scholarly journals Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Daishi Naoi ◽  
Koji Koinuma ◽  
Hideki Sasanuma ◽  
Yasunaru Sakuma ◽  
Hisanaga Horie ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Pancreatic Tumor ◽  
Laparoscopic Resection ◽  
Tumor Development ◽  
Colonic Polyps ◽  
Pancreatic Tumors ◽  
Beta Catenin ◽  
Adenomatous Polyposis ◽  
Solid Pseudopapillary Neoplasm ◽  
Extracolonic Manifestations

Abstract 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.

A resected case of solid pseudopapillary neoplasm of the pancreas complicated with familial adenomatous polyposis

Pancreatology ◽  
2016 ◽  
Vol 16 (4) ◽  
pp. S138
Author(s):  
Yuko Suzuki ◽  
Kenji Takahashi ◽  
Hidetaka Iwamoto ◽  
Keisuke Yamakita ◽  
Yohei Kitano ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Adenomatous Polyposis ◽  
Solid Pseudopapillary Neoplasm ◽  
Resected Case

scholarly journals Effect of physical fitness on colorectal tumor development in patients with familial adenomatous polyposis

Medicine ◽  
2019 ◽  
Vol 98 (38) ◽  
pp. e17076 ◽  
Author(s):  
Tomiyo Nakamura ◽  
Hideki Ishikawa ◽  
Toshiyuki Sakai ◽  
Makoto Ayabe ◽  
Keiji Wakabayashi ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Physical Fitness ◽  
Tumor Development ◽  
Colorectal Tumor ◽  
Adenomatous Polyposis

scholarly journals Inducible loss of one Apc allele in Lrig1-expressing progenitor cells results in multiple distal colonic tumors with features of familial adenomatous polyposis

2014 ◽  
Vol 307 (1) ◽  
pp. G16-G23 ◽  
Author(s):  
Anne E. Powell ◽  
Gregory Vlacich ◽  
Zhen-Yang Zhao ◽  
Eliot T. McKinley ◽  
M. Kay Washington ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Progenitor Cells ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Tumor Formation ◽  
Therapeutic Interventions ◽  
Adenomatous Polyposis ◽  
Extracolonic Manifestations ◽  
Predictable Pattern ◽  
Leucine Rich Repeats

Individuals with familial adenomatous polyposis (FAP) harbor a germline mutation in adenomatous polyposis coli ( APC). The major clinical manifestation is development of multiple colonic tumors at a young age due to stochastic loss of the remaining APC allele. Extracolonic features, including periampullary tumors, gastric abnormalities, and congenital hypertrophy of the retinal pigment epithelium, may occur. The objective of this study was to develop a mouse model that simulates these features of FAP. We combined our Lrig1-CreERT2/+ mice with Apcfl/+ mice, eliminated one copy of Apc in leucine-rich repeats and immunoglobulin-like domains protein 1 (Lrig1)-positive (Lrig1+) progenitor cells with tamoxifen injection, and monitored tumor formation in the colon by colonoscopy and PET. Initial loss of one Apc allele in Lrig1+ cells results in a predictable pattern of preneoplastic changes, culminating in multiple distal colonic tumors within 50 days of induction, as well as the extracolonic manifestations of FAP mentioned above. We show that tumor formation can be monitored by noninvasive PET imaging. This inducible stem cell-driven model recapitulates features of FAP and offers a tractable platform on which therapeutic interventions can be monitored over time by colonoscopy and noninvasive imaging.

Pancreaticoduodenectomy for Dysplastic Duodenal Adenoma in a Patient with Familial Adenomatous Polyposis

2008 ◽  
Vol 94 (6) ◽  
pp. 882-884
Author(s):  
Roberto Merenda ◽  
Giuseppe Portale ◽  
Francesca Galeazzi ◽  
Chiara Tosolini ◽  
Giacomo Carlo Sturniolo ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Colonic Polyps ◽  
Clinical History ◽  
Adenomatous Polyposis ◽  
Duodenal Adenoma ◽  
Colorectal Polyposis ◽  
Duodenal Polyps ◽  
Younger Age ◽  
History Of ◽  
Malignant Lesions

Colorectal polyposis is the main feature of familial adenomatous polyposis (FAP), but benign and malignant lesions have also been described in the stomach, duodenum, small bowel, biliary tract and pancreas. There are few reports on FAP patients with duodenal polyps that developed at a younger age and even fewer on cases with dysplastic degeneration. The progression to carcinoma usually presents quite late in the clinical history of FAP patients, typically at least 20 to 25 years after proctocolectomy. This report described the rare case of a patient presenting with duodenal adenomas with dysplastic changes and tumor infiltration as the first sign of FAP, who was treated by pancreaticoduodenectomy followed by proctocolectomy for subsequent dysplastic changes in colonic polyps.

Patterns of Adenoma Recurrence in Familial Adenomatous Polyposis Patients after Ileal Pouch-Anal Anastomosis

2015 ◽  
Vol 32 (6) ◽  
pp. 421-425 ◽  
Author(s):  
Adam L. Goldstein ◽  
Revital Kariv ◽  
Joseph M. Klausner ◽  
Hagit Tulchinsky
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Ileal Pouch ◽  
Medical Center ◽  
Colonic Polyps ◽  
Ileal Pouch Anal Anastomosis ◽  
Adenomatous Polyposis ◽  
Adenoma Recurrence ◽  
Duodenal Adenomas ◽  
Anal Anastomosis

Aim: This study aims at identifying the risk factors for the development of pre-cancerous pouch and/or cuff adenomas post ileal pouch anal anastomosis (IPAA) in familial adenomatous polyposis patients. Method: We retrospectively studied 59 patients operated at a single medical center during a 26-year period. Data on the timing and location of adenoma recurrence were recorded and possible correlations with patients' gender, age, presence of desmoid tumor, duodenal adenomas, type of anastomosis and number of operation stages were analyzed. Results: Thirty-five (59%) patients had at least one adenoma in either the cuff or the pouch, including 20 with isolated cuff adenomas, 4 with isolated pouch adenomas and 11 patients with adenomas in both the pouch and cuff. There was no significant correlation between gender, age at surgery, type of anastomosis, number of operative stages and the development of pouch or cuff adenomas. Desmoid tumor and pouch adenomas were significantly correlated with cuff adenomas formation. Duodenal adenomas were associated with pouch adenomas. There was some relationship between the development of cuff adenomas and the burden of colonic polyps, as well as the presence of duodenal adenomas. Conclusion: Adenomas in both the pouch and cuff commonly occur following IPAA, mandating lifelong annual endoscopic surveillance.

scholarly journals Expression Profiling of Proliferation and Apoptotic Markers along the Adenoma-Carcinoma Sequence in Familial Adenomatous Polyposis Patients

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Jayson Wang ◽  
Nabil El-Masry ◽  
Ian Talbot ◽  
Ian Tomlinson ◽  
Malcolm R. Alison ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Cyclin D1 ◽  
Caspase 3 ◽  
Normal Mucosa ◽  
Beta Catenin ◽  
Adenomatous Polyposis ◽  
Ki 67 ◽  
Cd10 Expression ◽  
Apc Protein ◽  
Apoptotic Factors

Introduction. Familial adenomatous polyposis (FAP) patients have a germline mutation in the adenomatous polyposis coli (APC) gene. The APC protein interacts with beta-catenin, resulting in the activation of the Wnt signalling pathway. This results in alterations in cell proliferation and apoptosis. We investigated the expression of beta-catenin and related proliferation and apoptotic factors in FAP patients, exploring the expression along the adenoma-carcinoma sequence.Methods. The expression of beta-catenin, p53, bcl-2, cyclin-D1, caspase-3, CD10, and Ki-67 proteins was studied by immunohistochemistry in samples of colonic nonneoplastic mucosa (n=71), adenomas (n=152), and adenocarcinomas (n=19) from each of the16 FAP patients.Results. The expression of beta-catenin, caspase-3, cyclin-D1, and Ki-67 was increased in both adenomas and carcinomas in FAP patients, compared with normal mucosa. p53 and CD10 expression was only slightly increased in adenomas, but more frequently expressed in carcinomas. Bcl-2 expression was increased in adenomas, but decreased in carcinomas.Conclusion. This is the first study investigating collectively the expression of these molecules together in nonneoplastic mucosa, adenomas, and carcinomas from FAP patients. We find that beta-catenin and related proliferative and apoptotic factors (cyclin-D1, bcl-2, caspase-3, and Ki-67) are expressed early in the sequence, in adenomas. However, p53 and CD10 are often expressed later in the sequence, in carcinomas.

scholarly journals Neoplasia sólida pseudopapilar del páncreas. Reporte de un caso

2020 ◽  
Vol 50 (3) ◽  
Author(s):  
Ignacio Maldonado Schoijet ◽  
Javiera Vásquez Poblete ◽  
Sergio Álvarez Diaz ◽  
Javier González Moscoso
Keyword(s):  
Pancreatic Tumor ◽  
Abdominal Ultrasound ◽  
Good Prognosis ◽  
Cystic Mass ◽  
The Body ◽  
Pancreatic Tumors ◽  
Imaging Features ◽  
Solid Pseudopapillary Neoplasm ◽  
Resonance Imaging ◽  
The One

Background. Solid pseudopapillary neoplasm of the pancreas is an uncommon exocrine pancreatic tumor of epithelial origin, which represents only 1% to 2% of all pancreatic tumors and mainly affects young women. Objective. To describe clinical and imaging features of these rare entity in a symptomatic young woman. Case report. 41-year-old female patient, without morbid history, consulted for intermittent epigastralgia of one-month evolution. Abdominal ultrasound and magnetic resonance imaging demonstrated the presence of a solid-cystic mass of neoplastic appearance in the body-tail of the pancreas. The case was presented to the institutional hepato-biliary committee where surgical resection was decided. Discussion. Although solid pseudopapillary neoplasm of the pancreas are very uncommon pancreatic tumors, it is necessary to keep them within the differential diagnosis on the one hand due to their low but existing malignant potential and on the other hand due to their good prognosis with surgical treatment.

Variable Phenotypic Expression of Identical MYH Germline Mutations in Siblings with Attenuated Familial Adenomatous Polyposis

2007 ◽  
Vol 73 (12) ◽  
pp. 1250-1253 ◽  
Author(s):  
Mustafa Raoof ◽  
Robert J. Canter ◽  
Philip B. Paty
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Locally Advanced ◽  
Phenotypic Expression ◽  
Clinical Manifestations ◽  
Colon Adenocarcinoma ◽  
Colonic Polyps ◽  
Germline Mutations ◽  
Adenomatous Polyposis ◽  
Attenuated Familial Adenomatous Polyposis ◽  
Myh Gene

Germline mutations in the Mutant-Y-homologue (MYH) gene have been linked to an attenuated form of familial adenomatous polyposis in patients who express a wild-type adenomatous polyposis coli gene. However, the diverse clinical manifestations of MYH mutations have not been fully elucidated. We report a case of siblings with identical germline mutations in the MYH gene, one of whom developed a locally advanced colon adenocarcinoma with few other adenomatous lesions, whereas the other had numerous benign colonic polyps. The variable genotype–phenotype manifestations of MYH mutations and the attenuated familial adenomatous polyposis syndrome are discussed.

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