{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T03:02:38Z","timestamp":1767841358461,"version":"3.49.0"},"reference-count":9,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Pancreatoblastoma, a rare pancreatic tumor, exhibits diverse differentiation pathways, including acinar, ductal, and neuroendocrine lineages, often with distinct squamoid nests [3]. We present a notable case of pancreatoblastoma coexisting with large cell neuroendocrine carcinoma in a 10-year-old boy, presenting with abdominal discomfort, weight loss, and lesions in the pancreas, spleen, and liver visible on imaging. A liver biopsy revealed a poorly differentiated carcinoma with neuroendocrine features, while a splenic biopsy showed acinar cell differentiation, raising possible diagnoses of pancreatoblastoma or acinar cell carcinoma. Subsequent surgical resection after chemotherapy revealed diverse components within the pancreatoblastoma, including well-differentiated acinar and neuroendocrine cells, squamoid nests, and a high-grade neuroendocrine carcinoma. Genetic analysis detected pathogenic variants in TP53 and SMAD4, rarely found in pancreatoblastomas. This juxtaposition of large cell neuroendocrine carcinoma and pancreatoblastoma suggests a potential evolution from well-differentiated neuroendocrine tumors to poorly-differentiated carcinomas within pancreatoblastomas.<\/jats:p>","DOI":"10.1093\/jscr\/rjae654","type":"journal-article","created":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T14:32:14Z","timestamp":1730471534000},"source":"Crossref","is-referenced-by-count":1,"title":["Large cell neuroendocrine carcinoma in pancreatoblastoma with TP53 and SMAD4 mutations: a clinicopathologic study of a rare entity"],"prefix":"10.1093","volume":"2024","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-6963-8815","authenticated-orcid":false,"given":"Ifeomachukwu E","family":"Nwosu","sequence":"first","affiliation":[{"name":"Department of Pathology, Banner-University Medical Center, College of Medicine, University of Arizona , 1625 N Campbell Ave, Tucson, AZ 85721 ,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jenny","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Banner-University Medical Center, College of Medicine, University of Arizona , 1625 N Campbell Ave, Tucson, AZ 85721 ,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexis S","family":"Elliott","sequence":"additional","affiliation":[{"name":"Department of Pathology, Banner-University Medical Center, College of Medicine, University of Arizona , 1625 N Campbell Ave, Tucson, AZ 85721 ,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michelina","family":"De La Maza","sequence":"additional","affiliation":[{"name":"Department of Pediatrics , Pediatrics Hematology and BMT, , 1625 N Campbell Ave, Tucson, AZ 85721 ,","place":["United States"]},{"name":"Banner-University Medical Center, College of Medicine, University of Arizona , Pediatrics Hematology and BMT, , 1625 N Campbell Ave, Tucson, AZ 85721 ,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Belinda L","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Pathology, Banner-University Medical Center, College of Medicine, University of Arizona , 1625 N Campbell Ave, Tucson, AZ 85721 ,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,11,1]]},"reference":[{"key":"2024110114320127200_ref1","doi-asserted-by":"publisher","first-page":"e6779","DOI":"10.7759\/cureus.6779","article-title":"A rare case of Pancreatoblastoma in a Pediatric patient","volume":"12","author":"Mahdi","year":"2020","journal-title":"Cureus"},{"key":"2024110114320127200_ref2","first-page":"1340","article-title":"Prolonged survival after resection of pancreatoblastoma and synchronous liver metastases in an 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