Vulvar cancer is a rare disease with a bimodal age distribution. Risk factors include HPV infection in younger women and lichen sclerosus and planus in older women. The 2015 ISSVD terminology encompasses Vulvar High-grade Squamous Intraepithelial Lesions and Vulvar Intraepithelial Neoplasia, differentiated type underlining the two different etiologies of vulvar cancer.
Review of current scientific literature
No systematic screening is available and recognition of precursors and invasive vulvar cancer relies on the presence of vulvar symptoms and on the accuracy of healthcare provider inspecting the vulva. Clinical examination must include direct extension of vulvar cancer to adjacent structures (urethra, vagina and anus) and fixation to the bone. Preoperative lymphnode evaluation with positron emission tomography (PET) scan may be helpful in selected cases.
Stage of vulvar cancer defines both treatment options and prognosis. Vulvar cancer is staged surgically with depth of stromal invasion and involvement of inguino-femoral nodes being the most important prognostic factors.
Conservative surgical techniques are evolved both for vulvar and nodal surgery with the aim of minimizing morbidity maintaining disease free and overall survival similar to more extensive surgical approach.
With this intent surgical treatment of vulvar lesion shifted during last decades from “en bloc” cancer and inguinal nodes dissection toward local radical resection, defined as a surgical excision of the lesion with at least 1 cm free deep and lateral margins, and separated inguinal incisions for node dissection.
Appropriate surgical assessment of the inguinal lymph nodes is imperative because groin node recurrences are almost all fatal. In unifocal lesion, less than 4 cm and without clinically suspicious groin node a sentinel node biopsy may be, if negative, a less morbidity alternative to complete inguino-femoral node dissection. Only centers with appropriate surgical, nuclear medicine and pathological expertise are candidate to perform sentinel node biopsy as omission of involved nodes identification leads patients to lethal groin progression of cancer.
Adjuvant groin and pelvic radiation and in selected studies chemotherapy in addition to radiation depends on type, number and side of involved inguinal nodes.
Tailored neoadjuvant chemotherapy and radiation is used to treat advanced vulvar cancer to preserve anal, rectal and bladder function.
Treatment of recurrent disease is determined by the localization of recurrence and prior treatment.
Management of vulvar cancer should be individualized and requires an experienced, multidisciplinary team approach in an oncological center.