Basal cell carcinoma (BCC) is the most common human malignancy, representing 75% of nonmelanoma skin cancers.1 BCC is most frequently located on the sun-exposed skin areas of the head and neck, but appears in unexposed areas such as the perineal and anal regions in less than 1% of cases.2-3 Vulvar BCC is rare, accounting for approximately 3% of vulvar cancers;2 such lesions are often diagnosed late because they grow slowly and tend to be asymptomatic, with no specific physical findings.4
A 65-year-old Latino woman presented with a nodule on her left labium majus (Fig. 1). She had first noted the lesion 10 years ago, and it had grown slowly but constantly since then. She also reported a 20-year history of pruritus and occasional pain of the left labium majus. The patient had not received any previous diagnoses or treatments for the condition. There was no relevant medical history (the patient reported no history of cancer, sexually transmitted disease, human papilloma virus, irradiation, or tobacco use) or family history. Physical examination revealed a 3.5 × 3.0 cm oval, erythematous lesion with a central rough, fissured surface. All other physical examination findings were normal, with no inguinal lymphadenopathy noted.
Contact dermatoscopy (Dermalite DL3 Gen) showed one blurred cluster of arborising vessels on a pinkish background with white homogeneous areas and converging radial lines (Fig. 2). Based on histopathological examination of an incisional biopsy, the lesion was diagnosed as a BCC.
Vulvar BCC is a rare malignancy that mainly affects Caucasian women older than 70 years but can occur in younger patients of all races, with the youngest registered patient being a 20-year-old nulligravid African American woman.1,4 Vulvar BCC presents most frequently in the labia majora as an eroded pigmented plaque in 3% of the Caucasian population and up to 81% of Chinese patients;2,5 however, vulvar BCC may also present as multiple, bilateral, or disseminated lesions,3 or as ulcerated lesions in 28% of Caucasian patients.6
The most common etiology for BCC is ultraviolet radiation exposure; however, as the vulva is unexposed, the cause of vulvar BCC is unclear. Mutations in tumor suppressor and regulatory genes such as p53 are present in 50% of patients with BCC.4 The reported risk factors for BCC include Gorlin syndrome, chronic radiation, chronic arsenic exposure, xeroderma pigmentosum, and immuno-suppression.7 BCC has also been associated with lichen sclerosis,5 Paget disease,8 local trauma, scars, and advanced age.6,8
BCC has no specific features and can present as an asymptomatic lesion, pruritus, irritation, pain, discomfort, bleeding, or a palpable mass.3 Vulvar BCC is most frequently misdiagnosed as inflammatory diseases, such as eczema, psoriasis, and chronic infection (especially when accompanied by pruritus), but must also be differentiated from Bowen disease, Paget disease, squamous cell carcinoma, melanoma, melanocytic nevus, seborrheic keratosis, pemphigus vegetans, and other vulvar tumors.7,9 This makes the diagnosis of vulvar BCC complex, with an average diagnostic delay of 5 to 6 years,7 and an average lesion size of 2.1 cm.6 Furthermore, the width of the BCC may not be directly related to the depth of invasion.5 The diagnosis of BCC is enhanced by dermatoscopy, as the dermatoscopic characteristics of vulvar BCC are the same as those of other cutaneous forms, featuring blue ovoid nests and arborizing telangiectasia.3,9
The preferred treatment in most cases of BCC is wide surgical excision, although local recurrence is possible if the margins are positive.4 The margins cannot be easily observed with the naked eye, leading to positive margins after excision, with 25% of cases requiring a second intervention.10 Dermatoscopy may aid in determining tumor margins, but is not exact. Patients with more advanced or aggressive tumors may benefit from Mohs micrographic surgery, which enables the intraoperative examination of margins and provides relative certainty that the margins are negative with minimal sacrifice of normal tissue.4
. Fleury AC, Junkins-Hopkins JM, Diaz-Montes T. Vulvar basal cell carcinoma in a 20-year-old: case report and review of the literature. Gynecol Oncol Case Rep 2011;2 (1):26–27. doi: 10.1016/j.gynor. 2011.11.005.
. DeAmbrosis K, Nicklin J, Yong-Gee S. Basal cell carcinoma of the vulva: a report of four cases. Australas J Dermatol 2008;49 (4):213–215. doi: 10.1111/j.1440-0960.2008.00471.x.
. Cinotti E, Tonini G, Perrot JL, et al. Dermoscopic and reflectance confocal microscopy features of two cases of vulvar basal cell carcinoma. Dermatol Pract Concept 2018;8 (1):68–71. doi: 10.5826/dpc.0801a17.
. Mulayim N, Foster Silver D, Tolgay Ocal I, et al. Vulvar basal cell carcinoma: two unusual presentations and review of the literature. Gynecol Oncol 2002;85 (3):532–537. doi: 10.1006/gyno.2001.6582.
. Lui PC, Fan YS, Lau PP, et al. Vulvar basal cell carcinoma in China: a 13-year review. Am J Obstet Gynecol 2009;200 (5):514.e1–514.e5. doi: 10.1016/j. ajog.2008.12.004.
. de Giorgi V, Salvini C, Massi D, et al. Vulvar basal cell carcinoma: retrospective study and review of literature. Gynecol Oncol 2005;97 (1):192–194. doi: 10.1016/j.ygyno.2004.12.008.
. Caucanas M, Müller G, Vanhooteghem O. Vulvar basal cell carcinoma: report of a case involving the mucosa and review of the literature. Dermatol Reports 2011;3 (3):e37. doi: 10.4081/dr.2011.e37.
. Mulvany NJ, Rayoo M, Allen DG. Basal cell carcinoma of the vulva: a case series. Pathology 2012;44 (6):528–533. doi: 10.1097/PAT.0-b013e328357a001.
. Dobrosavljevic Vukojevic D, Djurisic I, Lukic S, et al. Dermatoscopy in vulvar basal cell carcinoma. J Eur Acad Dermatol Venereol 2017;31 (4):e180–180e181. doi: 10.1111/jdv.13895.
. de Giorgi V, Massi D, Mannone F, et al. Dermoscopy in vulvar basal cell carcinoma. Arch Dermatol 2007;143 (3):426–427. doi: 10.1001/arch-derm.143.3.426.