Lynch syndrome
ORPHA:144· ICD-10 C18.2
Definition
A rare form of hereditary nonpolyposis colon cancer (HNPCC) characterized by predisposition to a wide variety of cancers, including neoplasms of the digestive tract, urinary tract, endometrium, ovary, brain, and prostate, as well as sebaceous skin tumors. LS-associated tumors are typically characterized by the presence of microsatellite instability (MSI) and loss of expression of MMR proteins in tumor tissue.
- Prevalence
- Unknown
- Inheritance
- Autosomal dominant
- Age of onset
- Adult