For patients with more advanced T2 lesions, management consists of radical vulvectomy and/or chemoradiation therapy, as discussed below. When the disease involves the distal urethra or anus, partial resection of these organs is required. Alternatively, it is often preferable to give preoperative radiation therapy with chemosensitization to allow for a less radical resection (see below).
Closure of Large Defects
After radical local excision, primary closure without tension can be accomplished for smaller defects. If an extensive dissection is required to treat a large primary lesion, a number of options are available to repair the defect: 1. An area may be left open to granulate, which it will usually do over a period of 6 to 8 weeks (88).
Figure 38.4 Small (T1) vulvar carcinoma at the posterior fourchette. (From Berek JS, Hacker NF. Berek & Hacker’s Gynecologic Oncology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2010:547, with permission.)
Advanced Disease: Large T2 and T3 Primary Tumors 2.
Full-thickness skin ﬂaps may be devised (89–92). The rhomboid ﬂap is best suited to covering large defects of the posterior vulva, whereas for lateral defects, a mons pubis pedicle ﬂap is advocated (89,92).
3.
Myocutaneous ﬂaps may be developed to cover the defect. Unilateral or bilateral gracilis myocutaneous grafts are useful when an extensive area from the mons pubis to the perianal area was resected. Because the graft brings a new blood supply to the area, it is particularly applicable if the vulva is poorly vascularized from prior surgical resection or radiation (93).
4.
If extensive defects exist in the groin and vulva, the tensor fascia lata myocutaneous graft may be the most applicable (94).
To achieve primary surgical clearance for tumors involving the upper urethra, anus, rectum, or rectovaginal septum, pelvic exenteration is needed in addition to radical vulvectomy and inguinal-femoral lymphadenectomy, which carries an extremely high physical and psychological morbidity (95,96). Reported 5-year survival rates with this approach are about 50% (97– 100). For many of these patients, a combined approach of surgery and radiation therapy offers improved survival and reduced morbidity and is the preferred treatment approach. Numerous small prospective and retrospective series report on the use of external beam radiation, often with concomitant chemotherapy to shrink the primary tumor. Reported initial response rates to chemoradiation are 80% to 90%, and operability is achieved in 63% to 92% of cases (101–108).
It is important that this chemoradiation is followed by a more limited resection of the tumor bed on an individualized basis. About one-half of the specimens will contain residual tumor, and local relapse rates are as high as 50% to 79% with external radiation alone (with or without concomitant chemotherapy), emphasizing the need for a combined approach that involves radiation and surgery (109–112).