{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T11:08:08Z","timestamp":1774609688986,"version":"3.50.1"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T00:00:00Z","timestamp":1774224000000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Appendiceal goblet cell adenocarcinoma (GCA) is a rare amphicrine neoplasm, which recent studies have characterized as mismatch repair proficient and microsatellite stable. A 49\u2010year\u2010old male presented to our hospital with a Borrmann type 3\u2010like ileocecal tumor extending continuously from a markedly swollen appendix. The patient underwent ileocecal resection. Pathologically, the tumor was diagnosed as high\u2010grade appendiceal GCA, accompanied by lymphocytic infiltration, staged as pStage IVB. Immunohistochemistry revealed loss of MSH2 and MSH6 expression, indicating mismatch repair deficiency (dMMR), and molecular analysis confirmed microsatellite instability (MSI)\u2010high status. Postoperatively, the patient was treated with chemotherapy, including immune checkpoint inhibitors as second\u2010line therapy, which provided temporary relief of duodenal stenosis caused by tumor metastasis. However, the patient died of disease progression 37 months after surgery. To our knowledge, this is the first report of appendiceal GCA with dMMR and MSI\u2010high status. Our findings underscore the necessity of incorporating MMR\/MSI testing into the diagnostic workup of rare tumors such as GCA, particularly in young patients or those with a family history suggestive of Lynch syndrome, to optimize personalized treatment strategies.<\/jats:p>","DOI":"10.1111\/pin.70108","type":"journal-article","created":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T04:21:16Z","timestamp":1774326076000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Appendiceal Goblet Cell Adenocarcinoma With Mismatch Repair Deficiency and Microsatellite Instability\u2010High Status: A Novel Molecular Signature Guiding Immuno\u2010Oncology Strategy"],"prefix":"10.1111","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1455-4974","authenticated-orcid":false,"given":"Hirotsugu","family":"Hashimoto","sequence":"first","affiliation":[{"name":"Department of Pathology Tokyo Women's Medical University Tokyo Japan"},{"name":"Department of Diagnostic Pathology NTT Medical Center Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirotomo","family":"Koda","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology NTT Medical Center Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4048-2895","authenticated-orcid":false,"given":"Kentaro","family":"Nakajima","sequence":"additional","affiliation":[{"name":"Department of Surgery NTT Medical Center Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takuro","family":"Mizukami","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology NTT Medical Center Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1795-4778","authenticated-orcid":false,"given":"Masashi","family":"Kusakabe","sequence":"additional","affiliation":[{"name":"Department of Radiology NTT Medical Center Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7559-2811","authenticated-orcid":false,"given":"Shouichi","family":"Satou","sequence":"additional","affiliation":[{"name":"Department of Surgery NTT Medical Center Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4945-6207","authenticated-orcid":false,"given":"Teppei","family":"Morikawa","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology NTT Medical Center Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,3,23]]},"reference":[{"key":"e_1_2_12_2_1","first-page":"149","volume-title":"WHO Classification of Tumours: Digestive System Tumours","author":"Misdraji J.","year":"2019"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2022.06.002"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.semdp.2024.08.003"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2013-0047-OA"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-021-10192-x"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12935-023-03183-3"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoncol.2020.1046"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.annonc.2024.11.012"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.21.01015"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers17132125"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318282649b"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prp.2014.01.002"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2017.184"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1158\/1535-7163.MCT-20-0318"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2009.12.064"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63994-6"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2006.02.009"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1186\/1749-7922-1-11"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers17243981"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.2353\/jmoldx.2008.080031"}],"container-title":["Pathology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pin.70108","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pin.70108","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pin.70108","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T10:22:00Z","timestamp":1774606920000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pin.70108"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3]]},"references-count":20,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["10.1111\/pin.70108"],"URL":"https:\/\/doi.org\/10.1111\/pin.70108","archive":["Portico"],"relation":{},"ISSN":["1320-5463","1440-1827"],"issn-type":[{"value":"1320-5463","type":"print"},{"value":"1440-1827","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3]]},"assertion":[{"value":"2026-02-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-14","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-23","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70108"}}