DR [Dermatology Reports 2011; 3:e37] [page 81] Vulvar basal cell carcinoma: report of a case involving the mucosa and review of the literature Marie Caucanas,1 Gebhard Müller,2 Olivier Vanhooteghem1 1Department of Dermatology, Sainte Elisabeth Hospital, Namur; 2Department of Anatomopathology, IPG, Gosselies, Belgium Abstract We report the case of woman who presented a vulvar basal cell carcinoma (BCC) on the inner part of the labium majus, treated with local resection. Vulvar BCC is a rare cancer but can be long misdiagnosed due to a non-specific presentation. Though even rarer, BCC involving the mucosal side of the labium majus has to be considered in the differential diagnosis of the vulvar tumors. A complete excision with free margins is the treatment most recommended. Other recommendations include the early iden- tification of aggressive subtypes, which carry a greater risk of recurrence and spreading poten- tial as well as a long-term follow-up with exhaustive muco-cutaneous examination. Case Report A 57-year-old woman presented with an asymptomatic genital eroded mucosal lesion that she had noticed two years earlier (Figure 1). Physical examination showed a papilloma- tous lesion of 2.2X1.5 cm, located on the mucos- al surface of the left labium majus. There was visible pigmentation and bleeding. Lymph nodes areas remained free. The patient’s med- ical history was unremarkable. Differential diagnosis was to be made between pemphigus vegetans and tumors of the vulvar area. A resec- tion-biopsy was performed under local anesthe- sia and revealed a basal cell carcinoma of com- pact, nodular and pigmented type, with erosion on surface. All margins of excision were free of disease. Follow-up to date showed no evidence of recurrent or metastatic carcinoma. Discussion Basal cell carcinoma (BCC) of the vulva rep- resents 2-3 % of the vulvar cancers and less than 1% of all BCC. Out of around 250 cases published so far, only twenty cases of BCC of the clitoris, the labia minora or the medial non-hair bearing aspect of the labia majora have been described.1 Review of the literature shows that, considering other mucosas, four cases related to the buccal mucosa have been published.2-5 To our knowledge, no case involv- ing the glans is reported. In the literature, vul- var BCC is commonly reported to affect white women, mostly in the post-menopausal period6 though some cases affecting younger women have been described.1 Diagnosis is difficult as presentation and clinical manifestations are unspecific. As a result, the delay for diagnosis reaches 5 to 6 years on average.1 Complaints mostly range from the discovery of an asympto- matic labial lesion to the evaluation of pruri- tus, pain or bleeding. Clinical manifestations are diverse and do not usually suggest BCC, lacking characteristic pearly and telangectasic aspects.1 They are showing an exophytic, ulcer- ated, pedunculate, infiltrating, nodular or pig- mented lesion, mostly located on the non- mucosal surface of the labia majora.7 Differential diagnosis has to be made between pemphigus vegetans and tumors of the vulva (Table 1).1,6,8-15 BCC of the vulva is not located on a surface exposed to UV and therefore other risk factors need to be determined. To date, there hasn’t been any evidence of clearly iden- tified risk factors, especially for BCC involving mucosa. Possible associations remain prior radiation therapy1,16 and previous trauma such as a burn or a scar.16 Common risk factors to cutaneous BCC have to be considered: Gorlin syndrome,17,18 chronic radiation,1,16,19 chronic arsenic exposure20, mutations in p5321, xero- derma pigmentosum.11 Immunosuppressive medication has been suggested.16 A few cases have been reported in association with pre- existing lesion: lichen sclerosis et atrophi- cus,22 Paget’s disease,23 multiple tumors of the follicular infundibulum.1 Biopsy of any suspect lesion is widely rec- ommended.7,16,24 Once diagnosis is confirmed, conservative surgery is most indicated, with free margins resection.1,25-27 Some cases of relapses have been reported and are possibly due to inadequate margins. Local recurrence varies from 0-25% in published reports1 with an average of 10-20%.7 The aggressiveness and recurrence of BCC vary according to histologi- cal pattern.7 Tumors of the morphea-like (nodular, sclerosing), metatypical (basosqua- mous), adenocystic or infiltrative types are more aggressive, leading to a higher rate of recurrences. An aggressive BCC is associated with often deep local infiltration and occasion- al perineural extension.7 Several cases of metastazing BCC have been reported with an incidence of 0.0028-0.1% and with a mean time from initial presentation reaching 9 years.7 These cases raise a challenging differential diagnosis in which it can be difficult to distin- guish BCC from adnexal tumors. Eventually, BCC may be associated with another vulvar tumor, such as melanoma or epidermoid carci- noma.1 Most reported associated cancer with BCC of the vulva is uterine neck cancer (UNC).1 Considering the role of HPV in the genesis of UNC, a few studies have assessed the presence of HPV in genital BCC on small Dermatology Reports 2011; volume 3:e37 Correspondence: Olivier Vanhooteghem, Depart- ment of Dermatology, Sainte Elisabeth Hospital, B - 5000, Namur, Belgium. E-mail: ovanhooteghem@hotmail.com Key words: vulvar basal cell carcinoma. Received for publication: 3 August 2011. Accepted for publication: 31 August 2011 This work is licensed under a Creative Commons Attribution NonCommercial 3.0 License (CC BY- NC 3.0). ©Copyright M. Caucanas, G. Müller and O. Vanhooteghem, 2011 Licensee PAGEPress, Italy Dermatology Reports 2011; 3:e37 doi:10.4081/dr.2011.e37 Figure 1. Eroded and pigmented papillo- matous lesion located on the mucosal sur- face of the left labium majus. Table 1. Vulvar cancers. Squamous-cell cancers 90% Non-squamous tumors 10% Malignant melanoma Bartholin’s gland carcinoma Verrucous carcinoma Paget’s disease Adenosquamous carcinoma Basal-cell carcinoma Sarcoma Leiomyosarcoma Malignant fibrous histiocytoma Dermatofibrosarcoma protuberans Kaposi’s sarcoma Metastatic malignant disease Lymphoma of the vulva Merckel-cell cancer No n- co mm er cia l u se on ly [page 82] [Dermatology Reports 2011; 3:e37] series of patients and with uneven results.1,28,29 Yet the implication of HPV in the BCC patho- genesis has not been proved. Most authors underline the fact that there is a possible sig- nificant morbidity and occasional mortality if a lesion such as BCC is neglected or improperly treated.19 Considering the rates of relapse and possi- ble aggressiveness of BCC, a close long-term follow-up is essential in order to decrease skin cancer morbidity through early detection and treatment.17 References 1. Mateus C, Fortier-Beaulieu M, Lhomme C, et al. 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