Rhabdoid tumor: Signs, origins, therapies, and prospects
Rare and Aggressive: Understanding Rhabdoid Tumors in Children
Rhabdoid tumors are a rare and highly aggressive form of cancer that primarily affect infants and young children, particularly those under the age of 3. These tumors most commonly develop in the kidneys, brain, or soft tissues.
Locations and Symptoms
The symptoms of rhabdoid tumors vary depending on their location. In the kidneys, they may cause abdominal pain, swelling, or an abdominal mass. Brain tumors, particularly atypical teratoid rhabdoid tumors (ATRT), can lead to neurological symptoms such as headache, paralysis, cranial nerve paralysis, and high blood pressure. Systemic symptoms like weight loss, lethargy, and low platelet count may also be present.
Causes and Genetics
Rhabdoid tumors are driven by genetic mutations, often involving the loss or mutation of the SMARCB1 gene, which is responsible for suppressing the formation of tumors. These tumors arise de novo rather than from pre-existing conditions.
Diagnosis and Treatment
To diagnose rhabdoid tumors, a doctor will typically need imaging tests and a biopsy, which involves taking a small sample of the tumor and sending it to a lab to test for the presence of the SMARCB1 gene.
Treatment options for rhabdoid tumors include surgery to remove the tumor, chemotherapy, and radiotherapy. However, due to the toxicity of radiotherapy, it is often limited in infants. Newer therapeutic approaches, such as targeted therapies and immunotherapy, are under investigation to improve efficacy while minimizing long-term side effects.
Prognosis and Support
Survival rates for rhabdoid tumors are low, with a 5-year survival rate around 20-25%. The outlook for a rhabdoid tumor depends on the location of the tumor, whether it has spread, and how much of the tumor remains following surgery.
Children and their families may benefit from working with a mental health professional, such as a psychologist, to cope with stress, process emotions, reduce distress during medical procedures, cope with grief and loss, support survivors, and manage sleep problems.
Resources and Information
Due to its rarity, no standard treatment exists for rhabdoid tumors. However, people may find financial assistance for treatment at organisations like CancerCare, the HealthWell Foundation, Ronald McDonald House, NeedyMeds, PAN Foundation, and by contacting the billing departments at hospitals.
For those requiring travel for treatment, some treatment centers are near a Ronald McDonald House, which provides low-cost or free housing. The National Cancer Institute (NCI) and the American Cancer Society (ACS) also provide resources and information to help people cope with cancer.
[1] National Cancer Institute. (2021). Rhabdoid Tumor. https://www.cancer.gov/types/rhabdoid-tumor [2] American Cancer Society. (2021). Rhabdoid Tumor. https://www.cancer.org/cancer/rhabdoid-tumor.html [5] Children's Brain Tumor Project. (2021). Atypical Teratoid Rhabdoid Tumor (ATRT). https://www.cbtp.org/atypical-teratoid-rhabdoid-tumor-atrt/
- In the field of oncology, rhabdoid tumors, alongside other cancers, are areas where medical science continually strives to make advances due to their aggressiveness and rarity in children.
- The symptoms of different locations of rhabdoid tumors, such as headaches and paralysis for brain tumors, mimic various medical conditions, making accurate diagnosis crucial for effective cancer care and health-and-wellness.
- Science-backed support services, including counseling and emotional aid, are vital for children suffering from rhabdoid tumors and their families, helping them cope with stress, grief, and other challenges associated with cancer treatments and health issues.
