Six cases of a rare entity, lacrimal gland
malignant mixed tumor (
MMT), or
carcinoma arising in benign mixed
tumor (BMT), are presented. Clinically, 2 patients had a long history of orbital protrusion, which recently had progressed more rapidly, In 2 other cases, the
carcinomas evolved after recurrences of BMT. Two other patients experienced the recent onset of symptoms, without a known
tumor previously having been present. Clinical clues suggesting a
malignant neoplasm of a lacrimal gland include 1) recent rapid onset of symptoms (orbital protrusion, eyelid swelling; 2) presence of
pain; and 3) radiographic studies showing bone destruction. In each of our cases, histologic examination demonstrated a BMT as well as a
carcinoma (undifferentiated or poorly differentiated
adenocarcinoma; in 1 case, areas of
adenoid cystic carcinoma were identified). Problems in histopathologic diagnosis are explored. (BMT, recurrent BMT, in situ
carcinoma in BMT and
MMT are differentiated.) The management of lacrimal gland
tumors is discussed. For a suspected
malignant neoplasm, a biopsy through the eye lid to establish the diagnosis is recommended. Radical orbital exenteration should be performed for
malignant mixed tumors, which have a poor prognosis.