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Pediatric Surgery International
Published in: Pediatric Surgery International 4/2020

Open Access 01-04-2020 | Trisomy 21 | Original Article

The overall risk of malignancies is not increased in patients with Hirschsprung disease

Authors: Anna Löf Granström, Gabriella Cohn-Cedermark, Tomas Wester

Published in: Pediatric Surgery International | Issue 4/2020

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Abstract

Purpose

Hirschsprung disease (HSCR) has previously been associated with increased risk of medullary thyroid cancer. The aim of this study was to assess the overall risk of malignancies in patients with Hirschsprung disease in a population-based cohort.

Methods

This was a nationwide, population-based cohort study. The study exposure was HSCR and the study outcome was malignancy. The cohort included all individuals with HSCR registered in the Swedish National Patient Register between 1964 and 2013 and ten age- and sex-matched controls per patient, randomly selected from the Population Register. Data were linked with the Swedish National Cancer Register to identify individuals with malignancy diagnosis.

Results

The cohort comprised 739 individuals with HSCR (565 male) and 7390 controls (5650 male). Median age of the cohort was 19 years (range 2–49). In total nine (1.2%) individuals in the exposed cohort were diagnosed with malignancies compared to 57 (0.8%) in the non-exposed cohort (p = 0.195). Median age at malignancy diagnosis was 3 years (range 0–46) in the exposed group, compared to 23 (range 0–42), p = 0.132. No cases with medullary carcinoma of the thyroid were found in this cohort.

Conclusions

There was no significant difference in risk of malignancies in the exposed group compared to the unexposed group.
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Metadata
Title
The overall risk of malignancies is not increased in patients with Hirschsprung disease
Authors
Anna Löf Granström
Gabriella Cohn-Cedermark
Tomas Wester
Publication date
01-04-2020
Publisher
Springer Berlin Heidelberg
Keyword
Trisomy 21
Published in
Pediatric Surgery International / Issue 4/2020
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-020-04631-1

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