Definition

A rare, inherited disorder that is caused by a mutation (change) in a gene called DICER1. Having this mutation increases the risk of certain types of tumors, including tumors of the kidney, thyroid, ovary, cervix, testicle, brain, eye, and lining of the lung. The tumors may be benign (not cancer) or malignant (cancer). A goiter (an enlarged thyroid) and polyps in the colon may also occur. Not all people who have a mutation in the DICER1 gene will develop tumors.