scholarly journals Molecular and Pathological Features of Gastric Cancer in Lynch Syndrome and Familial Adenomatous Polyposis

2018 ◽  
Vol 19 (6) ◽  
pp. 1682 ◽  
Author(s):  
Mara Fornasarig ◽  
Raffaella Magris ◽  
Valli De Re ◽  
Ettore Bidoli ◽  
Vincenzo Canzonieri ◽  
...  
2021 ◽  
Author(s):  
Jose G. Guillem ◽  
John B Ammori

The majority of cases of inherited colorectal cancer (CRC) are accounted for by two syndromes: Lynch syndrome and familial adenomatous polyposis (FAP). In the management of FAP, the role of prophylactic surgery is clearly defined, although the optimal procedure for an individual patient depends on a number of factors. In the management of Lynch syndrome, the indications for prophylactic procedures are emerging. The authors address the clinical evaluation, investigation findings, medical and surgical therapy, and extracolonic diseases of FAP, attenuated form of FAP (AFAP), MYH-associated polyposis, Lynch syndrome, familial colorectal cancer type X (FCCTX), hyperplastic polyposis syndrome, Peutz-Jeghers syndrome, and juvenile polyposis syndrome. AFAP has been described that is associated with fewer adenomas and later development of CRC compared with classic FAP. The AFAP phenotype occurs in less than 10% of FAP patients. The clinical criteria for AFAP are no family members with more than 100 adenomas before the age of 30 years and (1) at least two patients with 10 to 99 adenomas at age over 30 years or (2) one patient with 10 to 99 adenomas at age over 30 years and a first-degree relative with CRC with few adenomas. Given that polyposis has a later onset and the risk of CRC is less well established in AFAP, some authors question whether prophylactic colectomy is necessary in all AFAP patients. This review contains 26 tables and 173 references Keywords: Colorectal cancer, Lynch syndrome, hyperplastic polyp, Peutz-Jeghers syndrome, juvenile polyposis syndrome, familial adenomatous polyposis


Endoscopy ◽  
2020 ◽  
Author(s):  
Isabel Martin ◽  
Victorine H. Roos ◽  
Chukwuemeka Anele ◽  
Sarah-Jane Walton ◽  
Victoria Cuthill ◽  
...  

Abstract Background Patients with familial adenomatous polyposis (FAP) are at increased risk of developing gastric adenomas. There is limited understanding of their clinical course and no consensus on management. We reviewed the management of gastric adenomas in patients with FAP from two centers. Methods Patients with FAP and histologically confirmed gastric adenomas were identified between 1997 and 2018. Patient demographics, adenoma characteristics, and management/surveillance outcomes were collected. Results Of 726 patients with FAP, 104 (14 %; 49 female) were diagnosed with gastric adenomas at a median age of 47 years (range 19 – 80). The median size of gastric adenomas was 6 mm (range 1.5 – 50); 64 (62 %) patients had adenomas located distally to the incisura. Five patients (5 %) had gastric adenomas demonstrating high-grade dysplasia (HGD) on initial diagnosis, distributed equally within the stomach. The risk of HGD was associated with adenoma size (P = 0.04). Of adenomas > 20 mm, 33 % contained HGD. Two patients had gastric cancer at initial gastric adenoma diagnosis. A total of 63 patients (61 %) underwent endoscopic therapy for gastric adenomas. Complications occurred in three patients (5 %) and two (3 %) had recurrence, all following piecemeal resection of large (30 – 50 mm) lesions. Three patients were diagnosed with gastric cancer at median follow-up of 66 months (range 66 – 115) after initial diagnosis. Conclusions We observed gastric adenomas in 14 % of patients with FAP. Of these, 5 % contained HGD; risk of HGD correlated with adenoma size. Endoscopic resection was feasible, with few complications and low recurrence rates, but did not completely eliminate the cancer risk.


2015 ◽  
Vol 51 ◽  
pp. S335
Author(s):  
F. Taniguchi ◽  
T. Nagasaka ◽  
Y. Takehara ◽  
Y. Umeda ◽  
Y. Mori ◽  
...  

2013 ◽  
Vol 229 (2) ◽  
pp. 143-146 ◽  
Author(s):  
Chikashi Shibata ◽  
Hitoshi Ogawa ◽  
Koh Miura ◽  
Takeshi Naitoh ◽  
Jun-ichiro Yamauchi ◽  
...  

2018 ◽  
Vol 31 (03) ◽  
pp. 161-167
Author(s):  
Molly Ford

AbstractGrowing knowledge of inherited colorectal cancer syndromes has led to better surveillance and better care of this subset of patients. The most well-known entities, including Lynch syndrome and familial adenomatous polyposis, are continually being studied and with the advent of more sophisticated genetic testing, additional genetic discoveries have been made in the field of inherited cancer. This article will summarize many of the updates to both the familiar and perhaps less familiar syndromes that can lead to inherited or early-onset colorectal cancer.


2017 ◽  
Vol 153 (2) ◽  
pp. 353-354 ◽  
Author(s):  
Gautam Mankaney ◽  
Carol A. Burke ◽  
Michael Cruise ◽  
James Church ◽  
Vaibhav Wadhwa ◽  
...  

2004 ◽  
Vol 49 (2) ◽  
pp. 260-265 ◽  
Author(s):  
Shouji Shimoyama ◽  
Fumio Aoki ◽  
Masaki Kawahara ◽  
Naohisa Yahagi ◽  
Toru Motoi ◽  
...  

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