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ABSTRACT Basal cell carcinoma (BCC) is the most common malignancy of the skin, but BCC of the vulva is a rare entity. BCC of vulva accounts for less than 5% of all vulvar neoplasms and less than 1% of all basal cell carcinomas. Vulvar basal cell carcinoma has a low propensity for metastatic spread but has a high chance of local recurrence after simple excision. We report a 50- year-old woman presenting with pruritus vulva. Physical examination revealed a 2.5 x 1.5 cm hyperpigmented plaque on the left labium majus. The histopathology was consistent with superficially invasive basal cell carcinoma. The patient underwent radical local excision including the clitoris and remained disease free at postsurgical follow-up after eight months. 

KEYWORDS Basal Cell Carcinoma, Radical Local Excision, Vulva.

INTRODUCTION: Basal cell carcinoma (BCC) is the most common skin cancer in humans and accounts for 70-80% of dermatological malignancies. It is known to occur on sun exposed skin. Vulvar and perianal regions are the non-sun exposed atypical sites.1,2 Vulvar BCC accounts for less than 1% of all BCCs and less than 5% of all vulvar carcinoma.2 Vulvar BCC was first described by Temesvary in 1926. Approximately, 300 cases have been reported till date.3,4,5 Although the aetiology of vulvar BCC is not deciphered, several predisposing factors like chronic irritation and infection, trauma, arsenic, radiotherapy and syphilis4 have been implicated in the pathogenesis.5,6 They are slow growing indolent tumours, but are locally invasive and destructive, necessitating adequate surgical excision. They rarely metastasise to inguinal lymph nodes and are known to have local recurrence as high as 20% in some reports,5,6 which is attributed to inadequate surgical resection.7 We present an unusual case of vulvar BCC located on left labium majus in a 50-year-old woman treated with radical local excision. CASE REPORT: A 50-year-old woman presented to the Dermatology Outpatient Department with complaints of pruritus vulva for past six months. On clinical examination by the Dermatologist, a hyperpigmented plaque was identified on the left labium majus and biopsy was performed as a part of initial evaluation. The punch biopsy was sent for histopathological examination and was reported as basal cell carcinoma. Following this, the patient was referred to Gynaecologic Oncology for further management. Physical examination revealed a well-demarcated hyperpigmented plaque measuring 2.5 x 1.5 cm on left labium majus (Figure 1). Three satellite lesions, each measuring approximately 0.5 cm were identified surrounding the main lesion. There was no obvious inguinal lymphadenopathy. Rest of the vulva, vagina and cervix appeared normal on colposcopic examination. Pelvic examination revealed no abnormality and conventional cervical Pap smear showed no intraepithelial lesion. As a part of surgical workup, the patient was tested and found to be positive for syphilis which was confirmed by a positive TPHA test. The patient underwent radical local excision of the tumour which included amputation of the clitoris to get 2 cm surgical clearance and disease free margin. Histopathologic examination of the radical excision specimen showed a 2.5 x 1.5 cm ulceroproliferative growth in the left labium majus. On microscopic examination, nests of basaloid cells were seen arising from the basal layer of epidermis and infiltrating the superficial papillary dermis. These cell clusters showed peripheral palisading and clefting around the tumour nests, a feature characteristic of BCC (Figure 2). The deep dermis was uninvolved and all the surgical margins were free of tumour. The patient is disease free at eight months of postsurgical follow-up.

CONCLUSION: Any chronic, persistent lesion in vulvar region especially in advanced ages should be subjected to biopsy as BCC has subtle symptoms and nonspecific presentation. Clinician should be vigilant in cutaneous examination as other mucocutaneous diseases like contact dermatitis, psoriasis, Bowen’s disease, Paget’s are more common on vulva and rare presentation of basal cell carcinomas in genital skin should be kept in mind. It is suggested that all cases of vulvar basal cell carcinomas be registered, treated and followed up in a Gynaecologic Oncology service, irrespective of the status of the margins of surgical specimen. The importance of longterm follow-up has to be highlighted.