{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T09:15:46Z","timestamp":1782292546483,"version":"3.54.5"},"reference-count":42,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2022,10,6]],"date-time":"2022-10-06T00:00:00Z","timestamp":1665014400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genes"],"abstract":"<jats:p>Frantz tumors or solid pseudopapillary pancreatic neoplasm (SPN) are rare exocrine neoplasms that carry a favorable prognosis; they represent up to 3% of all tumors located in the region of the pancreas and have specific age and gender predispositions. In recent years, the rising curve of diagnosis is entitled to the evolution and access of diagnostic imaging. In this paper, we have retrospectively reviewed and described the clinical course of 40 patients with SPN from three institutions in Brazil, who had their diagnosis between 2005 and 2020, and analyzed the clinicopathological, genetic, and surgical aspects of these individuals. In accordance with the literature, most patients were women, 60% with unspecified symptoms at diagnosis, with tumors mainly located in the body and tail of the pancreas, of whom 70% underwent a distal pancreatectomy with sparing splenectomy as a standard procedure, and none of the cases have experienced recurrence to date. Surgery still remains the mainstay of treatment given the low metastatic potential, but more conservative approaches as observed in this cohort are evolving to become the standard of care. Herein, we present an in-depth analysis of cases focusing on the latest literature and report some of the smallest tumor cases in the literature. To our knowledge, this is the first report evaluating germline genetic testing and presenting a case of detected Li-Fraumeni syndrome.<\/jats:p>","DOI":"10.3390\/genes13101809","type":"journal-article","created":{"date-parts":[[2022,10,8]],"date-time":"2022-10-08T20:43:21Z","timestamp":1665261801000},"page":"1809","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Clinical Course, Genetic, and Immunohistochemical Characterization of Solid Pseudopapillary Tumor of the Pancreas (Frantz Tumors) in a Brazilian Cohort"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7831-0469","authenticated-orcid":false,"given":"Francinne T.","family":"Tostes","sequence":"first","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"},{"name":"Center for Personalized Medicine, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Parisina Fraga Dutra Cabral","family":"de Carvalho","sequence":"additional","affiliation":[{"name":"Department of Surgery, Universidade Federal de Medicina do Estado de S\u00e3o Paulo, S\u00e3o Paulo 04023-062, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7834-5944","authenticated-orcid":false,"given":"Raphael L. C.","family":"Ara\u00fajo","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rodrigo Chaves","family":"Ribeiro","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Hospital de Cancer de Barretos Funda\u00e7\u00e3o PIO XII, Barretos 14784-370, Brazil"},{"name":"Barretos School of Health Sciences Dr. Paulo Prata\u2014FACISB, Barretos 14784-370, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franz Robert","family":"Apodaca-Torrez","sequence":"additional","affiliation":[{"name":"Department of Surgery, Universidade Federal de Medicina do Estado de S\u00e3o Paulo, S\u00e3o Paulo 04023-062, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edson Jos\u00e9","family":"Lobo","sequence":"additional","affiliation":[{"name":"Department of Surgery, Universidade Federal de Medicina do Estado de S\u00e3o Paulo, S\u00e3o Paulo 04023-062, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diogo Bugano Diniz","family":"Gomes","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donato","family":"Callegaro-Filho","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gustavo","family":"Schvartsman","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernando","family":"Moura","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"},{"name":"Center for Personalized Medicine, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vladimir","family":"Schraibman","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Goldenberg","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"},{"name":"Department of Surgery, Universidade Federal de Medicina do Estado de S\u00e3o Paulo, S\u00e3o Paulo 04023-062, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernanda Teresa","family":"de Lima","sequence":"additional","affiliation":[{"name":"Center for Personalized Medicine, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5588-8508","authenticated-orcid":false,"given":"Vanderlei","family":"Segatelli","sequence":"additional","affiliation":[{"name":"Department of Pathology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro Luiz Serrano","family":"Uson Junior","sequence":"additional","affiliation":[{"name":"Department of Oncology, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"},{"name":"Center for Personalized Medicine, Hospital Israelita Albert Einstein, S\u00e3o Paulo 05652-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,10,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Us\u00f3n, P.L.S. 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