What gene is SMARCB1?

The SMARCB1 gene provides instructions for making a protein that forms one piece (subunit) of several different protein groupings called SWI/SNF protein complexes. SWI/SNF complexes regulate gene activity (expression) by a process known as chromatin remodeling.

What is INI1 gene?

Introduction. The INI1/SNF5/SMARCB1 gene at chromosomal band 22q11. 2 encodes a member of the SWI/SNF chromatin remodeling complex. This complex is a negative regulator of the cell cycle, modulates cytoskeleton organization and functions as a tumor suppressor gene (1-6).

What does Rhabdoid mean?

: a rod-shaped protoplasmic body in the sensitive cells of leaves of various plants of the family Droseraceae.

What is sinonasal undifferentiated carcinoma?

Sinonasal undifferentiated carcinoma (SNUC) is a rare cancer of the nasal cavity and/or paranasal sinuses. Initial symptoms range from bloody nose, runny nose, double vision, and bulging eye to chronic infections and nasal obstruction.

Is rhabdoid tumor curable?

Long-term Outcomes for Children with Atypical Teratoid Rhabdoid Tumor (ATRT) ATRT is challenging to cure, and, unfortunately, the outcomes remain poor. Older children with ATRT tend to do better than younger children.

How common is rhabdoid tumors?

About 20 to 25 new cases of malignant rhabdoid tumors are diagnosed each year in the United States.

Are rhabdoid tumors genetic?

Inheritance. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to predispose the affected individual to rhabdoid tumors. The majority of cases of RTPS are caused by SMARCB1 gene mutations.