Sarcomas of the Vulva and Vagina

Sarcomas are a rare and heterogeneous group of malignant tumors arising from mesenchymal tissue. This chapter reviews vulvar and vaginal sarcomas, as well as aggressive angiomyxoma, a locally invasive mesenchymal tumor which can arise from the lower genital tract. Following biopsy of the primary lesion and confirmation of diagnosis of primary vulvar or vaginal sarcoma, a comprehensive general physical and pelvic examination is recommended with focused attention to measurement of the diameter of the primary tumor, palpation of regional lymph nodes, and assessment of direct tumor extension to adjacent structures. The histologic diagnosis of these rare tumors can be challenging and may require expert review. Surgery remains the mainstay of treatment. However, these patients should be evaluated by a multidisciplinary team in order to optimize treatment and minimize toxicity.