Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
Fam Cancer. 2009;8(3):209-13. doi: 10.1007/s10689-008-9230-8. Epub 2009 Jan 8.

Sarcomas associated with hereditary nonpolyposis colorectal cancer: broad anatomical and morphological spectrum.

Author information

  • 1Clinical Research Centre, Hvidovre Hospital, Copenhagen University, KettegĂ„rd allĂ© 30, 2650 Hvidovre, Denmark. Mef.Nilbert@med.lu.se

Abstract

Hereditary nonpolyposis colorectal cancer (HNPCC) is primarily linked to colorectal and endometrial cancer, but is associated with a broad tumor spectrum. Though not formally part of the syndrome, occasional sarcomas have been reported in individuals with HNPCC. We used the national Danish HNPCC-register to identify HNPCC families in which sarcomas had been diagnosed. Fourteen sarcomas were identified in families with mutations in MSH2, MSH6, and MLH1. The median age at sarcoma diagnosis was 43 (15-74) years. Soft tissue sarcomas predominated followed by uterine sarcomas and eight histopathological subtypes were represented with recurrent diagnoses of liposarcoma, leiomyosarcoma, and carcinosarcoma. Tumor tissue from eight cases was available for analysis of mismatch-repair (MMR) status using immunohistochemical staining and analysis of microsatellite instability, which revealed MMR defects in six of the eight tumors investigated. This suggests that sarcomas may be part of the HNPCC tumor spectrum and that colorectal cancer should be considered in the family history of sarcoma patients.

PMID:
19130300
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Springer
    Write to the Help Desk