What are Rhabdoid Tumors?
Rhabdoid tumors are rare and aggressive forms of cancer that can occur in various parts of the body, most commonly in the kidneys (renal rhabdoid tumors) and the central nervous system (atypical teratoid/rhabdoid tumors or AT/RT). These tumors are known for their rapid growth and poor prognosis.
Who is Affected by Rhabdoid Tumors?
Rhabdoid tumors primarily affect infants and young children, although they can occasionally be found in older children and adults. The median age at diagnosis is typically around 15 months for renal rhabdoid tumors and 3 years for AT/RT.
What Are the Symptoms?
The symptoms of a rhabdoid tumor can vary depending on its location. Common symptoms include:
Abdominal pain or swelling
Vomiting
Headaches
Neurological deficits
Seizures
How is it Diagnosed?
Diagnosis of rhabdoid tumors typically involves a combination of imaging studies such as
MRI or
CT scans, followed by a biopsy to confirm the diagnosis. Genetic testing may also be performed to identify mutations in the
SMARCB1 or
SMARCA4 genes, which are commonly associated with these tumors.
Surgery: To remove as much of the tumor as possible.
Chemotherapy: Often used in combination with surgery to target remaining cancer cells.
Radiation therapy: May be employed, although its use can be limited due to the young age of most patients.
What is the Prognosis?
The prognosis for rhabdoid tumors is generally poor due to their aggressive nature and tendency to spread rapidly. Survival rates are often less than 30%, and the prognosis can be particularly dire for infants diagnosed with these tumors.
Ongoing Research and Future Directions
Research into rhabdoid tumors is ongoing, with scientists focusing on understanding the genetic and molecular mechanisms underlying these cancers. Targeted therapies and
immunotherapy are areas of active investigation, offering hope for more effective treatments in the future.
Support and Resources
Families affected by rhabdoid tumors can find support through various organizations and online forums. These resources offer valuable information and community support, helping families navigate the challenges of a cancer diagnosis.
