Standard treatment for early disease consists of wide radical excision of the primary tumor with sentinel lymph node (SLN) biopsy and/or inguinal lymphadenectomy.
Advanced disease is often managed with adjuvant/neoadjuvant radiation and/or chemotherapy.
Surgical therapy options include:
- stage IA, local regional squamous cell carcinoma of the vulva. Microinvasive carcinomas (<2 cm size and <1 mm stromal invasion) are treated with wide local excision with at least a 1 cm free margin. Local recurrence and lymph node metastases are rare in these cancers and therefore lymphadenectomy is generally not recommended as part of the upfront surgical procedure
- stages IB and II cancers were treated with radical vulvectomy with bilateral inguinofemoral lymphadenectomy (the en-bloc 'butterfly' or 'long horn' resection)
- radical wide local excision (1-2-cm margin) of the primary tumor has generally replaced the radical vulvectomy
- bilateral surgical evaluation of inguinofemoral lymph nodes is necessary for patients with tumors larger than 2 cm in diameter, more than 5 mm in depth, any positive ipsilateral nodes, and midline or bilateral lesions
- if nodes are clinically positive, some suggest that a complete lymphadenectomy should be avoided since full groin dissection followed by adjuvant radiotherapy may result in severe lymphedema
- radical vulvectomy combined with partial or total pelvic exenteration remains an option for patients with locally advanced and clinically resectable lesions.
The relative merits of exenterative surgery must be weighed carefully as most patients are elderly. Mortality in radical surgery may approach 13%. Morbidity is mainly due to haemorrhage and sepsis.
Radiotherapy has been used as an adjunct to surgery for many years despite the relative radio-insensitivity of vulval carcinoma. The combination of radiotherapy and radical vulvectomy has been shown to give a better prognosis than exenterative sugery and radical vulvectomy in the treatment of advanced or recurrent disease.
Chemotherapy may be used before treatment to reduce the tumour bulk.
- chemotherapy - mitomycin and fluorouracil
- role of chemotherapy in the management of patients with invasive squamous cell cancer of the vulva is still limited; however, chemotherapy has been progressively integrated into the treatment modalities over the last two decades
- investigators have examined neoadjuvant chemoradiation in patients with locally advanced vulvar cancer to render them operable or to reduce the radicality of the surgical procedure
- most frequently used drug combinations with radiation are 5-fluorouracil (5-FU)/cisplatin and 5-FU/mitomycin C. The most common toxicity noted with these treatments was skin toxicity from the radiation component
Notes:
- diagnosis of vulvar cancer is made by biopsy
- with advanced primary lesions, cystourethroscopy, proctoscopy and imaging modalities such as computerized tomography (CT), magnetic resonance imaging (MRI) or positron emission tomography (PET) can be utilized to assist in staging, which may assist in the preoperative diagnosis of metastatic disease and are more sensitive than physical examination
- lymph node metastasis is the most important prognostic factor and PET CT has been shown to be a relatively insensitive but highly specific modality in predicting lymph node metastasis
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