Grossly, the tumors have a cauliﬂowerlike appearance; microscopically, they contain multiple papillary fronds that lack the central connective tissue core that characterizes condylomata acuminata (Fig. 38.9). The gross and microscopic features of a verrucous carcinoma are very similar to those of the giant condyloma of Buschke-Loewenstein, and they probably represent the same disease entity (206). Adequate biopsy from the base of the lesion is required to differentiate a verrucous carcinoma from a benign condyloma acuminatum or a squamous cell carcinoma with a verrucous growth pattern.
Verrucous carcinomas usually occur in postmenopausal women, and they are slow-growing but locally destructive lesions. Even bone may be invaded. Metastasis to regional lymph nodes is rare but was reported (220). Radical local excision is the basic treatment, although any palpably suspicious groin nodes should be evaluated with fine-needle aspiration cytology or excisional biopsy. Usually, enlarged nodes will be caused by inﬂammatory hypertrophy (221). If the nodes contain metastases, radical vulvectomy and bilateral inguinal-femoral lymphadenectomy are indicated.
Several small studies failed to document any therapeutic advantage with radiation therapy (221). There is concern that radiation may induce anaplastic transformation with subsequent regional and distant metastasis (222). One study reported a corrected 5-year survival rate of 94% for 17 patients treated with surgery alone, compared with 42% for 7 patients treated with surgery and radiation (221). The latter patients had more advanced disease. If there is a recurrence, further surgical excision is the treatment of choice, which occasionally may necessitate some type of exenteration.
Sarcomas represent 1.5% of vulvar malignancies and constitute a heterogenous group of tumors (223). Leiomyosarcomas are the most common, and other histologic types include fibrosarcomas, neurofibrosarcomas, liposarcomas, rhabdomyosarcomas, angiosarcomas, epithelioid sarcomas, and malignant schwannomas.
Leiomyosarcomas usually appear as enlarging, often painful masses, usually in the labium majus. Smooth muscle tumors of the vulva that show at least three of the following four criteria should be regarded as sarcomas: (i) diameter greater than 5 cm, (ii) infiltrating margins, (iii) 5 or more mitotic figures per 10 high-power fields, (iv) moderate-to-severe cytological atypia (224). The absence of one, or even all, of these features does not guarantee against recurrence (225). Lymphatic metastases are uncommon, and radical local excision is the usual treatment.
Epithelioid sarcomas characteristically develop in the soft tissues of the extremities of young adults but rarely may occur on the vulva. In a description of two cases and review of three other reports, the authors concluded that these tumors might mimic a Bartholin cyst, leading to inadequate initial treatment (226). They believed that vulvar epithelioid sarcomas behave more aggressively than their extragenital counterparts, with four of the five patients dying of metastatic disease. They suggested that early recognition and wide excision should improve the prognosis.