Appendiceal neuroendocrine tumors in children and adolescents: The European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) diagnostic and therapeutic recommendations

IF 2.7 2区医学 Q1 SURGERY

Surgery Pub Date : 2025-05-31 DOI:10.1016/j.surg.2025.109451

Calogero Virgone MD, PhD , Jelena Roganovic MD, PhD , Guido Rindi MD, PhD , Michaela Kuhlen MD , Jan Jamsek MD , Paraskevi Panagopoulou MD , Viera Bajciova MD , Tal Ben-Ami MD , Martine F. Raphael MD , Guido Seitz MD , Patrizia Dall’Igna MD , Sheila C.E.J. Terwisscha van Scheltinga MD, PhD , Miranda P. Dierselhuis MD, PhD , Ramon R. Gorter MD, PhD , Said Bachiri MD, PhD , Ulrich-Frank Pape MD, PhD , Florent Guérin MD, PhD , Ines B. Brecht MD , Brice Fresneau MD, PhD , Daniel Orbach MD , Antje Redlich MD

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Abstract

Neuroendocrine tumors of the appendix, formerly known as carcinoid tumors, represent a rare entity. They are slow-growing tumors, characterized by an indolent clinical course. In pediatric patients, the 5-year overall survival is estimated to be 100% and the event-free survival slightly less than 100%, with only one reported case of local relapse to date. Nevertheless, a proportion of these patients still undergo a second surgery, mostly represented by right hemicolectomy with mesenteric lymphadenectomy, in consideration of the presence of certain risk factors (size >1.5 or 2 cm; tumors of the appendiceal base with or without suspicious residuals, mesoappendiceal invasion, lymphovascular invasion, serosal breach). This approach represents an overtreatment of patients with a benign clinical course regardless of the presence of risk factors and/or whether a second surgery is performed. National recommendations for diagnosis, treatment, and follow-up of neuroendocrine tumors of the appendix in pediatric age are available in France, Italy, and Germany, but international consensus is lacking. This review presents the internationally harmonized recommendations for the diagnosis and treatment of neuroendocrine tumors of the appendix in children and adolescents, established by the European Cooperative Study Group for Pediatric Rare Tumors group.

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儿童和青少年阑尾神经内分泌肿瘤：欧洲儿科罕见肿瘤合作研究小组（专家）诊断和治疗建议

阑尾神经内分泌肿瘤，以前称为类癌肿瘤，是一种罕见的肿瘤。它们是生长缓慢的肿瘤，临床表现为惰性。在儿科患者中，5年总生存率估计为100%，无事件生存率略低于100%，迄今为止仅报告了一例局部复发。然而，考虑到某些危险因素(肿瘤大小1.5 cm或2 cm；阑尾肿瘤伴或不伴可疑残余、阑尾系膜侵犯、淋巴血管侵犯、浆膜破裂)。这种方法代表了对具有良性临床病程的患者的过度治疗，而不管是否存在危险因素和/或是否进行第二次手术。儿童期阑尾神经内分泌肿瘤的诊断、治疗和随访的国家建议在法国、意大利和德国都有，但缺乏国际共识。本综述介绍了由欧洲儿科罕见肿瘤合作研究小组制定的儿童和青少年阑尾神经内分泌肿瘤诊断和治疗的国际统一建议。

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来源期刊

Surgery 医学-外科

CiteScore

5.40

自引率

5.30%

发文量

687

审稿时长

64 days

期刊介绍： For 66 years, Surgery has published practical, authoritative information about procedures, clinical advances, and major trends shaping general surgery. Each issue features original scientific contributions and clinical reports. Peer-reviewed articles cover topics in oncology, trauma, gastrointestinal, vascular, and transplantation surgery. The journal also publishes papers from the meetings of its sponsoring societies, the Society of University Surgeons, the Central Surgical Association, and the American Association of Endocrine Surgeons.