these nonirradiated patients than in patients who received prior radiation therapy.
3.
Women with central pelvic recurrence after radiation therapy are candidates for pelvic exenteration similar to that used for cervical cancer.
4.
Surgical staging with resection of enlarged lymph nodes followed by radiation therapy may improve the control of pelvic disease.
Radiation therapy is the treatment of choice for all patients except those described previously. Small superficial lesions may be treated with intracavitary radiation alone (225). Larger, thicker lesions should be treated first with external teletherapy to decrease tumor volume and to treat the regional pelvic nodes, followed by intracavitary and interstitial therapy to deliver a high dose to the primary tumor (218,226). If the uterus is intact and the lesion involves the upper vagina, an intrauterine tandem and ovoids can be used. If the uterus was previously removed, a vaginal cylinder may be used for superficial irradiation. When brachytherapy
Table 36.7 Primary Vaginal Carcinoma: 5-Year Survival