Neuroendocrine tumors of the orbit--clinicopathological findings in 3 more cases of this rare entity and review of the literature.

ABSTRACT

INTRODUCTION: Neuroendocrine orbital tumors are rare occurrences. They are poorly characterized histologically and a spectrum of different cell types exists. This short case series studies the various tumor morphologies as well as the patients' clinical profiles. METHODS AND MATERIALS Patients treated and followed up at the Singapore National Eye Centre over a period of 8 years from 1(st) January 2002 to 31(st) December 2009 were identified from the orbital tumor board results. The case notes of patients with the diagnosis of neuroendocrine tumors were analysed, and a review of the literature performed. RESULTS: Three patients are described in this series. The history, clinical examination findings, imaging findings as well as tumor histology were described. The mean age was 63 years, and 2 patients were male. All presented with proptosis. Only one patient had systemic symptoms on presentation. All patients had surgical excision of the tumor and two had adjuvant radiotherapy of the orbit. Median follow-up period was 3.5 years. DISCUSSION: Patients with a biopsy-proven diagnosis of orbital neuroendocrine tumors should be monitored even when systemic examination fails to identify peripheral disease. Such examination should always include a full endoscopic gastrointestinal review.