PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS
ABSTRACT BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well....
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| Format: | Article |
| Language: | English |
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Colégio Brasileiro de Cirurgia Digestiva
2023-01-01
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| Series: | ABCD: Arquivos Brasileiros de Cirurgia Digestiva |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0102-67202022000100345&tlng=en |
| _version_ | 1797951377331191808 |
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| author | José Donizeti MEIRA-JÚNIOR Gustavo Gonçalves YOGOLARE Daniel de Paiva MAGALHÃES Guilherme Naccache NAMUR Fabio Guilherme CAMPOS Vanderlei SEGATELLI Sergio Carlos NAHAS Jose JUKEMURA |
| author_facet | José Donizeti MEIRA-JÚNIOR Gustavo Gonçalves YOGOLARE Daniel de Paiva MAGALHÃES Guilherme Naccache NAMUR Fabio Guilherme CAMPOS Vanderlei SEGATELLI Sergio Carlos NAHAS Jose JUKEMURA |
| author_sort | José Donizeti MEIRA-JÚNIOR |
| collection | DOAJ |
| description | ABSTRACT BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible. |
| first_indexed | 2024-04-10T22:29:38Z |
| format | Article |
| id | doaj.art-62260bcb3b7a47be9b5f2abcc889df41 |
| institution | Directory Open Access Journal |
| issn | 0102-6720 |
| language | English |
| last_indexed | 2024-04-10T22:29:38Z |
| publishDate | 2023-01-01 |
| publisher | Colégio Brasileiro de Cirurgia Digestiva |
| record_format | Article |
| series | ABCD: Arquivos Brasileiros de Cirurgia Digestiva |
| spelling | doaj.art-62260bcb3b7a47be9b5f2abcc889df412023-01-17T07:31:18ZengColégio Brasileiro de Cirurgia DigestivaABCD: Arquivos Brasileiros de Cirurgia Digestiva0102-67202023-01-013510.1590/0102-672020220002e1718PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSISJosé Donizeti MEIRA-JÚNIORhttps://orcid.org/0000-0002-4064-1166Gustavo Gonçalves YOGOLAREhttps://orcid.org/0000-0002-2932-2666Daniel de Paiva MAGALHÃEShttps://orcid.org/0000-0001-7476-8950Guilherme Naccache NAMURhttps://orcid.org/0000-0001-8102-6568Fabio Guilherme CAMPOShttps://orcid.org/0000-0002-6233-6904Vanderlei SEGATELLIhttps://orcid.org/0000-0002-5588-8508Sergio Carlos NAHAShttps://orcid.org/0000-0002-2268-4146Jose JUKEMURAhttps://orcid.org/0000-0002-3943-7088ABSTRACT BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0102-67202022000100345&tlng=enbeta CateninPancreatectomyColorectal NeoplasmsPancreatic NeoplasmsAdenomatous Polyposis Coli |
| spellingShingle | José Donizeti MEIRA-JÚNIOR Gustavo Gonçalves YOGOLARE Daniel de Paiva MAGALHÃES Guilherme Naccache NAMUR Fabio Guilherme CAMPOS Vanderlei SEGATELLI Sergio Carlos NAHAS Jose JUKEMURA PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS ABCD: Arquivos Brasileiros de Cirurgia Digestiva beta Catenin Pancreatectomy Colorectal Neoplasms Pancreatic Neoplasms Adenomatous Polyposis Coli |
| title | PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS |
| title_full | PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS |
| title_fullStr | PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS |
| title_full_unstemmed | PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS |
| title_short | PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS |
| title_sort | pancreatic solid pseudopapillary neoplasm in patients with familial adenomatous polyposis |
| topic | beta Catenin Pancreatectomy Colorectal Neoplasms Pancreatic Neoplasms Adenomatous Polyposis Coli |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0102-67202022000100345&tlng=en |
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