Acute teratoid rhabdoid tumor
Disease Information
In-Depth
What are the symptoms of a rhabdoid tumor?
The symptoms depend on the location of the tumor and the age of the child. The following are the most common symptoms of a rhabdoid tumor; however each child may experience symptoms differently. Common symptoms may include:
- seizures - can be the only presenting sign for supratentorial lesions
- hydrocephalus - enlargement of the skull and pressure on the brain
- raised intracranial pressure - the tumor blocks the normal flow of cerebrospinal fluid and the tumor causes increased production of fluid, resulting in headaches, morning vomiting, lethargy, and disturbances in walking
- enlarged head size or fontanels (the soft "spot" that occurs before the bones in the head become solid) in infants
Due to the aggressiveness of these tumors, the duration of symptoms before coming to medical attention is relatively short.
The symptoms of a brain tumor may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
How are rhabdoid tumors classified?
Rhabdoid tumors are considered mesenchymal nonmeningothelial tumors within the WHO classification. These tumors are distinct from rhabdomyosarcomas. Immunohistochemical staining shows positivity for vimentin, cytokeratin, and epithelial membrane antigen. Neural markers are variably present. A characteristic cytogenetic abnormality has been identified, the deletion of 22q (22q11) which can aid in differentiating these tumors from other poorly differentiated malignant brain tumors.
