Primary infiltrating signet ring carcinoma of the eyelids

A 61-year old man presented with a five-year history of a swelling initially developing in his right lower lid that progressed to involve the lateral canthal skin and eventually the upper lid and anterior orbit. He was discovered to have an infiltrating, poorly differentiated, mucin-producing carcinoma. Systemic work-up failed to disclose a visceral malignancy, and it was concluded that his tumor was primary in the lids, arising from an adnexal sweat gland. Three other reports in the literature also share almost identical clinical and pathologic features, in that all of the earlier reports dealt with middle-aged or elderly men who had diffusely indurated lids. Histopathologically, the tumor cells grow diffusely in a sclerotic stroma, and resemble the "histiocytoid" variant of metastatic breast carcinoma to the lids in women. Ultrastructural studies in our case point toward an apocrine origin, although earlier authors have favored an eccrine origin. Despite its indolent clinical course, the tumor is capable of producing regional and distant metastases on long-term follow-up. Complete local excision, possibly necessitating radical surgery, is probably the preferred method of treatment, but local radiotherapy may have a beneficial effect in retarding spread of the disease.