Survey of orbital tumors at a comprehensive cancer center in the United States

Abstract

The purpose of this study was to evaluate the frequencies of various types of orbital lesions seen at a comprehensive cancer center in the United States. In this retrospective case series, we reviewed the medical records of 268 consecutive patients referred to our orbital oncology service for evaluation of an orbital mass between November 1998 and February 2009. Each orbital lesion was documented by CT and/or MRI, and in most cases diagnosis was established with a tissue biopsy. Patients who were seen for second opinions and had inadequate follow-up data were excluded, as were patients with thyroid eye disease or orbital hemorrhage. The study included 134 men and 134 women aged 1 to 89 years at diagnosis (median, 55 years). Follow-up ranged from 0.06 to 192 months (median, 15 months). Of the tumors, 171 (64%) were primary orbital, 69 (26%) were secondary orbital, and 28 (10%) were metastatic tumors. Lesion types were as follows: secondary orbital tumors, 69 (26%); lymphoproliferative lesions, 68 (25%); metastases, 28 (10%); epithelial lacrimal gland tumors, 27 (10%); inflammatory conditions, 21 (8%); vascular lesions, 20 (7%); mesenchymal tumors, 18 (7%); optic nerve and nerve sheath tumors, 7 (3%); peripheral nerve tumors, 3 (1%); histiocytic lesions, 3 (1%); cystic lesions, 3 (1%); and other lesions, 1 (<1%). The most common histopathologic diagnoses were lymphoma, 50 cases (19%); orbital extension of sinus tumor, 25 (9%); lacrimal gland adenoid cystic carcinoma, 18 (7%); cavernous hemangioma, 15 (6%); orbital extension of brain tumor, 14 (5%); idiopathic orbital inflammation, 14 (5%); plasmacytoma, 8 (3%); reactive lymphoid hyperplasia, 7 (3%); metastatic breast cancer, 7 (3%); orbital extension of ocular adnexal basal cell carcinoma, 7 (3%); orbital extension of ocular adnexal melanoma, 6 (2%), rhabdomyosarcoma, 6 (2%); metastatic gastrointestinal cancer, 5 (2%); sarcoidosis, 5 (2%); and other less common lesions. Forty-two lesions (16%) were intraconal, and 226 (84%) were extraconal. There were 169 (63%) malignant tumors and 99 (37%) were benign tumors. The rate of malignant lesions was 65% in children and 63% in adults (≥18 years). Malignant conditions encountered at a higher rate than previously reported included lymphoma (19% vs 8% to 13%), secondary orbital tumors (26% vs 13% to 20%), orbital metastases (10% vs 2% to 7%), and malignant epithelial lacrimal gland tumors (9% vs 2% to 3%). Our findings highlight the distinctive nature of the orbital oncology experience at a comprehensive cancer center. We found higher than previously reported rates of malignant tumors (63% of all tumors), particularly secondary tumors, malignant epithelial lacrimal gland tumors, and orbital metastases.