Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2021-01-28

AUTHORS

Daishi Naoi, Koji Koinuma, Hideki Sasanuma, Yasunaru Sakuma, Hisanaga Horie, Alan Kawarai Lefor, Kokichi Sugano, Mineko Ushiama, Teruhiko Yoshida, Naohiro Sata

ABSTRACT

BackgroundFamilial 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.Case presentationA 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 APC gene, which resulted in decreased expression of the APC gene.ConclusionsA 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. More... »

PAGES

35

Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/s40792-021-01121-x

DOI

http://dx.doi.org/10.1186/s40792-021-01121-x

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1134949539

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/33511474


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