{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T16:14:51Z","timestamp":1778170491789,"version":"3.51.4"},"reference-count":18,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T00:00:00Z","timestamp":1611792000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T00:00:00Z","timestamp":1611792000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["surg case rep"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Familial adenomatous polyposis (FAP) is characterized by the presence of hundreds to thousands of colonic polyps, and extracolonic manifestations are likely to occur. Pancreatic tumors are rare extracolonic manifestations in patients with FAP, among which solid-pseudopapillary neoplasm (SPN) are extremely rare. We report here a patient with an SPN of the pancreas found during the follow-up of FAP.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 20-year-old woman was diagnosed with FAP 3 years previously by colonoscopy which revealed less than 100 colonic polyps within the entire colon. She complained of left upper abdominal pain and a 10-cm solid and cystic pancreatic tumor was found by computed tomography scan. Solid and cystic components within the tumor were seen on abdominal magnetic resonance imaging. Simultaneous laparoscopic resection of the distal pancreas and subtotal colectomy was performed. Histopathological findings confirmed the pancreatic tumor as an SPN without malignancy. Abnormal staining of beta-catenin was observed by immunohistochemical study. Multiple polyps in the colorectum were not malignant. Molecular biological analysis from peripheral blood samples revealed a decrease in the copy number of the promoter 1A and 1B region of the <jats:italic>APC<\/jats:italic> gene, which resulted in decreased expression of the <jats:italic>APC<\/jats:italic> gene.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>A rare association of SPN with FAP is reported. The genetic background with relation to beta-catenin abnormalities is interesting to consider tumor development. So far, there are few reports of SPN in a patient with FAP. Both lesions were treated simultaneously by laparoscopic resection.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s40792-021-01121-x","type":"journal-article","created":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T18:03:30Z","timestamp":1611857010000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report"],"prefix":"10.1186","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5557-762X","authenticated-orcid":false,"given":"Daishi","family":"Naoi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koji","family":"Koinuma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideki","family":"Sasanuma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasunaru","family":"Sakuma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hisanaga","family":"Horie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alan Kawarai","family":"Lefor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kokichi","family":"Sugano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mineko","family":"Ushiama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Teruhiko","family":"Yoshida","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naohiro","family":"Sata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,1,28]]},"reference":[{"key":"1121_CR1","doi-asserted-by":"publisher","first-page":"704","DOI":"10.1136\/gut.2007.136127","volume":"57","author":"HF Vasen","year":"2008","unstructured":"Vasen HF, Moslein G, Alonso A, Aretz S, Bernstein I, Bertario L, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut. 2008;57:704\u201313.","journal-title":"Gut"},{"key":"1121_CR2","doi-asserted-by":"publisher","first-page":"446","DOI":"10.1136\/gut.51.3.446","volume":"51","author":"F Maire","year":"2002","unstructured":"Maire F, Hammel P, Terris B, Olschwang S, O\u2019Toole D, Sauvanet A, et al. Intraductal papillary and mucinous pancreatic tumour: a new extracolonic tumour in familial adenomatous polyposis. Gut. 2002;51:446\u20139.","journal-title":"Gut"},{"key":"1121_CR3","doi-asserted-by":"publisher","first-page":"2439","DOI":"10.1245\/s10434-008-9981-3","volume":"15","author":"EJ Groen","year":"2008","unstructured":"Groen EJ, Roos A, Muntinghe FL, Enting RH, de Vries J, Kleibeuker JH, et al. Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol. 2008;15:2439\u201350.","journal-title":"Ann Surg Oncol"},{"key":"1121_CR4","doi-asserted-by":"publisher","first-page":"837","DOI":"10.1053\/gast.2000.16508","volume":"119","author":"RW Burt","year":"2000","unstructured":"Burt RW. Colon cancer screening. Gastroenterology. 2000;119:837\u201353.","journal-title":"Gastroenterology"},{"key":"1121_CR5","doi-asserted-by":"publisher","first-page":"1349","DOI":"10.2169\/internalmedicine.54.4061","volume":"54","author":"T Inoue","year":"2015","unstructured":"Inoue T, Nishi Y, Okumura F, Mizushima T, Nishie H, Iwasaki H, et al. Solid pseudopapillary neoplasm of the pancreas associated with familial adenomatous polyposis. Intern Med. 2015;54:1349\u201355.","journal-title":"Intern Med"},{"key":"1121_CR6","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1016\/S1091-255X(02)00045-8","volume":"6","author":"L Ruo","year":"2002","unstructured":"Ruo L, Coit DG, Brennan MF, Guillem JG. Long-term follow-up of patients with familial adenomatous polyposis undergoing pancreaticoduodenal surgery. J Gastrointest Surg. 2002;6:671\u20135.","journal-title":"J Gastrointest Surg"},{"key":"1121_CR7","first-page":"447","volume":"3","author":"F Farahmand","year":"2012","unstructured":"Farahmand F, Shoaran M, Fariborzi M, Ashjaei B, Monajemzadeh M, Mehdizadeh M. Pancreatic pseudopapillary tumor in association with colonic polyposis. J Med Med Sci. 2012;3:447\u201351.","journal-title":"J Med Med Sci"},{"key":"1121_CR8","doi-asserted-by":"publisher","first-page":"26011","DOI":"10.1038\/srep26011","volume":"6","author":"K Yamaguchi","year":"2016","unstructured":"Yamaguchi K, Nagayama S, Shimizu E, Komura M, Yamaguchi R, Shibuya T, et al. Reduced expression of APC-1B but not APC-1A by the deletion of promoter 1B is responsible for familial adenomatous polyposis. Sci Rep. 2016;6:26011.","journal-title":"Sci Rep"},{"key":"1121_CR9","doi-asserted-by":"publisher","first-page":"1382","DOI":"10.5858\/arpa.2018-0570-RA","volume":"143","author":"P Dinarvand","year":"2019","unstructured":"Dinarvand P, Davaro EP, Doan JV, Ising ME, Evans NR, Phillips NJ, et al. familial adenomatous polyposis syndrome: an update and review of extraintestinal manifestations. Arch Pathol Lab Med. 2019;143:1382\u201398.","journal-title":"Arch Pathol Lab Med"},{"key":"1121_CR10","doi-asserted-by":"publisher","first-page":"308","DOI":"10.1007\/s10147-004-0414-4","volume":"9","author":"T Iwama","year":"2004","unstructured":"Iwama T, Tamura K, Morita T, Hirai T, Hasegawa H, Koizumi K, et al. A clinical overview of familial adenomatous polyposis derived from the database of the Polyposis Registry of Japan. Int J Clin Oncol. 2004;9:308\u201316.","journal-title":"Int J Clin Oncol"},{"key":"1121_CR11","doi-asserted-by":"publisher","first-page":"965","DOI":"10.1016\/j.jamcollsurg.2005.02.011","volume":"200","author":"T Papavramidis","year":"2005","unstructured":"Papavramidis T, Papavramidis S. Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg. 2005;200:965\u201372.","journal-title":"J Am Coll Surg"},{"key":"1121_CR12","doi-asserted-by":"publisher","first-page":"747","DOI":"10.1007\/s00423-010-0599-0","volume":"395","author":"L Romics Jr","year":"2010","unstructured":"Romics L Jr, Olah A, Belagyi T, Hajdu N, Gyurus P, Ruszinko V. Solid pseudopapillary neoplasm of the pancreas\u2014proposed algorithms for diagnosis and surgical treatment. Langenbecks Arch Surg. 2010;395:747\u201355.","journal-title":"Langenbecks Arch Surg"},{"key":"1121_CR13","doi-asserted-by":"publisher","first-page":"276","DOI":"10.1016\/j.jss.2005.11.585","volume":"131","author":"CH Peng","year":"2006","unstructured":"Peng CH, Chen DF, Zhou GW, Yang WP, Tao ZY, Lei RQ, et al. The solid-pseudopapillary tumor of pancreas: the clinical characteristics and surgical treatment. J Surg Res. 2006;131:276\u201382.","journal-title":"J Surg Res"},{"key":"1121_CR14","doi-asserted-by":"publisher","first-page":"573","DOI":"10.1002\/jhbp.96","volume":"21","author":"I Hosokawa","year":"2014","unstructured":"Hosokawa I, Shimizu H, Ohtsuka M, Kato A, Yoshitomi H, Furukawa K, et al. Preoperative diagnosis and surgical management for solid pseudopapillary neoplasm of the pancreas. J Hepatobiliary Pancreat Sci. 2014;21:573\u20138.","journal-title":"J Hepatobiliary Pancreat Sci"},{"key":"1121_CR15","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1136\/jcp.2004.020925","volume":"58","author":"R Chetty","year":"2005","unstructured":"Chetty R, Salahshor S, Bapat B, Berk T, Croitoru M, Gallinger S. Intraductal papillary mucinous neoplasm of the pancreas in a patient with attenuated familial adenomatous polyposis. J Clin Pathol. 2005;58:97\u2013101.","journal-title":"J Clin Pathol"},{"key":"1121_CR16","doi-asserted-by":"publisher","first-page":"492","DOI":"10.1097\/PPO.0b013e31827459b6","volume":"18","author":"LD Wood","year":"2012","unstructured":"Wood LD, Hruban RH. Pathology and molecular genetics of pancreatic neoplasms. Cancer J. 2012;18:492\u2013501.","journal-title":"Cancer J"},{"key":"1121_CR17","doi-asserted-by":"publisher","first-page":"4135","DOI":"10.3748\/wjg.v23.i23.4135","volume":"23","author":"L Roncucci","year":"2017","unstructured":"Roncucci L, Pedroni M, Mariani F. Attenuated adenomatous polyposis of the large bowel: present and future. World J Gastroenterol. 2017;23:4135\u20139.","journal-title":"World J Gastroenterol"},{"key":"1121_CR18","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1023\/A:1023286520725","volume":"2","author":"AL Knudsen","year":"2003","unstructured":"Knudsen AL, Bisgaard ML, B\u00fclow S. Attenuated familial adenomatous polyposis (AFAP). A review of the literature. Fam Cancer. 2003;2:43\u201355.","journal-title":"Fam Cancer."}],"container-title":["Surgical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s40792-021-01121-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s40792-021-01121-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s40792-021-01121-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,1,29]],"date-time":"2021-01-29T06:42:04Z","timestamp":1611902524000},"score":1,"resource":{"primary":{"URL":"https:\/\/surgicalcasereports.springeropen.com\/articles\/10.1186\/s40792-021-01121-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,28]]},"references-count":18,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["1121"],"URL":"https:\/\/doi.org\/10.1186\/s40792-021-01121-x","relation":{},"ISSN":["2198-7793"],"issn-type":[{"value":"2198-7793","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1,28]]},"assertion":[{"value":"14 January 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 January 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 January 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The need for ethical approval for this paper was waived by the committee of Jichi Medical University Hospital. Genetic Counseling and subsequent DNA testing of the <i>APC<\/i> gene was performed at the division of the genetic counseling clinic, Tochigi Cancer Center and the department of genetic medicine and services, National Cancer Center Hospital, Tokyo, according to the protocol approved by the IRB at the Tochigi Cancer Center (Study Nos. A-019 and A-291).","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of this case report and accompanying images.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"All authors declare no conflicts of interest regarding the publication of this paper.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"35"}}