This is a retrospective review of 28 patients with carcinoid tumours affecting the orbit. The mean age at presentation of the orbital involvement was 62 years, with a slight female preponderance, and 21% had carcinoid syndrome. Proptosis and diplopia were the commonest presenting symptoms. Nearly 80% had extraocular muscle involvement, usually single muscles. There was a wide range of locations of the primary tumour, but the most common sites were the gut and lung. In eight patients the primary tumour remained unidentified, and five-year survival from the time of orbital metastasis diagnosis was 50%. Treatment with surgery ranged from debulking to excisional attempts and exenteration, but none was associated with an increased survival compared to the others. Similarly, there was no difference in survival comparing those who had external radiotherapy or radiolabelled octreatide with those who had none. Patients who received chemotherapy had lower five-year survival rates, presumably as this was only offered to those with extensive or advanced disease. This is the largest published series of orbital carcinoid, but unfortunately the numbers are still too small to draw firm conclusions regarding optimum management.
Carcinoid tumours
Reviewed by James Hsuan
Carcinoid tumors of the orbit and ocular adnexa.
