神经内分泌肿瘤（NENs）源于神经内分泌细胞，主要发生在胃肠道和肺部，眼眶发生极为罕见。本文描述了三例眼眶NENs的临床病理因素、治疗和预后。三个患者的平均年龄为59岁，其中两名女性，一名男性。两例表现为眼部症状，包括单侧眼球突出和眼睑肿块，而第三例表现为库欣综合征的全身症状。所有三例患者的肿瘤均进行了手术切除。免疫组化检测显示所有病例中均为泛细胞角蛋白和上皮膜抗原阳性表达。此外，两例中神经细胞粘附分子1（也称为CD56）和突触素呈阳性表达。病理诊断显示案例1和2为神经内分泌癌，并在诊断后三个月内去世。案例3被诊断为神经内分泌肿瘤，并表现为库欣综合征的症状经手术逐渐改善。此外，在四年的随访期间未观察到复发。这些案例表明，眼眶神经内分泌肿瘤由于类型的不同呈现出不同的临床表现。病理学可以明确诊断、分类和分级，并为治疗和预后提供参考价值。

Neuroendocrine neoplasms (NENs) originate from neuroendocrine cells, and mainly occur in the gastrointestinal tract and lungs, rarely occurring in the orbit. Here, the clinicopathologic factors, treatments and prognosis of three cases of orbital NENs are described. The mean age of the three patients (two females and one male) was 59 years. Two cases exhibited ocular symptoms, including unilateral proptosis and eyelid mass, while the third case presented systemic symptoms exhibited as Cushing's syndrome. The tumours were surgically resected in all three patients. Immunohistochemistry assays revealed positive expression for pan cytokeratin and epithelial membrane antigen in all cases. Additionally, neural cell adhesion molecule 1 (also known as CD56) and synaptophysin were positive in two cases. The pathological diagnosis for case 1 and 2 was 'neuroendocrine carcinoma' and both patients died three months after diagnosis. Case 3 was diagnosed with a neuroendocrine tumour and the symptoms of Cushing's syndrome gradually improved following surgery. In addition, no recurrence was observed during the four-year follow-up period. These cases demonstrate that orbital neuroendocrine tumours show different clinical manifestations due to the different types. Pathology may clarify the diagnosis, classification and grading, and provide a reference value for treatment and prognosis.