Extensive pigmented vulvar basal-cell carcinoma presenting as pruritus in an elderly womanDept. of Dermatology, Ed. Herriot Hospital (Pav. R), Lyon, France. email@example.com
Jean Kanitakis MD, Elise Arbona-Vidal MD, Michel Faure MD
Dermatology Online Journal 17 (1): 8
Basal cell carcinoma (BCC), the most common human cancer, is seldom seen in the genital area. We present a case of an extensive pigmented BCC that developed on the mucosal surface of the vulva of an elderly woman and briefly review the relevant literature on vulvar BCC.
An 87-year-old woman sought advice for vulvar pruritus that had been present for several weeks prior to consultation. Clinical examination disclosed a well-demarcated erythematous, slightly infiltrated plaque with a partially hyperpigmented border circumferentially surrounding the vaginal introitus (Figure 1). The patient’s medical history was significant for adult-onset diabetes mellitus and two basal cell carcinomas of the face that had been excised one year before. Clinical diagnoses of the vulvar lesion included pigmented Bowen disease, Paget disease, and mucous membrane lichen planus.
Histopathologic examination of three biopsies taken from different areas of the lesion showed a similar picture: under a thinned epithelium, a dermal tumor was found, made up of confluent nodules consisting of basophilic cells, assuming a palisaded arrangement at their periphery (Figure 2). Melanin deposits were seen within tumor nodules and within dermal melanophages surrounding the tumor. These features were diagnostic of pigmented nodular BCC. Search for HPV by immunohistochemistry on tissue sections and by PCR after DNA extraction, using a combination of probes detecting both cutaneous and mucosal HPV types, proved negative. A superficial vulvectomy was proposed to the patient, but she was lost to follow up.
Basal cell carcinoma (BCC) is the most common human cancer. It develops predominantly on the head and neck (75%) and more rarely on the trunk and limbs. BCC very rarely develops on the genital area (1% of all cases), including the vulva. About 300 cases of vulvar BCC have been published in the literature to date [1-45].
Basal cell carcinoma accounts for 1-8 percent of all vulvar carcinomas [1, 3, 5, 10, 11, 14, 16, 31, 33, 35, 38]. It affects elderly women (mean age 68 years, range 34-92). The great majority of patients are Caucasians, as was our patient; a few cases have been reported in black women [5, 10, 15, 30] and in Asian women from Thailand , Japan  and China . The mean delay of diagnosis (5-6 years) is long and may reach 20 years  because of the location of the tumor, which prevents patients from seeking consultation and the frequent misdiagnosis. Vulvar BCC often causes symptoms such as discomfort, pain, bleeding, and pruritus that may be the presenting sign . The clinical aspects vary. Basal cell carcinomas may present as vegetating, ulcerated, pedunculated, infiltrated, nodular, pigmented, or depigmented lesions. They develop on the cutaneous (and less often the inner surface) of labia majora and, more rarely as in our patient, on the labia minora and the clitoris [2, 8]. The tumors may be bilateral , as in our patient, or multifocal.
The diagnosis of vulvar BCC is seldom made clinically. Basal cell carcinomas are usually misdiagnosed as Bowen disease, Paget disease, squamous cell carcinoma, leukoplakia, lichen planus, lichen sclerosus, melanocytic nevus, or melanoma. The correct diagnosis rests on the histological examination. Histopathologically, vulvar BCCs do not show noticeable differences from their cutaneous counterparts . They may occur as mixed tumors associated with squamous cell carcinoma [3, 16, 26, 39, 39], Paget disease , melanoma [38, 39], or in association with lichen sclerosus . BCCs should be differentiated from adenoid cystic (basal cell) carcinomas of the vestibular glands that may behave more aggressively .
The pathogenesis of vulvar BCC is unknown. Risk factors include chronic exposure to arsenic , basal cell nevus syndrome , local trauma , chronic irritation, and X-ray treatment [26, 40]. The direct role of UV radiation is obviously unlikely, even though several patients with vulvar BCCs have similar tumors on sun-exposed sites, as in our patient. Conversely, the fact that the genital region is sun-protected, may help account for the rarity of BCCs developing in this area. On the other hand HPV types that seem to play a co-carcinogenic role in non-melanoma skin cancers , have only exceptionally been detected in genital BCCs [26, 47-50]. In our patient, a search for HPV proved negative, in accordance with most previous studies.
The treatment of vulvar BCC is surgical, by partial or total vulvectomy, although conservative excision with histologic examination of the margins may be adequate. Mohs micrographic surgery has been also used [28, 37, 40]. If surgery is contraindicated or impossible, X-ray treatment is an alternative option. Local immunomodulators (namely imiquimod), alone or combined with surgery  could also be effective for superficial tumors, although tolerance of this treatment in this body area could understandably be poor.
The growth of vulvar BCCs is usually slow. As a rule, the prognosis is favorable provided the lesions are recognized early and completely excised. However, since this is not always the case, vulvar BCCs may behave aggressively, with local recurrences and metastases to the lymph-nodes [16, 19, 29], the skin , and the skeleton  leading to a fatal outcome [16, 26, 30]. Therefore, early diagnosis is important in order to spare patients unfavorable results.
In conclusion, any chronic, persistent vulvar lesion should be subjected to histological examination. Even though other mucocutaneous diseases are more common on the vulva (e.g., contact dermatitis, psoriasis inversa, fungal infections, Bowen disease, bowenoid papulosis, and Paget disease), the diagnosis of BCC should be considered for chronic vulvar growths, particularly in elderly women.
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