Neuroendocrine tumours of the orbit: report of three cases and a literature review

Abstract

Abstract Background Neuroendocrine tumours (NETs) are malignancies originating from neuroendocrine cells. NETs mainly occur in the gastrointestinal tract and lungs and can metastasise to the eye orbit in rare cases. We analysed three orbital NET cases in the present study and described their clinicopathologic factors, treatments and prognosis. Case presentation Three orbital NET cases were described in the present study. The average age of the patients was 59 years old. One patient was male, and two were female. Two of them showed ocular symptoms, including unilateral proptosis and eyelid mass; the other showed systemic symptoms as Cushing syndrome. The three patients underwent surgical resection of the tumour. Tumours were analysed using immunohistochemistry assays and were positive for PCK and EMA. Besides, two patients were positive for CD56 and Syn. Cases 1 and 2 were pathologically diagnosed with "neuroendocrine carcinoma", refused treatment, and died three months later. Case 3 was diagnosed with a "neuroendocrine tumour", and after the operation, the Cushing syndrome symptoms gradually disappeared. Finally, no recurrence was found after four years of follow-up. Conclusion Neuroendocrine tumours can originate from orbit and show different clinical manifestations due to the different types of orbital NETs. Pathology can clarify the diagnosis, classification and grading, provide a reference value for treatment strategy and prognosis.