Abstract

Adenoid cystic carcinoma of the lacrimal gland is rarely reported in Japan compared with other countries in Europe and the U.S.A. Here is a report of this rare case. A 26-year-old female had complained of left visual disturbance and left exophthalmos with orbital pain. CT scan and MRI revealed a lacrimal gland tumor in that eye. The tumor was removed via a left frontal craniotomy, and diagnosed as adenoid cystic carcinoma. After operation, she was locally irradiated with a total dose of 5,000 rads using 15 MeV Lineac source. Chemotherapy was started with 5-FU after radiation therapy. An abnormal uptake was observed in the sternum in the bone scintigraphy performed after operation, suggesting metastasis. Visual disturbance improved and exophthalmos and orbital pain disappeared. At the last follow-up at 8 months after operation, she had no signs and symptoms of recurrence or metastasis. Adenoid cystic carcinoma is reported to be the most frequent among the malignant tumors of the lacrimal gland in the Western countries. Prognosis is generally unfavorable with the mortality of 45% to 70%. The 5-year and 10-year survival rate are 45% and 25%, respectively. The mean post-operative survival time is 4.5 years. Since the tumor grows infiltratively into the surrounding tissues, radical excision with total exenteration of the orbit is the treatment of choice. However, it is a difficult decision whether or not to remove the eye with the intact visual function. As for operation, an approach from above through the frontal craniotomy provides a wider operative field and an easier access to the tumor than the lateral approach employed by ophthalmologists.