Sebaceous carcinoma of the vulva

Abstract

A 49 year old nullipara was admitted with vaginal bleeding. She had had a total abdominal hysterectomy at the age of 31 for uterine fibroids. She also suffered from hypertension and was on propranolol 40 mg twice daily. Her other medication included amitriptyline 25 mg three times daily and conjugated equine oestrogens 1.25 mg daily. She also had acquired partial lipodystrophy, which started at 8 years of age. On examination, she was found to have a papilloma on the right labium majus measuring 5 mm in diameter. Excision biopsy of this lesion was performed. The tumour was well circumscribed (Fig. 1) and was composed of mainly poorly differentiated mitotically active small basophilic cells mixed with clusters of cells showing differentiation into sebaceous cells (Fig. 2). No squamous or basal cell elements were present. The appearance was of a sebaceous carcinoma. One month later, she underwent a wide local excision of the area on the right vulva and vagina. The depth of the biopsy was down to the fascia and muscles of the urogenital diaphragm and measured 3 3 2 cm. Bilateral inguinal lymphadenectomy was also performed. Histology confirmed a small focus of residual sebaceous gland carcinoma in the biopsy specimen; excision was complete at all the margins of the resection. Two lymph nodes contained metastatic tumour. Computed tomography showed a mass in the left groin measuring 4 cm in diameter, which is thought to be due to a post-operative seroma or abscess. There were enlarged lymph nodes in the right external iliac region suggestive of malignancy. On the left side of the pelvis, there was also a complex cystic mass measuring 6 cm in diameter, consistent with an ovarian tumour. She underwent laparotomy three months after her initial admission. Left oophorectomy, pelvic lymph node biopsies, omentectomy and incision and drainage of an infected lymphocyst in the left groin were performed and peritoneal washings were taken. Histology and cytological examination of all these tissues showed no cancer. She received external beam radiotherapy (50 Gy in 25 fractions over five weeks) to both groins. Seven months after her initial admission, she developed a lump on the right side of the vulva measuring 5 mm in diameter. Excision biopsy showed a recurrence of her sebaceous gland carcinoma. Histological examination showed no lymphatic or vascular permeation, and excision was complete. Four months later, two further recurrences were excised from the skin of the right groin, and one year after her initial admission, she developed many small nodules in the right groin, the vulva and perianal region. Magnetic resonance imaging showed bilateral pelvic and para-aortic lymphadenopathy. She received palliative chemotherapy with epirubicin 50 mg/m, cisplatin 60 mg/m and 5-fluorouracil 200 mg/ m/24 hours every three weeks, with an excellent clinical response. At the time of writing we are still following her up.