Survival of hereditary non-polyposis colorectal cancer patients compared with sporadic colorectal cancer patients.

Vittoria Stigliano,Marcella Mottolese,Vincenzo Casale,Daniela Assisi,Lupe Sanchez Mete,Diana Giannarelli,Maurizio Cosimelli,Raffaele Palmirotta,Raffaello Mancini

doi:10.1186/1756-9966-27-39

Abstract

BackgroundPatients with hereditary non-poliposys colorectal cancer (HNPCC) have better prognosis than sporadic colorectal cancer (CRC). Aim of our retrospective study was to compare the overall survival between sporadic CRC and HNPCC patients.MethodsWe analyzed a cohort of 40 (25 males and 15 females) HNPCC cases with a hospital consecutive series of 573 (312 males and 261 females) sporadic CRC observed during the period 1970–1993. In 15 HNPCC patients we performed mutational analysis for microsatellite instability. Survival rates were calculated by Kaplan-Meier method and compared with log rank test.ResultsThe median age at diagnosis of the primary CRC was 46.8 years in the HNPCC series versus 61 years in sporadic CRC group. In HNPCC group 85% had a right cancer location, vs. 57% in the sporadic cancer group. In the sporadic cancer group 61.6% were early-stages cancer (Dukes' A and B) vs. 70% in the HNPCC group (p = ns). The crude 5-years cumulative survival after the primary CRC was 94.2% in HNPCC patients vs. 75.3% in sporadic cancer patients (p < 0.0001).ConclusionOur results show that overall survival of colorectal cancer in patients with HNPCC is better than sporadic CRC patients. The different outcome probably relates to the specific tumorigenesis involving DNA mismatch repair dysfunction.

Highlights

Patients with hereditary non-poliposys colorectal cancer (HNPCC) have better prognosis than sporadic colorectal cancer (CRC)
The crude 5-years cumulative survival after the primary CRC was 94.2% in HNPCC patients vs. 75.3% in sporadic cancer patients (p < 0.0001)
Our results show that overall survival of colorectal cancer in patients with HNPCC is better than sporadic CRC patients

Summary

Introduction

Patients with hereditary non-poliposys colorectal cancer (HNPCC) have better prognosis than sporadic colorectal cancer (CRC). Colorectal tumours with microsatellite instability (MSI phenotype) have mutations in mismatch repair (MMR) genes and in particular in the MSH2 and MLH1 genes, respectively found on chromosome arms 2p and 3p [5,6,7,8,9]. These mutations lead to inactivation of the genes and to a defect in replication/repair of DNA and an accumulation in the cancer cell genome of ubiquitous somatic clonal mutations [10]. MSI seems to be important in the development of various human cancer such as sporadic endometrial cancer [18] and oral squamous cell carcinoma [19]

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Conclusion