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Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report
BACKGROUND: 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 (...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7843828/ https://www.ncbi.nlm.nih.gov/pubmed/33511474 http://dx.doi.org/10.1186/s40792-021-01121-x |
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author | Naoi, Daishi Koinuma, Koji Sasanuma, Hideki Sakuma, Yasunaru Horie, Hisanaga Lefor, Alan Kawarai Sugano, Kokichi Ushiama, Mineko Yoshida, Teruhiko Sata, Naohiro |
author_facet | Naoi, Daishi Koinuma, Koji Sasanuma, Hideki Sakuma, Yasunaru Horie, Hisanaga Lefor, Alan Kawarai Sugano, Kokichi Ushiama, Mineko Yoshida, Teruhiko Sata, Naohiro |
author_sort | Naoi, Daishi |
collection | PubMed |
description | BACKGROUND: 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. CASE PRESENTATION: 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 APC gene, which resulted in decreased expression of the APC gene. CONCLUSIONS: 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. |
format | Online Article Text |
id | pubmed-7843828 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-78438282021-02-04 Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report Naoi, Daishi Koinuma, Koji Sasanuma, Hideki Sakuma, Yasunaru Horie, Hisanaga Lefor, Alan Kawarai Sugano, Kokichi Ushiama, Mineko Yoshida, Teruhiko Sata, Naohiro Surg Case Rep Case Report BACKGROUND: 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. CASE PRESENTATION: 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 APC gene, which resulted in decreased expression of the APC gene. CONCLUSIONS: 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. Springer Berlin Heidelberg 2021-01-28 /pmc/articles/PMC7843828/ /pubmed/33511474 http://dx.doi.org/10.1186/s40792-021-01121-x Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. |
spellingShingle | Case Report Naoi, Daishi Koinuma, Koji Sasanuma, Hideki Sakuma, Yasunaru Horie, Hisanaga Lefor, Alan Kawarai Sugano, Kokichi Ushiama, Mineko Yoshida, Teruhiko Sata, Naohiro Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report |
title | Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report |
title_full | Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report |
title_fullStr | Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report |
title_full_unstemmed | Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report |
title_short | Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report |
title_sort | solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7843828/ https://www.ncbi.nlm.nih.gov/pubmed/33511474 http://dx.doi.org/10.1186/s40792-021-01121-x |
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