{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T21:11:14Z","timestamp":1777756274318,"version":"3.51.4"},"reference-count":40,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2020,6,3]],"date-time":"2020-06-03T00:00:00Z","timestamp":1591142400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"The TREP project is partially supported by a grant from \u2018\u2018Fondazione Giovanni Celeghin ONLUS,\u201d Padova."}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra\u2010adrenal counterparts arising from ganglia along the sympathetic\/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline mutation is commonly associated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and methods<\/jats:title><jats:p>Among over 1000 patients registered into the Tumori Rari in Et\u00e0 Pediatrica\u2014rare tumors in pediatric age project\u2014from 2000 to 2019, 50 were affected by PC\/PGL. All clinical and therapeutic data were evaluated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010eight patients had PC and 22 had PGL. Age at diagnosis ranged between 5 and 17 years. Thirty\u2010five patients had symptoms related to catecholamine hypersecretion; in 7 of 50 patients, diagnosis was incidental or done during assessment of a familial syndrome. In all cases, conventional imaging was effective to assess the presence of a tumor. In addition, 18 of 38 functional imaging studies were positive (61%). Forty\u2010eight patients were eligible for surgery: a complete resection was more frequently achieved in PC than in PGL (26\/28\u00a0vs 11\/22). All relapses were treated with surgery alone, surgery plus medical treatment, or chemotherapy alone; one PC with metastasis at diagnosis received radiotherapy only. Forty\u2010four patients were in the first, second, or third complete remission (10\/50 recurred; 8\/10 carried a germline mutation). Five of 50 patients were alive with disease. One patient died of disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Surgery can be curative in most tumors but it may not be always effective in removing PGLs. Severe postsurgical sequelae may affect these patients. Genetic tests should always be considered in individuals affected, and genetic counseling should be offered to their families.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28332","type":"journal-article","created":{"date-parts":[[2020,6,3]],"date-time":"2020-06-03T14:07:14Z","timestamp":1591193234000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP)"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3651-9416","authenticated-orcid":false,"given":"Calogero","family":"Virgone","sequence":"first","affiliation":[{"name":"Pediatric Surgery Unit Department of Women's and Children's Health University Hospital of Padua  Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marina","family":"Andreetta","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Unit 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