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Background emphasizes rarity and indolent behavior with low recurrence or metastasis, while biological and prognostic traits limit adult guideline transfer. Methods describe a mini-review covering epidemiology, presentation, diagnostic workup, pathology, surgery, and follow-up. Results support risk-adapted management, often simple appendectomy, with MDT input for atypical cases.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/appendiceal-neuroendocrine-tumors-in-children-and-adolescents/354266/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/appendiceal-neuroendocrine-tumors-in-children-and-adolescents/354266.png","ImageObject",300,407,{"name":92,"@type":93},"supergirl","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-29","2026-09-22",true,{"@type":102,"interactionType":103,"userInteractionCount":39},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What are appendiceal neuroendocrine tumors (aNETs) in children and adolescents?","Question",{"text":112,"@type":113},"aNETs are rare gastrointestinal epithelial neuroendocrine neoplasms in this age group. They are typically well-differentiated and often follow an indolent clinical course.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How do pediatric aNETs usually present clinically?",{"text":117,"@type":113},"They are often found incidentally during appendectomy for suspected acute appendicitis or abdominal pain. Symptoms are usually nonspecific, such as right lower quadrant pain and sometimes nausea, vomiting, or fever.",{"name":119,"@type":110,"acceptedAnswer":120},"Why is pediatric-specific management important and what is the typical treatment approach?",{"text":121,"@type":113},"Adult-derived guidelines may not capture pediatric biological and prognostic differences. Evidence supports a risk-adapted strategy, where simple appendectomy is curative for most cases, while MDT discussion is important for atypical or borderline situations.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},354266,1790731122,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":39,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":24,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144},962088121634,"https://ap-avatar.wpscdn.com/davatar_9964176cb1d06d4a9deccf72a44ae3dc","Clinical Medicine   Mini-Review  \nActa Medica Academica 2026;55(1):72-76 DOI: 10.5644/ama2006-124 .496  \nAppendiceal Neuroendocrine Tumors in Children and Adolescents  \nJelena Roganovic1, 2, Luisa Santoro3, Calogero Virgone4, 5  \n1Department of Pediatric Hematology and Oncology, Children’s Hospital Zagreb, Zagreb, Croatia, 2Faculty of Biomedicine and Drug Development, University of Rijeka, Rijeka, Croatia, 3Surgical Pathology and Cytopathology Unit, Department of Medicine-DIMED, University of Padova, Padova, Italy, 4Pediatric Surgery Division, University Hospital of Padua, Padua, Italy, 5Department of Women’s and Children’s Health, University Hospital of Padua, Padua, Italy [Correspondence:](Correspondence: jelena.roganovic02@gmail.com)[ jelena.roganovic02@gmail.com](Correspondence: jelena.roganovic02@gmail.com); [jelena.roganovic@kdbz.hr](jelena.roganovic@kdbz.hr); Tel.: + 385 1 6445775  \nReceived: 16 November 2025; Accepted: 8 January 2026  \nAbstract  \nObjective. To synthesize current evidence on the diagnosis, histopathological evaluation, clinical features, management, and follow-up of appendiceal neuroendocrine tumors (aNETs) in children and adolescents, and to outline key differences from their adult counterparts. Background. Pediatric aNETs are rare gastrointestinal neoplasms that typically exhibit an indolent clinical course with minimal risk of recurrence or metastasis. Their biological and prognostic features differ from those in adults, limiting the applicability of adult-derived guidelines in children. Methods. A mini review of the current literature was conducted, focusing on epidemiology, clinical presentation, diagnostic workup, key pathological features, surgical management, and follow-up strategies for pediatric aNETs. Discussion. Contemporary evidence supports a de-escalated, risk-adapted approach to management, with simple appendectomy being curative in most cases. Multidisciplinary Team (MDT) discussion remains critical for atypical or borderline cases requiring individualized decision-making. Differences from adult aNETs highlight the need for pediatric-specific clinical decision-making. Conclusion. Early recognition, accurate histopathologic evaluation, and tailored surgical management are essential to optimize outcomes for children and adolescents with aNETs.  \nKeyWords: Appendix 􀂃 Neuroendocrine 􀂃 Neoplasms 􀂃 Children and Adolescents.  \n\n| Introduction\u003Cbr>Appendiceal neuroendocrine tumors (aNETs) are very rare neoplasms in children and adolescents, yet they represent the most frequent gastrointestinal epithelial tumors in this age group (1). Historically referred to as “carcinoids,” aNETs are now recognized as a heterogeneous group of well-differentiated neuroendocrine neoplasms with distinct clinical, pathological, and molecular features (1, 2). Despite growing awareness, pediatric-specific data remain limited, and adult-derived guidelines might not adequately capture the unique biological and clinical aspects of pediatric aNETs (3-5).\u003Cbr>Epidemiology\u003Cbr>The incidence of pediatric aNETs is reported to range between 1:100,000 and 1.14:1,000,000\u003Cbr>72 | children per year (1). The precise frequency among all appendectomies remains uncertain. In adults, it is estimated at around 0.2%, whereas in children, the reported rate is 0.169%( 1, 6) . More recently, a multicenter study in eight U.S. tertiary hospitals reported a slightly higher incidence of 0.4% of all appendectomy specimens (4) . The median age at diagnosis is 12 to 14 years, with a slight female predominance (4, 5) .\u003Cbr>Clinical Presentation\u003Cbr>Pediatric aNETs typically present incidentally during appendectomy for suspected acute appendicitis or abdominal pain (4, 6) . Symptoms are usually nonspecific, including right lower quadrant pain, nausea, vomiting, and occasional fever (6, 7) . Larger tumors (>2 cm) may manifest with |\n| --- | --- |\n\nCopyright © 2026 Roganovic et al. This article is available under a Creative Commons License (Attribution 4","cbCaiiOqKLNgxuPH","https://ap.wps.com/l/cbCaiiOqKLNgxuPH","pdf",1646795,"English","# Introduction\n## Epidemiology\n## Clinical Presentation\n# Pathology and Molecular Features\n## Immunohistochemistry and Markers\n## Grading and Prognostic Factors\n# Figures","[{\"question\":\"What are appendiceal neuroendocrine tumors (aNETs) in children and adolescents?\",\"answer\":\"aNETs are rare gastrointestinal epithelial neuroendocrine neoplasms in this age group. They are typically well-differentiated and often follow an indolent clinical course.\"},{\"question\":\"How do pediatric aNETs usually present clinically?\",\"answer\":\"They are often found incidentally during appendectomy for suspected acute appendicitis or abdominal pain. Symptoms are usually nonspecific, such as right lower quadrant pain and sometimes nausea, vomiting, or fever.\"},{\"question\":\"Why is pediatric-specific management important and what is the typical treatment approach?\",\"answer\":\"Adult-derived guidelines may not capture pediatric biological and prognostic differences. Evidence supports a risk-adapted strategy, where simple appendectomy is curative for most cases, while MDT discussion is important for atypical or borderline situations.\"}]","Appendiceal Neuroendocrine Tumors in Children and Adolescents | PDF",1790109776,13]