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Hereditary Cancer in Clinical Practice
Published in: Hereditary Cancer in Clinical Practice 1/2014

Open Access 01-12-2014 | Research

Familial cancer among consecutive uterine cancer patients in Sweden

Authors: Gerasimos Tzortzatos, Ofra Wersäll, Kristina Gemzell Danielsson, Annika Lindblom, Emma Tham, Miriam Mints

Published in: Hereditary Cancer in Clinical Practice | Issue 1/2014

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Abstract

Background

Uterine cancer (UC) represents 5.1% of all female malignancies in Sweden. Accumulation of UC in families occurs in around 5% of cases. We wanted to identify any familial association between UC and other selected cancers and to study the frequency of Lynch,Cowden and cancer syndromes among consecutive UC patients in Sweden.

Methods

481 UC patients were included. Information on the cancer diagnoses of their relatives (first- (FDRs) and second-degree (SDRs) relatives and first cousins) was obtained. The relative frequencies of different cancers among relatives were compared to those in the Swedish general cancer population in 1970 and 2010. Families that fulfilled the criteria for hereditary cancer syndromes were tested for mutations in the causative genes. Families with at least one case of UC in addition to the index patient were compared to families with no additional cases to investigate possible characteristics of putative hereditary cancer syndromes.

Results

There was an increased prevalence of UC in our study population compared to the Swedish general cancer population in 1970 and 2010 (6% vs. 4% and 3%, respectively). Seven families had Lynch Syndrome according to the Amsterdam II criteria. No families fulfilled the criteria for Cowden syndrome. In total 13% of index patients had at least one relative with UC and these families tended to have more cases of early onset cancer among family members. In addition, 16% of index patients were diagnosed with at least one other cancer. No families fulfilled the criteria for Cowden syndrome.

Conclusion

We showed a familial clustering of UC among relatives of our index patients. Of the seven families with mutation-verified Lynch Syndrome, only one had been previously diagnosed, highlighting the need to increase gynecologists’ awareness of the importance of taking family history. Our data on multiple cancers and young age of onset in families with uterine cancer is compatible with the existence of additional hereditary uterine cancer syndromes.
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Metadata
Title
Familial cancer among consecutive uterine cancer patients in Sweden
Authors
Gerasimos Tzortzatos
Ofra Wersäll
Kristina Gemzell Danielsson
Annika Lindblom
Emma Tham
Miriam Mints
Publication date
01-12-2014
Publisher
BioMed Central
Published in
Hereditary Cancer in Clinical Practice / Issue 1/2014
Electronic ISSN: 1897-4287
DOI
https://doi.org/10.1186/1897-4287-12-14

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