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Surgery Today
Published in: Surgery Today 3/2018

01-03-2018 | Review Article

Malignant tumors associated with juvenile polyposis syndrome in Japan

Authors: Hideyuki Ishida, Keiichiro Ishibashi, Takeo Iwama

Published in: Surgery Today | Issue 3/2018

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Abstract

Purpose

The risk of malignant tumors developing in association with juvenile polyposis syndrome (JPS) was evaluated to explore the optimal treatment for this rare disease.

Methods

We reviewed the data on JPS cases reported in Japan between January, 1971 and March, 2016.

Results

A total of 171 cases were evaluable. Of these 171 patients, 83 (48.5%) were female and the median age at diagnosis was 28 years (range 1–80 years). The polyps were located in the stomach alone (n = 62; 36.3%), in the stomach and intestine (n = 47; 27.4%), or in the colorectum alone (n = 62; 36.3%). The sites of malignant tumors were the stomach (n = 31), colorectum (n = 29), small intestine (n = 2), breast (n = 1), and thyroid (n = 1). The lifetime risk (at 70 years) of any malignant tumor was 86.2%. The lifetime risk of gastric cancer was 73.0% and that of colorectal cancer was 51.1%. The risk of these cancers developing was dependent on the type of polyp distribution.

Conclusions

Long-term surveillance of the stomach and colorectum based on the phenotype of JPS seems a reasonable approach to monitor these patients for the development of malignant tumors.
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Metadata
Title
Malignant tumors associated with juvenile polyposis syndrome in Japan
Authors
Hideyuki Ishida
Keiichiro Ishibashi
Takeo Iwama
Publication date
01-03-2018
Publisher
Springer Singapore
Published in
Surgery Today / Issue 3/2018
Print ISSN: 0941-1291
Electronic ISSN: 1436-2813
DOI
https://doi.org/10.1007/s00595-017-1538-2

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