The overall risk of malignancies is not increased in patients with Hirschsprung disease
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In brief
In brief
Swedish nationwide cohort study of 739 HSCR patients found no increased overall malignancy risk compared to matched controls, contradicting previous associations with medullary thyroid cancer. Median follow-up age was 19 years with similar cancer rates between groups (1.2% vs 0.8%, p=0.195).
Written by the GCMD Library team from the article.
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.