Superficial vulvectomy is appropriate to treat extensive and recurrent VIN 3 (147). The goal of the surgery is to extirpate all of the disease while preserving as much of the normal vulvar anatomy as possible. The anterior vulva and the clitoris should be preserved if possible. In some patients, the disease extends up the anus, which must be resected. An effort should be made to close the vulvar defect primarily, reserving the use of skin grafts for instances in which the vulvar defect cannot be closed because the resection is so extensive. Split-thickness skin grafts can be harvested from the thighs or buttocks, but the latter is more easily concealed (149).
Paget’s Disease Unlike squamous cell VIN 3, in which the histologic extent of disease correlates closely with the macroscopic lesion, Paget’s disease usually extends well beyond
Figure 19.22 Paget’s disease of the labium majus.
the gross lesion (150). This extension results in positive surgical margins and frequent local recurrence unless a wide local excision is performed (151). Underlying adenocarcinomas are apparent clinically, but this finding does not occur invariably; therefore, the underlying dermis should be removed for adequate histologic evaluation. For this reason, laser therapy is unsatisfactory in treating primary Paget’s disease. If underlying invasive carcinoma is present, it should be treated in the same manner as a squamous vulvar cancer. This treatment usually requires radical vulvectomy and at least an ipsilateral inguinal-femoral lymphadenectomy.
Recurrent lesions are almost always in situ, although there was at least one report of an underlying adenocarcinoma in recurrent Paget’s disease (143). It is reasonable to treat recurrent lesions with surgical excision.
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