Mahanti S*, More V**, Chaudhari HK ***
(* Senior Resident, **Assistant Professor, ***Associate Professor, Head of Unit, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)
Basal cell carcinoma of the vulva is very rare. This entity in gynecological and dermatological oncology presents a diagnostic challenge in view of the late presentation of the patients, who are usually elderly. Here we have a case of a 72 year old woman, who presented to our outpatient department with complains of itching and irregular swelling in the genital area.
A 72 year old, postmenopausal woman presented to the gynecological outpatient department with complains of swelling and discoloration in the genital area for 1 month. She had no complaints of pain over the area, bleeding or associated abdominal pain. History of itching over the lesion was present. On examination, her vital parameters and systemic examination were stable and normal. On local examination, a 2x2 cm, dark pigmented, ulcerative lesion was noted over the superior aspect of the right labia majora, around 2 cm lateral to the midline. The margins of the lesion were irregular but there was no associated involvement of the clitoris, urethral orifice, labia minora. There was no palpable inguinal lymphadenopathy. On per abdomen examination, all quadrants were soft and non-tender and on per speculum examination, cervix and vagina appeared atrophic but healthy. On per vaginal examination, uterus was atrophic and fornices were free and not tender. Differential diagnosis at this stage included lichen sclerosus or lichen simplex atrophicus. She was referred to the dermatological outpatient department (OPD) for their expert opinion
In the dermatological OPD, the above clinical findings were noted and confirmed. Dermascopy that was performed was highly suggestive of basal cell carcinoma and hence biopsy of the lesion was advised. Punch biopsy was performed and histopathology report confirmed the diagnosis of basal cell carcinoma of the vulva. She was referred to onco-surgeon for further management of the same. They performed a wide local excision. She is presently following up with the oncosurgeons.
Basal cell carcinoma is one of the commonest malignancies involving the skin. But the predilection is for the sites exposed to sun, including the head and neck in view of role of ultraviolet rays in the pathogenesis of the same. Hence basal cell carcinoma of the vulva is an extremely rare entity accounting for less than 5 percent of all vulvar malignancies and less than 1 percent of all basal cell carcinomas.
Although the exact etio-pathogenesis is not known, exposure to ultraviolet radiation, chronic irritation of the vulvar region and exposure to arsenic are identified risk factors. Apart from these, genetic conditions such as xeroderma pigmentosum, nevoid basal cell carcinoma and mutations in p53 genes are also known to predispose to basal cell carcinoma of the vulva.
The diagnosis is often delayed because of the morphological similarity of the lesions to Paget’s disease of the vulva and lichen simplex atrophicus which are benign lesions. It is therefore recommended to perform incisional biopsies of all suspicious vulvar lesions especially in patients in the postmenopausal age group. Basal cell carcinoma diagnosis is often delayed in view of its similarity to other dermatological diagnosis such as eczema, psoriasis, seborrheic dermatitis and angiokeratoma.
Even though slow growing, the lesion should be examined with high clinical suspicion for malignancy especially in the elderly, for early diagnosis and timely treatment.
Treatment is fairly direct in the form of wide local excision of the lesion or Moh’s micrographic excision. A margin of 1cm around the lesion is usually considered adequate for excision of the lesion. In view of the slow growing and indolent course of the disease, wide local excision of the lesion is usually considered sufficient treatment. However, in some large invasive tumors with large lymph node involvement, selective lymphadenectomy maybe performed. Reconstruction of the excision site can be done with advancement of skin flaps. Patients who are considered surgically unfit may undergo radiotherapy.
Although extremely rare, basal cell carcinoma is a recognized entity in gynecological oncology with an indolent course and fairly direct mode of management. Clinical suspicion for malignancies should be especially high in vulvar lesions in the postmenopausal age group.
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Mahanti S, More V, Chaudhari HK. Basal Cell Carcinoma Of The Vulva. JPGO 2019. Vol 6 No. 8. Available from: https://www.jpgo.org/2019/08/basal-cell-carcinoma-of-vulva.html