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Hereditary Nonpolyposis Colorectal Cancer (HNPCC): From Diagnosis to Surgical Management of the Main Hereditary Form of Colorectal Cancer: A Review

Author(s): Guido Mantovani, Michela De Angelis, Corrado Asteria, Francesco Di Lecce, Paolo Mazza, Luca Vicenzi, Luigi Boccia

Colorectal cancer (CRC) is the third most common cancer worldwide, the 5% of CRC are hereditary. Among hereditary forms of CRC the most frequent is Hereditary Nonpolyposis Colorectal Cancer (HNPCC). HNPCC is an autosomal dominant genetic disease. Molecular basis of HNPCC are linked to genetic alterations, involving several mismatch repair (MMR) genes. Diagnosis of HNPCC is frequently difficult: HNPCC diagnosis process starts from family medical history and genetic counselling and continue with molecular diagnosis and genetic tests which often identify MMR genes mutation. In HNPCC the role of screening is irreplaceable for prevention and early diagnosis of CRC and extracolic cancer, such as endometrium, ovary, urothelium, stomach, small bowel, pancreas, brain, biliary ducts neoplasms. Surgical management of HNPCC is significantly different between colonic and rectal cancer. The main problem in surgical approach for HNPCC patients is related to the possibility of developing metachronous colorectal cancer. The choice is always between extensive and segmental resection. The aim of this study is to give some practical indications to surgeons for the management of HNPCC patients.

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