{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,6]],"date-time":"2026-06-06T06:19:44Z","timestamp":1780726784213,"version":"3.54.1"},"reference-count":46,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,2,1]],"date-time":"2021-02-01T00:00:00Z","timestamp":1612137600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Extra\u2010appendicular neuroendocrine tumors (NETs) are very rare tumors. While diagnostic and therapeutic guidelines are well established for adults, data on children and adolescents are lacking.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods<\/jats:title><jats:p>Patients with a diagnosis of extra\u2010appendicular NET registered on the Tumori Rari in Et\u00e0 Pediatrica \u2010 Rare Tumors in Pediatric Age (TREP) from 2000 to 2020 were analyzed. Clinical characteristics including patients\u2019 presentation, tumor features, treatment, and outcome were reviewed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010seven patients with extra\u2010appendicular NET registered on TREP with a median age of 173\u00a0months. The primary site was the pancreas (12) or bronchi (10) in the majority of cases. Other primary sites included the thymus, Meckel's diverticulum, and liver. Thirteen (48%) of tumors extended beyond the organ of origin: four invaded neighboring organs and\/or regional nodes and nine involved distant metastases. The 3\u2010year event\u2010free survival (EFS) for those with localized disease was superior to those with metastatic disease (66.6% 95% CI 5\u201095% vs 33% 95% CI 5\u201068%, respectively; <jats:italic>P<\/jats:italic>\u00a0=\u00a0.005). A complete resection was feasible in 17 patients. The 3\u2010year EFS in these patients was superior to those with no or incomplete resection (R0 vs R1\/R2, respectively; <jats:italic>P<\/jats:italic>\u00a0=\u00a0.007). Overall, 16 children had no evidence of disease at follow\u2010up, and one is alive with disease; five died, and five were lost to follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Data from our experience demonstrated a wide heterogeneity of presentation and outcome of these tumors. Localized disease and complete surgical resection were the main prognostic factors of good outcome. Other therapies may have a role in prolonging survival in metastatic disease.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28880","type":"journal-article","created":{"date-parts":[[2021,2,2]],"date-time":"2021-02-02T05:30:50Z","timestamp":1612243850000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Extra\u2010appendicular neuroendocrine tumors: A report from the TREP project (2000\u20102020)"],"prefix":"10.1002","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3651-9416","authenticated-orcid":false,"given":"Calogero","family":"Virgone","sequence":"first","affiliation":[{"name":"Pediatric Surgery, Department of Women's and Children's Health University Hospital of Padua  Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4724-0517","authenticated-orcid":false,"given":"Andrea","family":"Ferrari","sequence":"additional","affiliation":[{"name":"Hematology Oncology IRCCS Istituto Nazionale dei Tumori  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Chiaravalli","sequence":"additional","affiliation":[{"name":"Hematology Oncology IRCCS Istituto Nazionale dei Tumori  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Debora","family":"De Pasquale","sequence":"additional","affiliation":[{"name":"Department of Oncohematology Bambino Ges\u00f9 Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5663-8674","authenticated-orcid":false,"given":"Alessandro","family":"Inserra","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Bambino Ges\u00f9 Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4021-8472","authenticated-orcid":false,"given":"Paolo","family":"D'Angelo","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology Unit A.R.N.A.S. 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