Objective: To describe a series of patients with metastatic orbital carcinoid tumors.
Design: Retrospective case series and literature review.
Participants and methods: Patients were identified from the orbital database and pathology records at Moorfields Eye Hospital. Records were evaluated for age at presentation, race, gender, laterality, visual function and clinical features, site of orbital metastasis, treatment, and outcome. The site of the primary tumor, presence of systemic symptoms, diagnosis and treatment of the primary tumor, and current survival status were also noted.
Main outcome measures: Time from tumor to death.
Results: Thirteen patients (9 male) had an average age at biopsy-proven diagnosis of 65.3 years. The primary tumor was ileal in 7, colonic in 4, and from bronchus and breast (1 each); in 9 patients, the primary tumor was already known. Eleven of the 13 patients had noted a mass, 8 had diplopia, 3 had reduced vision, and only 2 had orbital pain. One patient presented with visual hallucinations. Imaging showed single muscle enlargement in 7 patients, a discrete mass in 5, and diffuse fat infiltration in 1 patient. Six patients underwent a meta-iodobenzyl guanidine/octreotide scan and metastatic disease was found in 4. Four patients underwent exenteration, 5 had radiotherapy after tumor debulking, 2 had radiotherapy alone, and 2 patients had local radiotherapy with receptor-targeted chemotherapy. Seven patients died from metastatic tumor. Kaplan-Meier analysis showed a 5-year survival rate of 72% and a 10-year survival rate of 38%. Comparison of the current series with other published series shows a significant difference (improvement) in survival at 5 years (P = 0.027) but not at 10 years (P = 0.08).
Conclusions: The largest published series of orbital carcinoid tumors is presented, there being a recent trend towards less aggressive surgical management and probably a greater survival rate.