Turcot syndrome with polyposis

Definition

Turcot syndrome with polyposis or Turcot syndrome type 2 is a form of familial adematous polyposis, characterized by the concurrence of thousands of colonic adenomatous polyposis or colorectal cancer (CRC) and a primary central nervous system tumor (principally medulloblastoma). It is also associated with pigmented ocular fundus lesions.

Disease data

Classification

Clinical subtype

ORPHA code

99818

OMIM code

175100

ICD10 code

D12.6

ICD11 code

*Soruce