PDF(Pubmed)
Abstract：
BACKGROUND: The ocular involvement of neuroendocrine neoplasms (NENs) is uncommon and mainly represented by metastases from gastrointestinal and lung neuroendocrine tumors. Primary orbital NENs are even less common and their diagnostic and therapeutic management is a challenge.
METHODS: A systematic review of the literature was conducted from 1966 to September 2023 on PubMed to identify articles on orbital NENs and to summarize their clinical-pathological features, diagnosis and therapeutic management. Furthermore, we presented a case of a locally advanced retro-orbital primary neuroendocrine tumor that was referred to the certified Center of Excellence of Sant\'Andrea Hospital, La Sapienza University of Rome, Italy.
RESULTS: The final analysis included 63 records on orbital NENs and 11 records focused on primary orbital NENs. The localization was mostly unilateral and in the right orbit; proptosis or exophthalmos represented the initial symptoms. The diagnostic work-up and therapeutic management was discussed and a diagnostic algorithm for the suspicion of primary orbital NENs was proposed.
CONCLUSIONS: A multidisciplinary approach is required for the management of primary orbital NENs, emphasizing the importance of early referral to dedicated centers for prompt differential diagnosis, tailored treatment, and an improved quality of life and survival.
METHODS: A systematic review of the literature was conducted from 1966 to September 2023 on PubMed to identify articles on orbital NENs and to summarize their clinical-pathological features, diagnosis and therapeutic management. Furthermore, we presented a case of a locally advanced retro-orbital primary neuroendocrine tumor that was referred to the certified Center of Excellence of Sant\'Andrea Hospital, La Sapienza University of Rome, Italy.
RESULTS: The final analysis included 63 records on orbital NENs and 11 records focused on primary orbital NENs. The localization was mostly unilateral and in the right orbit; proptosis or exophthalmos represented the initial symptoms. The diagnostic work-up and therapeutic management was discussed and a diagnostic algorithm for the suspicion of primary orbital NENs was proposed.
CONCLUSIONS: A multidisciplinary approach is required for the management of primary orbital NENs, emphasizing the importance of early referral to dedicated centers for prompt differential diagnosis, tailored treatment, and an improved quality of life and survival.
摘要：
背景：神经内分泌肿瘤（NENs）的眼部受累并不常见，主要表现为胃肠道和肺神经内分泌肿瘤的转移。原发性眼眶NENs更不常见，其诊断和治疗管理是一个挑战。
方法：从1966年至2023年9月在PubMed上对文献进行了系统回顾，以确定有关眼眶NENs的文章并总结其临床病理特征，诊断和治疗管理。此外，我们介绍了一例局部晚期眶后原发性神经内分泌肿瘤，该肿瘤被转诊至Sant\'Andrea医院卓越认证中心，LaSapienza罗马大学，意大利。
结果：最终分析包括63个轨道NEN记录和11个主要轨道NEN记录。定位主要是单侧的，在正确的轨道上；眼球突出或眼球突出代表最初的症状。讨论了诊断工作和治疗管理，并提出了怀疑原发性眼眶NEN的诊断算法。
结论：需要多学科方法来管理主要眼眶NEN，强调早期转诊到专门中心进行及时鉴别诊断的重要性，量身定制的治疗，生活质量和生存质量的提高。
方法：从1966年至2023年9月在PubMed上对文献进行了系统回顾，以确定有关眼眶NENs的文章并总结其临床病理特征，诊断和治疗管理。此外，我们介绍了一例局部晚期眶后原发性神经内分泌肿瘤，该肿瘤被转诊至Sant\'Andrea医院卓越认证中心，LaSapienza罗马大学，意大利。
结果：最终分析包括63个轨道NEN记录和11个主要轨道NEN记录。定位主要是单侧的，在正确的轨道上；眼球突出或眼球突出代表最初的症状。讨论了诊断工作和治疗管理，并提出了怀疑原发性眼眶NEN的诊断算法。
结论：需要多学科方法来管理主要眼眶NEN，强调早期转诊到专门中心进行及时鉴别诊断的重要性，量身定制的治疗，生活质量和生存质量的提高。
