MicroRNA Processor Tumor Predisposition Syndrome

**Semantic type:** Disease or Syndrome

**Definition:** An autosomal dominant inherited tumor predisposition syndrome caused by germline mutations in the DROSHA gene or DGCR8 gene. Together these two genes encode the microprocessor complex that mediates the genesis of microRNAs. Carriers are at an increased risk of developing various neoplasms, including thyroid neoplasms and schwannomas.