Adenoid cystic carcinoma of the lacrimal gland in a 14-year-old girl

The discovery of unilateral exophthalmia requires multidisciplinary care made all the more difficult in the case of a 14-year-old girl living in a tropical environment. The child, C.A., resident of the town of Djenne in Mali, was examined for nonpulsate, nonretractile, left lateral unilateral exophthalmia, painful upon palpation of a left upper-external mass under the orbital rim. The initial examination highlighted the existence of an intraorbital tumor with a moderate effect on visual function. In view of the slow and progressive development over 2 years, and with the assistance of an association, the child was transferred to a specialized medical structure where the tumorous nature was confirmed after surgical exeresis. The diagnosis was cylindroma, or adenoid cystic carcinoma. Neither chemotherapy nor radiation were used. At 15 months after surgery, the child showed no clinical sign of recurrence. Intraorbital tumors in children present diagnostic and therapeutic problems in countries with limited medical resources. Cylindromas or adenoid cystic carcinomas of the lachrymal gland are the second most common cause of epithelial tumors in this gland, which are characterized by a high degree of malignancy, a very high rate of recurrence, and a low survival rate at 5 years. In a tropical environment, diagnostic and therapeutic difficulties are compounded by limits to comprehensive care for the child.