Early onset colon cancer affected by Lynch syndrome

Lynch syndrome is an autosomal dominant disorder in which one's DNA mismatch repair mechanism is impaired, resulting in an increased risk of developing colon and endometrial cancers, as well as other malignancies. With such an increased risk of colorectal cancer, patients diagnosed with Lynch s...

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Main Authors: Kayla Olson, Larisa Debelenko, Gustavo Stringel, Xiang Da Dong
Format: Article
Language:English
Published: Elsevier 2018-09-01
Series:Journal of Pediatric Surgery Case Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2213576618301477
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author Kayla Olson
Larisa Debelenko
Gustavo Stringel
Xiang Da Dong
author_facet Kayla Olson
Larisa Debelenko
Gustavo Stringel
Xiang Da Dong
author_sort Kayla Olson
collection DOAJ
description Lynch syndrome is an autosomal dominant disorder in which one's DNA mismatch repair mechanism is impaired, resulting in an increased risk of developing colon and endometrial cancers, as well as other malignancies. With such an increased risk of colorectal cancer, patients diagnosed with Lynch syndrome should undergo colonoscopy screening every 1–2 years beginning at ages 20 to 25 or two to five years prior to earliest diagnosis of colorectal cancer in their family members. In patients with Lynch syndrome, colorectal cancer usually presents after the fourth decade of life. Few cases have been reported in pediatric patients with early onset colorectal cancer. We report here a 16-year-old patient who presented initially with symptoms characteristic of appendicitis. Following a CT scan suggesting perforated appendicitis, the patient was treated with intravenous antibiotics with a scheduled interval appendectomy three months later. Pathology reports from the interval appendectomy showed that the specimen contained a well-to-moderately differentiated adenocarcinoma. The patient then underwent robot assisted right hemicolectomy as definitive management, which demonstrated that the tumor originated in the cecum with invasion into the submucosa and focally infiltrating the muscularis propria. This case illustrates the need to be cognizant that cancer can present similarly to appendicitis, to be aware of the increasing incidence of colon cancer in younger patients, and to catalyze the acceptability of robotics for minimally invasive surgery. Keywords: Lynch syndrome, Pediatric colon cancer, Robotic colectomy
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spelling doaj.art-c1f4021ac22b4b51858bb31493f1ef732022-12-22T03:07:14ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662018-09-01361315Early onset colon cancer affected by Lynch syndromeKayla Olson0Larisa Debelenko1Gustavo Stringel2Xiang Da Dong3New York Medical College, USAWestchester Medical Center, Department of Pathology, USAWestchester Medical Center, Department of Surgery, USAWestchester Medical Center, Department of Surgery, USA; Corresponding author.Lynch syndrome is an autosomal dominant disorder in which one's DNA mismatch repair mechanism is impaired, resulting in an increased risk of developing colon and endometrial cancers, as well as other malignancies. With such an increased risk of colorectal cancer, patients diagnosed with Lynch syndrome should undergo colonoscopy screening every 1–2 years beginning at ages 20 to 25 or two to five years prior to earliest diagnosis of colorectal cancer in their family members. In patients with Lynch syndrome, colorectal cancer usually presents after the fourth decade of life. Few cases have been reported in pediatric patients with early onset colorectal cancer. We report here a 16-year-old patient who presented initially with symptoms characteristic of appendicitis. Following a CT scan suggesting perforated appendicitis, the patient was treated with intravenous antibiotics with a scheduled interval appendectomy three months later. Pathology reports from the interval appendectomy showed that the specimen contained a well-to-moderately differentiated adenocarcinoma. The patient then underwent robot assisted right hemicolectomy as definitive management, which demonstrated that the tumor originated in the cecum with invasion into the submucosa and focally infiltrating the muscularis propria. This case illustrates the need to be cognizant that cancer can present similarly to appendicitis, to be aware of the increasing incidence of colon cancer in younger patients, and to catalyze the acceptability of robotics for minimally invasive surgery. Keywords: Lynch syndrome, Pediatric colon cancer, Robotic colectomyhttp://www.sciencedirect.com/science/article/pii/S2213576618301477
spellingShingle Kayla Olson
Larisa Debelenko
Gustavo Stringel
Xiang Da Dong
Early onset colon cancer affected by Lynch syndrome
Journal of Pediatric Surgery Case Reports
title Early onset colon cancer affected by Lynch syndrome
title_full Early onset colon cancer affected by Lynch syndrome
title_fullStr Early onset colon cancer affected by Lynch syndrome
title_full_unstemmed Early onset colon cancer affected by Lynch syndrome
title_short Early onset colon cancer affected by Lynch syndrome
title_sort early onset colon cancer affected by lynch syndrome
url http://www.sciencedirect.com/science/article/pii/S2213576618301477
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AT larisadebelenko earlyonsetcoloncanceraffectedbylynchsyndrome
AT gustavostringel earlyonsetcoloncanceraffectedbylynchsyndrome
AT xiangdadong earlyonsetcoloncanceraffectedbylynchsyndrome