Acta Dermato-Venereologica Issue 8, 2017 97-8CompleteContent | Page 18

SHORT COMMUNICATION 959 Sebaceous Carcinomas of the Skin: 24 Cases and a Literature Review Roger HABER 1 , Maxime BATTISTELLA 2 , Cecile PAGES 1 , Martine BAGOT 1,3 , Celeste LEBBE 1,3 and Nicole BASSET-SEGUIN 1,3 1 Department of Dermatology and Venereology, 2 Department of Pathology, Université Paris 7, Saint Louis Hospital, 1 Avenue Claude Vellefaux, FR-75010 Paris, France. E-mail: [email protected] Accepted Apr 27, 2017; Epub ahead of print Apr 27, 2017 Sebaceous carcinoma (SC) is a highly aggressive tumour arising from the sebaceous glands (1). SC is a rare neo­ plasm that represents 1% of all eyelid tumours and 4.7% of malignant epithelial eyelid tumours. The majority of SC are located in the head and neck region, primarily in the periocular area (75%), i.e. eyelid, orbit and conjunctiva, where sebaceous glands of Meibomius and Zeis are the most common (1–3). SC may occur sporadically or be part of Muir-Torre syndrome (MTS), a rare autosomal- dominant genodermatosis characterized by sebaceous neoplasms and one or more visceral malignancies (4). SC most commonly presents as a gradually enlarging, firm nodule or as an ulcerated papule or plaque in elderly individuals (1), and can mimic basal cell carcinoma, squamous cell carcinoma, pyogenic granuloma, metastatic tumour or benign adnexal tumours (1, 2). Diagnosis of SC is therefore often delayed (5, 6) and confirmed only by histology (1, 3, 5). The disease is classified as either ocular (OSC) or extraocular (EOSC) depending on the location of the primary lesion (1–3, 6). Heterogeneity in incidence patterns, associated cancer risks (3, 6, 7) and mortality (6, 7) have been reported between OSC and EOSC, but are still controversial. Standard surgical resection with wide margins and Mohs micrographic surgery are common treatments for SC (3, 8, 9). However, to date, there are no tumour-specific staging system or management guidelines for EOSC (5). The aim of this study was to determine the demographic, clinical and histopathological characteris- tics, treatment strategy and prognosis of SC in a series of patients and to compare these results with published data. METHODS All patients presenting to Saint Louis Hospital, Paris, a major skin cancer referral centre in France, from 1995 to 2015, with a confirmed histology of SC of the skin, were included in this retrospective study. Sex, age, race, immune status (immunode- pression in HIV and transplantation), family history, tumour loca- tion, tumour size, stage and extent of disease, pathology report, imaging techniques, time to diagnosis, lymph node involvement, metastasis, radiation use, surgery type and extent, chemotherapy and survival data were collected through patients charts. SC were classified as OSC when occurring in orbital sites (eyelid, orbit, and conjunctiva) or EOSC if elsewhere (lip, external ear, scalp or neck, trunk and upper and lower limb). Diagnosis was confirmed histologically in all cases (3). RESULTS Twenty-four patients with SC were identified between 1995 and 2015. Ten had MTS, 21 had EOSC (4 of whom had 2 primary SC) and 3 had OSC (Table SI 1 ). Median age at diagnosis was 68 years overall (range 44–94 years) and 64.5 years in cases of MTS. There was a slight pre- dominance of men (62.5%). Seventy-five percent of cases occurred in Caucasians. Risk factors of SC were present in 58% of cases and included MTS (41.6%, 10 cases) and immunosuppression (16.6%, 4 cases, of which one with MTS). No case of previous irradiation on the site of SC was noted. Family history of SC was found in 10 patients, 9 of whom had MTS. Most frequently involved skin sites were the nose (10 lesions, 35.7%) and the scalp/ neck (5 lesions, 17.8%). Time to diagnosis ranged from 1 to 5 years in 39.2% of cases and less than 1 year in the 4 cases of recurrent SC. SC presented mostly as a painless, pink or yellow, round nodule, or as a diffuse thickening or a pedunculated lesion (Fig. S1 1 ). Frequently associated malignancies included colorectal carcinoma (33%) and urogenital carcinoma (16.6%), all occurring in patients with MTS. Colonoscopy was performed in all cases of MTS. Lymph node sampling was performed in 2 cases because of aggressive clinical and histological features; pathology results were negative in all cases. There was only one case of lung metastasis that occurred in an immunocompromi- sed patient with MTS. Wide surgical excision was the major treatment in all cases. Margins of 5–6 mm were used only for small lesions (diameter < 6 mm), mostly located on the face, with no aggressive clinical or histological pattern. Margins of 2 cm and more were used for recurrent lesions and those with aggressive clinical/histological features. There was no case of amputation or use of adjuvant radiotherapy or of chemotherapy. In the patient with lung metastasis, treatment included wide surgical excision of the primary SC and pulmonary lobectomy. Median follow-up dura- tion was 3 years (range 3 months to 11 years). Overall, physical examination with or without regional lymph node ultrasonography was performed every 6 months for the first year then yearly. Lesions did not recur in 85.7% of cases; all recurrences (16.6%) occurred less than 5 years after initial diagnosis. There was no statistically signifi- cant relationship between initial tumour size and time to recurrence (Pearson correlation coefficient = 0.25). During the follow-up period, cause of death was attributable to colon cancer in one case; none was attributable to the SC. https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-2685 1 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2017 Acta Dermato-Venereologica. doi: 10.2340/00015555-2685 Acta Derm Venereol 2017; 97: 959–961