{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T16:44:16Z","timestamp":1779900256448,"version":"3.53.1"},"reference-count":29,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2025,5,7]],"date-time":"2025-05-07T00:00:00Z","timestamp":1746576000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2025,9]]},"abstract":"\n Pediatric pancreatic acinar cell carcinoma (PACC) is a rare malignancy, comprising 5-15% of pediatric pancreatic tumors.\n BRAF<\/jats:italic>\n rearrangement is found in 20%\u201330% of PACC cases. We report a case of PACC with a novel\n GLCCI1::BRAF<\/jats:italic>\n fusion and independent amplifications in\n MYC<\/jats:italic>\n and\n MYCN<\/jats:italic>\n . A 10-year-old male presented with 6\u2009months of weight loss, back pain, and loose stools. Imaging demonstrated concentric soft tissue thickening around the superior mesenteric artery, prompting biopsy of a periaortic lymph node showing metastatic PACC. Pancreaticoduodenectomy revealed PACC and metastatic deposits in multiple lymph nodes and retroperitoneal soft tissue. Fluorescence\n in situ<\/jats:italic>\n hybridization of both the periaortic lymph node and pancreaticoduodenectomy specimens demonstrated\n BRAF<\/jats:italic>\n gene rearrangement, with the partner identified as\n GLCCI1<\/jats:italic>\n by next generation sequencing and fusion assays. Chromosomal microarray analysis demonstrated amplification of\n MYC<\/jats:italic>\n in the periaortic lymph node biopsy and amplification of\n MYCN<\/jats:italic>\n in the resection specimen. The patient was treated with neoadjuvant chemotherapy, radiation, and a pan-RAF inhibitor, but developed new widespread metastasis and was deceased 22\u2009months after presentation. The combination of the primary\n GLCCI1::BRAF<\/jats:italic>\n fusion with secondary amplification of\n MYC<\/jats:italic>\n and\n MYCN<\/jats:italic>\n is likely to drive the aggressive behavior and metastasis in this case of PACC.\n <\/jats:p>","DOI":"10.1177\/10935266251337376","type":"journal-article","created":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T01:42:11Z","timestamp":1746668531000},"page":"395-399","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["A Novel\n GLCCI1::BRAF<\/i>\n Fusion With Independent\n MYC<\/i>\n and\n MYCN<\/i>\n Amplifications in Pediatric Pancreatic Acinar Cell Carcinoma"],"prefix":"10.1177","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3212-7896","authenticated-orcid":false,"given":"Lauren J.","family":"Miller","sequence":"first","affiliation":[{"name":"Department of Pathology, University of Michigan, Michigan Medicine, Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6522-2454","authenticated-orcid":false,"given":"Amer","family":"Heider","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Michigan, Michigan Medicine, Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina","family":"Shao","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Michigan, Michigan Medicine, Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2025,5,7]]},"reference":[{"key":"e_1_3_3_2_2","first-page":"1","volume-title":"Pathology of the Pancreas","author":"La Rosa S","unstructured":"La Rosa S, Sciarra A. Acinar cell carcinoma. In: Esposito I, Karamitopoulou-Diamantis E, editors. Pathology of the Pancreas. 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GLCCI1 glucocorticoid induced 1 [ Homo sapiens (human) ]. National Center for Biotechnology Information: National Library of Medicine; 2025. 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