Size and location of the tumor are important in planning treatment.

Stage 1 tumors less than 2 cm in size, localized to the lateral vulva, defined as 2 cm from a midline structure, can undergo radical partial vulvectomy (Fig. 2.5).

Larger stage 1 tumors that involve most of the vulva but confined to one side may undergo a radical hemivulvectomy (Fig. 2.6).

Tumors localized to the perineum may also undergo radical local excision (Fig. 2.7).

Tumors occupying both sides of the vulva will need a radical complete vulvectomy. For anterior lesions the perineum may be spared but greater than 80% of the vulva will be removed.

Stage 2 tumors are those extending to the lower one-third of the vagina, urethra, or anus. They are managed as follows:

Lesions extending to the anus or rectum that would require removal of the anus should receive sphincter-sparing radiation therapy.

Medical comorbidities may preclude radical resection. Radiation therapy plus radiation-sensitizing doses of chemotherapy followed by biopsy of the tumor bed is indicated. If there is persistent disease, then a smaller resection may be possible.

Patients with extension of the tumor into the vagina, bladder, or anus should undergo radiation therapy and chemotherapy in an effort to prevent a total pelvic exenteration.

If the tumor persists after 4 months, then a pelvic exenteration may be necessary.