{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T09:44:30Z","timestamp":1753868670743,"version":"3.41.2"},"reference-count":19,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2024,8,26]],"date-time":"2024-08-26T00:00:00Z","timestamp":1724630400000},"content-version":"vor","delay-in-days":25,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Reports"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic neoplasm. Recently, molecular analysis revealed that PACC shows a high frequency of the <jats:italic>BRCA<\/jats:italic>1\/2 mutation and is likely to be considered a cancer associated with hereditary breast and ovarian cancer (HBOC). Hereditary cancers, including HBOC, are characterized by multifocal and\/or metachronous tumors. However, no case reports exist of germline <jats:italic>BRCA1<\/jats:italic>\u2010mutated synchronous and metachronous PACC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case<\/jats:title><jats:p>A 58\u2010year\u2010old man was diagnosed with synchronous and metachronous PACC at the age of 56 and underwent two surgeries. Ten months after the second surgery, the patient developed multiple liver metastases. Gemcitabine plus nab\u2010paclitaxel therapy was administered as first\u2010line chemotherapy. After seven cycles, computed tomography examination revealed progressive disease (PD). Therefore, modified FOLFIRINOX (mFFX) was administered as second\u2010 line chemotherapy. After 19 cycles of mFFX, comprehensive cancer genomic profiling (CGP) identified a <jats:italic>BRCA1<\/jats:italic> pathogenic variant that was confirmed to be germline origin. Accordingly, we treated the patient with olaparib; however, he was diagnosed with PD after 4\u2009months. He subsequently died 5\u2009years and 9\u2009months after the initial surgery, and 3\u2009years and 10\u2009months after chemotherapy. Based on the genetic data of the patients, his family members received genetic counseling followed by cascade testing. Consequently, the same g<jats:italic>BRCA1<\/jats:italic> pathogenic variant was detected in the son and his surveillance for HBOC\u2010related cancers was initiated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We diagnosed a 58\u2010year\u2010old man with a synchronous and metachronous PACC with germline <jats:italic>BRCA1<\/jats:italic> pathogenic variant. Considering that PACC is likely to have <jats:italic>BRCA1\/2<\/jats:italic> mutations responsible for HBOC, we need to be aware of the possible presence of multifocal and\/or metachronous tumors in patients with PACC. Additionally, patients with PACC should undergo genetic examinations, which would be beneficial in determining treatment strategies and health care for blood relatives.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cnr2.70007","type":"journal-article","created":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T06:28:15Z","timestamp":1724740095000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Germline <scp><i>BRCA1<\/i><\/scp>\u2010Mutated Synchronous and Metachronous Pancreatic Acinar Cell Carcinoma With Long\u2010Term Survival"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5585-0972","authenticated-orcid":false,"given":"Tomohiro","family":"Kubo","sequence":"first","affiliation":[{"name":"Department of Medical Oncology Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuki","family":"Ikeda","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joji","family":"Muramatu","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuma","family":"Ishikawa","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makoto","family":"Yoshida","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuharu","family":"Kukita","sequence":"additional","affiliation":[{"name":"Department of Surgery, Surgical Oncology and Science Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masafumi","family":"Imamura","sequence":"additional","affiliation":[{"name":"Department of Surgery, Surgical Oncology and Science Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shintaro","family":"Sugita","sequence":"additional","affiliation":[{"name":"Department of Surgical Pathology Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akihiro","family":"Sakurai","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics and Genomics Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kohichi","family":"Takada","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology Sapporo Medical University School of Medicine  Sapporo Hokkaido Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,8,26]]},"reference":[{"doi-asserted-by":"publisher","key":"e_1_2_8_2_1","DOI":"10.1097\/00006676-200404000-00002"},{"doi-asserted-by":"publisher","key":"e_1_2_8_3_1","DOI":"10.1158\/2159-8290.CD-14-0617"},{"doi-asserted-by":"publisher","key":"e_1_2_8_4_1","DOI":"10.1038\/nature14169"},{"doi-asserted-by":"publisher","key":"e_1_2_8_5_1","DOI":"10.5858\/arpa.2019-0472-RA"},{"doi-asserted-by":"publisher","key":"e_1_2_8_6_1","DOI":"10.1200\/JCO.23.00561"},{"doi-asserted-by":"publisher","key":"e_1_2_8_7_1","DOI":"10.1002\/jgh3.12380"},{"doi-asserted-by":"publisher","key":"e_1_2_8_8_1","DOI":"10.6004\/jnccn.2021.0017"},{"doi-asserted-by":"publisher","key":"e_1_2_8_9_1","DOI":"10.1038\/ng1837"},{"doi-asserted-by":"publisher","key":"e_1_2_8_10_1","DOI":"10.1038\/nature05609"},{"doi-asserted-by":"publisher","key":"e_1_2_8_11_1","DOI":"10.1200\/JCO.2007.12.5922"},{"doi-asserted-by":"publisher","key":"e_1_2_8_12_1","DOI":"10.1097\/mpa.0b013e31804bfbd3"},{"doi-asserted-by":"publisher","key":"e_1_2_8_13_1","DOI":"10.1177\/17562848211014818"},{"doi-asserted-by":"publisher","key":"e_1_2_8_14_1","DOI":"10.1056\/NEJMoa1903387"},{"doi-asserted-by":"publisher","key":"e_1_2_8_15_1","DOI":"10.1093\/jjco\/hyad131"},{"doi-asserted-by":"publisher","key":"e_1_2_8_16_1","DOI":"10.1002\/path.4310"},{"doi-asserted-by":"publisher","key":"e_1_2_8_17_1","DOI":"10.1002\/gcc.23222"},{"doi-asserted-by":"publisher","key":"e_1_2_8_18_1","DOI":"10.1093\/jjco\/hyt036"},{"doi-asserted-by":"publisher","key":"e_1_2_8_19_1","DOI":"10.1002\/ijc.29032"},{"doi-asserted-by":"publisher","key":"e_1_2_8_20_1","DOI":"10.1038\/s10038-017-0355-1"}],"container-title":["Cancer Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cnr2.70007","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,28]],"date-time":"2024-08-28T02:55:57Z","timestamp":1724813757000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/cnr2.70007"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8]]},"references-count":19,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2024,8]]}},"alternative-id":["10.1002\/cnr2.70007"],"URL":"https:\/\/doi.org\/10.1002\/cnr2.70007","archive":["Portico"],"relation":{},"ISSN":["2573-8348","2573-8348"],"issn-type":[{"type":"print","value":"2573-8348"},{"type":"electronic","value":"2573-8348"}],"subject":[],"published":{"date-parts":[[2024,8]]},"assertion":[{"value":"2024-04-30","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-10","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-26","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70007"}}