Surgical management of locally advanced adenoid cystic carcinoma of the lacrimal gland

PURPOSE: To review our experience with multidisciplinary surgical management of locally advanced adenoid cystic carcinoma of the lacrimal gland. METHODS: We present a case series of 7 patients with lacrimal gland adenoid cystic carcinoma treated at our institution between June 2001 and October 2003. Clinical records, histologic sections, and radiographic images were reviewed. RESULTS: The study included 3 men and 4 women (mean age at diagnosis, 44 years). All 7 patients underwent an orbital exenteration with bone removal. Five patients had an orbitectomy through a craniotomy approach and 2 patients had an exenteration through a fronto-orbito-zygomatic approach, all with removal of the bone of the superior and lateral wall. Six patients underwent reconstruction of the socket through the use of a vascularized flap. The surgical approach involved a neurosurgeon, an oculoplastic or head and neck surgeon, and a plastic surgeon. Six patients received postoperative radiation therapy. One patient with a recurrent tumor had already received radiation therapy, which precluded additional radiation therapy after surgical resection. The radiation field included the orbit and the skull base because all patients had evidence of perineural invasion. As of this writing, there have been no local recurrences. Five patients had development of distant metastases and died of disease, at follow-up times from 12 to 32 months after surgery. Two patients are alive without evidence of disease, both at 24 months' follow-up. CONCLUSIONS: Orbitectomy with bone removal may be indicated for achieving local and regional control in advanced cases of adenoid cystic carcinoma of the lacrimal gland. This surgery does not decrease the risk of distant metastasis. The cases in our series highlight the locally invasive and metastatic behavior of this cancer.