La Revue de médecine interne
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Review Comparative Study
[HNPCC syndrome (hereditary non polyposis colon cancer): identification and management].
The hereditary non-polyposis colon cancer (HNPCC) syndrome is an inherited condition defined by clinical and genealogical information, known as Amsterdam criteria. In about 70% of cases, HNPCC syndrome is caused by germline mutations in MMR genes, leading to microsatellite instability of tumor DNA (MSI phenotype). Patients affected by the disease are at high risk for colorectal and endometrial carcinomas, but also for other organs tumors. HNPCC syndrome is responsible for 5% of colorectal cancers. ⋯ The identification of germline MMR mutations has no major consequence on the cancer treatments, but influences markedly the long-term follow-up and the management of at-risk relatives. Gene carriers will enter a follow-up program regarding their colorectal and endometrial cancer risks; other organs being at low lifetime risk, no specific surveillance will be proposed.
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Retroperitoneal Liposarcoma is a rare and primary malignancy developed from mesenchymal tissue. It's generally unique and shows an only one histologic component. We report a case of multicentric and synchronous liposarcoma (intraperitoneal and retroperitoneal) and with different histologic types. ⋯ Pathological, therapeutic and prognostic aspects of abdominal liposarcoma will be reviewed in this article.