BACKGROUND. The reports in the literature indicate that extraocular sebaceous carcinoma (SC), in contrast to orbital SC, is less aggressive and rarely metastasizes. METHODS. Of 2422 epithelioma cases observed over 10 years, 8 (0.33%) resulted as histologically proven SCs, all of which were located in the head. RESULTS. The clinical behavior of these tumors was observed by following patients for an average period of 50 months. Three of the tumors originated from the orbital region and five from other areas of the head (extraocular). In one case, the SC was associated with renal carcinoma (Muir-Torre syndrome). None of the orbital tumors metastasized, whereas two of the five cases of extraocular carcinoma metastasized to the locoregional lymph nodes. CONCLUSION. It should be stressed that in this case study aggressive biologic behavior was observed in carcinoma arising from extraorbital areas.
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