The relation of human papilloma virus (HPV) infection to the development of cervical carcinoma suggests that a similar process may contribute to vulvar neoplasia (25) but additional epigenetic events (26) may be active in the progression to vulvar neoplasia. HPV-mediated transformation of human epithelial cells has been recognized as a multistep process resulting from the deregulated transcription of the viral oncogenes, E6 and E7, in proliferating cells. Interference with cell cycle regulators induces genetic instability and oncogenic alterations may lead to a malignant pheno-type with tumor cell immortalization and inactivation of tumor suppressor genes. But, it has been suggested that there are two pathways leading to the development of SCC of the vulva (27). As with cervical carcinoma, one route is HPV dependent with premalignant changes of vulvar intraepithelial neoplasia analogous to cervical disease. But, an HPV-independent genesis without high-risk HPV seems to occur, although loss of or occult HPV may confuse the issue.
The diagnosis of vulvar dysplasia or carcinoma in situ is usually the result of a biopsy provoked by abnormal appearance to vulvar skin with or without symptoms. The risk of progression to invasive carcinoma is not established but local ablation is generally recommended. Options for treatment include surgical excision (wide local excision) or other tissue destruction techniques such as cryosurgery, laser vaporization, or topical chemotherapy, all of which include the side effects of treatment and the possibility of incomplete removal (28-33).
Invasive squamous cell cancer (SCC) of the vulva is usually identified on direct inspection but requires biopsy confirmation (34). Sentinel node sampling is gaining acceptance in the evaluation and management of the extent of disease (35-38). Surgical excision has been the standard treatment with the goal of removing the primary tumor and extending the surgery to include the primary drainage in the groin nodes. For disease extending to Cloquet's node or to the deep pelvic nodes, radiation therapy is generally added. Combined therapy including conventional surgery or cryosurgery or other local destructive therapy for the primary tumor has been used with radiation therapy added for control of nodal disease (34,39-42) and the role of chemotherapy in conjunction with other modalities has been studied (43,44).
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