Pancreatoblastoma with a novel fusion gene ofIQSEC1‐RAF1

Pediatric Blood & CancerVolume 70, Issue 4 e30155 LETTER TO THE EDITOR Pancreatoblastoma with a novel fusion gene of IQSEC1-RAF1 Hironori Goto, Hironori Goto orcid.org/0000-0003-2120-3440 Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Department of Pediatrics, Oita University Faculty of Medicine, Oita, JapanSearch for more papers by this authorYuhki Koga, Corresponding Author Yuhki Koga [email protected] Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Department of Pediatric and Perinatal Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Correspondence Yuhki Koga, Department of Pediatric and Perinatal Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Email: [email protected]Search for more papers by this authorKenichi Kohashi, Kenichi Kohashi Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorHiroaki Ono, Hiroaki Ono Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorJunkichi Takemoto, Junkichi Takemoto Department of Pediatric Surgery, Reproductive and Developmental Medicine Faculty of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorToshiharu Matsuura, Toshiharu Matsuura Department of Pediatric Surgery, Reproductive and Developmental Medicine Faculty of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorTatsuro Tajiri, Tatsuro Tajiri Department of Pediatric Surgery, Reproductive and Developmental Medicine Faculty of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorKenji Ihara, Kenji Ihara Department of Pediatrics, Oita University Faculty of Medicine, Oita, JapanSearch for more papers by this authorYoshinao Oda, Yoshinao Oda Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorShouichi Ohga, Shouichi Ohga Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this author Hironori Goto, Hironori Goto orcid.org/0000-0003-2120-3440 Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Department of Pediatrics, Oita University Faculty of Medicine, Oita, JapanSearch for more papers by this authorYuhki Koga, Corresponding Author Yuhki Koga [email protected] Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Department of Pediatric and Perinatal Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Correspondence Yuhki Koga, Department of Pediatric and Perinatal Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Email: [email protected]Search for more papers by this authorKenichi Kohashi, Kenichi Kohashi Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorHiroaki Ono, Hiroaki Ono Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorJunkichi Takemoto, Junkichi Takemoto Department of Pediatric Surgery, Reproductive and Developmental Medicine Faculty of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorToshiharu Matsuura, Toshiharu Matsuura Department of Pediatric Surgery, Reproductive and Developmental Medicine Faculty of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorTatsuro Tajiri, Tatsuro Tajiri Department of Pediatric Surgery, Reproductive and Developmental Medicine Faculty of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorKenji Ihara, Kenji Ihara Department of Pediatrics, Oita University Faculty of Medicine, Oita, JapanSearch for more papers by this authorYoshinao Oda, Yoshinao Oda Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this authorShouichi Ohga, Shouichi Ohga Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSearch for more papers by this author First published: 15 December 2022 https://doi.org/10.1002/pbc.30155Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Supporting Information Filename Description pbc30155-sup-0001-FigureS1.pdf188.5 KB Figure S1. Clinical course of the patient with RAF1-positive pancreatoblastoma. FDG-PET imaging (A, B, C) and CT showed multiple tumors in the pancreatic head and body, pleural effusion and the regrowth of multiple lung lesions. The recurrent tumor progressed after chemotherapy but showed transient amelioration during combination therapy with adjunctive dabrafenib and trametinib. His performance status (PS) came to be stable over 3 months of dabrafenib and trametinib therapy. AFP, α-fetoprotein; RT, radiation therapy; auto-PBSCT, autologous peripheral blood stem cell transplantation. pbc30155-sup-0002-FigureS2.pdf247.4 KB Figure S2. Disease spectrum of primary pancreatic neoplasms with or without RAF1-positivity in pediatric and adult patients. This figure shows the age distribution, cell origin, histopathological features, AFP levels, involvement of Wnt signaling in tumorigenesis, and genetic features in primary pancreatic neoplasms. pbc30155-sup-0003-TableS1.docx21.8 KB Table S1 Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article. REFERENCES 1Huang Y, Yang W, Hu J, et al. Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center. Pediatr Surg Int. 2019; 35(11): 1231-1238. 2Huang X, Li M, Zhang L, Xiong J, Lu H, Tian B. Clinical characteristics and treatment analysis of pancreatic acinar cell carcinoma: a single institutional comparison to pancreatic ductal adenocarcinoma. Surg Oncol. 2021; 37:101528. 3Isobe T, Seki M, Yoshida K, et al. 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