Frequency and clinical features of visceral malignancy in a consecutive case series of patients with periocular sebaceous gland carcinoma

Purpose: To report the frequency and clinical features of internal visceral malignancy in an unselected series of patients with sebaceous gland carcinoma (SGC). Methods: A non-comparative retrospective case series of consecutive patients with a histologically proven diagnosis of periocular sebaceous cell carcinoma treated at the University Eye Hospital, University of Erlangen-Nurnberg between 1981 and 2000. Results: Twenty-three patients were identified, each with one tumor. Men and women were almost equally affected with a mean age at diagnosis of 62 years (range 37-83 years). The majority of tumors were located in the upper lid, but five tumors were situated in the lower lid or caruncle. In 22 of the 23 patients the diagnosis at referral was either incorrect or not suspected. In all cases, the SGC was treated by surgical excision. In the systems review, 11 of the 23 patients (48%) had evidence of previous systemic malignancy, the most common being colorectal cancer followed by liver and bronchial carcinoma. Forty-five percent of patients with SGC and internal visceral malignancy eventually died as a result of their tumors. Conclusion: Periocular SGC remains an underdiagnosed entity for which a high level of clinical suspicion is critical for early recognition. Almost half the patients additionally had an internal visceral malignancy. Internal visceral malignancy in unselected patients with SGC may be more common and have a more guarded prognosis than has been appreciated.