Extraocular sebaceous carcinoma: a series of three cases with varied presentation

Abstract

Background: Extraocular sebaceous carcinomas are rare cutaneous malignancies that account for only about 25% of all sebaceous carcinomas. The most common site of occurrence is the head/neck region. They are aggressive neoplasms that possess metastatic potential to regional or distant sites. Wide local excision of the lesion with removal of regional lymph nodes is the usual mode of therapy. Methods: We present a series of three cases of sebaceous carcinomas occurring in extraocular sites outside the head and neck areas, like the axilla, chest wall, arm and thigh. One of these cases had an associated colonic carcinoma and constituted Muir-Torre syndrome (MTS). Another case presented with a skin nodule and regional nodal metastasis. The third case had a history of recurrent similar lesions at the same site. Conclusion: Although rare, extraocular sebaceous carcinomas are seen at varied sites and frequently pose problems in diagnosis. A longterm follow-up of these cases is warranted due to their aggressive behaviour, risks of recurrence, metastasis and the possibility of development of visceral malignancies.

Extraocular Sebaceous Carcinoma on the Chest Wall – A Case Report

“…Other reported locations include the external genitalia, the parotid and submandibular glands, the external auditory canal, the trunk, the extremities, the laryngeal and pharyngeal cavities, the uterine cervix and ovaries, the nasal vestibule and the oral mucosa [4,10]. So far only 180 cases are reported in extraocular sites [11].…”

“…[table/ Fig-3 (HMFG 1) [2], Human milk fat globule subclass 2 (HMFG 2) [2], Breast carcinoma antigen 255 (BCA 225) [2], Epithelial membrane antigen (EMA) [2,5,10]. They are negative for Carcinoembryonic antigen (CEA) [2], PAS [2], Alcian blue staining [2], S100 [5].…”

“…Incidence of local recurrence is 9% to 36% within 5 yrs [8]. Sites of distant metastasis include the liver, lungs, bones, and brain [6,8,10]. Incidence of metastasis is equal among both ocular and extra ocular sebaceous carcinoma [8].…”

“…Metastasis has been reported to occur as late as five years after the initial diagnosis, lending support to the continual surveillance of patients with sebaceous carcinoma. Around 20-25% of cases show distant metastasis [6,8,10].…”

“…Few studies say there is no sex predilection [3]. Few studies concluded women tend to be affected 57-77% more than men [8,10].…”