Hatsis Alexander J, Henry Roger K, Curtis Mark T, Bilyk Jurij R, Sivalingam Meera D, Eagle Ralph C, Milman Tatyana
Department of Pathology, Wills Eye Hospital.
Rutgers Robert Wood Johnson Medical School.
Orbit. 2021 Dec;40(6):488-498. doi: 10.1080/01676830.2020.1839108. Epub 2020 Nov 3.
To describe a patient with orbital neuroendocrine neoplasm (NEN)/carcinoid tumor and to review the clinical presentation, systemic work-up, histopathologic features, and outcome of all previously reported ocular adnexal (OA) NENs.
A systematic literature review. PubMed/MEDLINE and Google Scholar databases were searched for all well-documented cases of OA NENs.
Final analysis yielded 94 patients with OA NENs, 50 females (53%) and 44 (47%) males with an average age of 63 years (range 14-86). Of 91 patients with known information, the most common presenting signs were proptosis (56/91, 61%) and visual disturbances (42/91, 47%), induced by a mass most commonly associated with an extraocular muscle (49/63, 78%). The majority of tumors (88/94, 94%) were metastases, most commonly from the gastrointestinal tract (52/88, 59%). OA NEN metastasis presented following detection of primary tumor in 73/94 (78%) patients (median time to metastasis 36 months, range 0-288 months) and as an initial manifestation of disease in 15/94 (16%) patients (median time to primary detection 18 months, range 1-108 months). Systemic work-up included extra-OA NEN biopsy (37/54, 69%), multimodal imaging (42/54, 78%), and other laboratory studies (32/54, 59%). Resection with or without adjuvant chemotherapy, radiotherapy, and biologics was the most common intervention for OA NENs (36/82, 44%). Of 67 patients with available follow-up, the median survival was 108 months (95% CI 55-161 months) and the absolute 5-year survival rate was 68%.
OA NENs are almost exclusively metastases and can precede detection of primary tumor by many months, requiring appropriate diagnostic work-up.
描述1例眼眶神经内分泌肿瘤（NEN）/类癌患者，并回顾所有既往报道的眼附属器（OA）NEN的临床表现、全身检查、组织病理学特征及预后。
进行系统的文献综述。在PubMed/MEDLINE和谷歌学术数据库中检索所有记录完整的OA NEN病例。
最终分析纳入94例OA NEN患者，其中女性50例（53%），男性44例（47%），平均年龄63岁（范围14 - 86岁）。在91例有已知信息的患者中，最常见的表现体征是眼球突出（56/91，61%）和视力障碍（42/91，47%），由肿物引起，该肿物最常与眼外肌相关（49/63，78%）。大多数肿瘤（88/94，94%）为转移瘤，最常见于胃肠道（52/88，59%）。73/94（78%）例患者在原发性肿瘤被发现后出现OA NEN转移（转移的中位时间为36个月，范围0 - 288个月），15/94（16%）例患者以OA NEN转移作为疾病的初始表现（初次发现原发性肿瘤的中位时间为18个月，范围1 - 108个月）。全身检查包括眼附属器外NEN活检（37/54，69%）、多模态成像（42/54，78%）和其他实验室检查（32/54，59%）。手术切除联合或不联合辅助化疗、放疗及生物制剂是OA NEN最常见的干预措施（36/82，44%）。在67例有随访信息的患者中，中位生存期为108个月（95%CI 55 - 161个月），5年绝对生存率为68%。
OA NEN几乎均为转移瘤，可在原发性肿瘤被发现前数月出现，需要进行适当的诊断检查。
