Rhabdoid tumor: Symptoms, causes, treatment, and outlook Health ConditionsHealth ConditionsAlzheimer's & DementiaAnxietyArthritisAsthma & AllergiesBreast CancerCancerCardiovascular HealthCOVID-19Dermatology & SkincareDiabetesEnvironment & SustainabilityExercise & FitnessEye HealthHeadache & MigraineHealth EquityHIV & AIDSHuman BiologyInflammatory Bowel DiseaseLeukemiaLGBTQIA+Men's HealthMental HealthMultiple Sclerosis (MS)NutritionParkinson's DiseasePsoriasisSexual HealthWomen's HealthDiscoverNewsLatest NewsOriginal SeriesMedical MythsHonest NutritionThrough My EyesNew Normal HealthPodcastsHow to understand chronic painWhat is behind vaccine hesitancy?The amazing story of hepatitis C, from discovery to cureNew directions in dementia researchCan psychedelics rewire a depressed, anxious brain?Why climate change matters for human healthToolsGeneral HealthDrugs A-ZHealth HubsHealth ToolsBMI Calculators and ChartsBlood Pressure Chart: Ranges and GuideBreast Cancer: Self-Examination GuideSleep CalculatorHealth ProductsAffordable Therapy OptionsBlood Pressure MonitorsDiabetic SuppliesFitness TrackersHome GymsGreen Cleaning ProductsHow to Shop for CBDQuizzesRA Myths vs FactsType 2 Diabetes: Managing Blood SugarAnkylosing Spondylitis Pain: Fact or FictionConnectAbout Medical News TodayWho We AreOur Editorial ProcessContent IntegrityConscious LanguageNewslettersSign UpFollow UsMedical News TodayHealth ConditionsDiscoverToolsConnectSubscribe What to know about rhabdoid tumorsMedically reviewed by Teresa Hagan Thomas PHD, BA, RN — By Jenna Fletcher on August 30, 2022Rhabdoid tumors (RT) are large cell tumors that form in the soft tissues, such as the kidneys, liver, peripheral nerves, and the central nervous system (CNS). It is a rare, aggressive cancer that usually develops in young children and infants. A 2018 article notes that although the outlook has improved for some individuals with rhabdoid tumors, the overall outlook remains negative. This article covers what rhabdoid tumors are and their symptoms, causes, and treatment options. What is it Share on PinterestMarvin 101, CC BY-SA 3.0, via Wikimedia CommonsA rhabdoid tumor is a rare, highly aggressive tumor that affects soft tissue anywhere in the body. A 2022 article states that they often develop in the:CNSkidneysliverneckthoraxpelvisback of the abdomen If this tumor develops in the brain or spinal cord, it is known as a subtype called an atypical teratoid rhabdoid tumor. It typically affects children, particularly those under the age of 3 years and, most commonly, those between the ages of 11 and 18 months. How common are they Rhabdoid tumors are rare. In the United States, there are fewer than 1,000 people with the disease. Some experts estimate that it affects fewer than 1 person for every 1 million people. Symptoms The National Institute of Health’s (NIH) Genetic and Rare Diseases Information Center notes that the age of onset for rhabdoid tumors can vary from before someone is born until they reach 11 years old. The exact symptoms will depend on where the tumor initially develops. They typically start when the growing tumor puts pressure on surrounding tissue. Symptoms may include:abdominal painanemiacerebral palsycranial nerve paralysisheadacheparalysis in the arm, leg, and in some cases, the face on one side of the bodyhigh blood pressureinternal bleedingirritabilityswelling of the lymph nodesnausea and vomitinga reduced ability to control the movement of the eyereduced appetiterespiratory issueslesions on the skin that measure over 5 millimetersweight losslow platelet count Causes Researchers and experts are still unsure of the exact cause of rhabdoid tumors. However, according to the National Cancer Institute (NCI), nearly all these tumors are missing the SMARCB1 gene. The SMARCB1 gene is responsible for suppressing the formation of tumors. Changes in its makeup may make tumor formation more likely. In rare cases, it may develop due to changes to the SMARCA4 gene. Is it hereditary The NCI states that malignant RTs do not run in families. Treatment Due to its rarity, no standard treatment exists for rhabdoid tumors. However, doctors may try one or more different strategies to treat the tumor. These may include:surgery to remove as much of the tumor as possiblehigh dose chemotherapy to help kill a fast-growing tumorradiation therapy to help shrink and kill the tumor A doctor may also recommend a stem cell transplant or combining surgery with other treatment options. They may also recommend combining high dose chemotherapy with radiation therapy and stem cell transplant for optimal outcomes. A parent or caregiver should discuss treatment options with a doctor. Each treatment comes with benefits and side effects to consider. Who will be on the treatment team A child’s treatment team may vary according to their physical location and where the tumor developed in the body. The team may consist of:A primary care provider: These medical professionals can provide referrals to specialists and help coordinate overall medical care. A pediatrician: A specialist in providing healthcare for children. An oncologist: Specializes in diagnosing and treating cancer and malignant tumors. Alongside the above medical professionals, the medical team may also include radiologists and surgeons. Outlook and life expectancy The outlook for a rhabdoid tumor depends on:the location of the tumorwhether it has spreadhow much of the tumor remains following surgery Typically, the outlook for this type of tumor is negative. The NCI says that children with atypical teratoid rhabdoid tumor, a CNS tumor, have a 5-year survival rate of 32.2%. Additionally, the NCI says that most children with extra-cranial malignant rhabdoid tumors do not survive for more than a few years. However, those who receive a diagnosis after they reach 2 years old have a more positive diagnosis. It is important to remember that due to the rarity of the disease, these survival rates may not be accurate. People should speak with the child’s care team to determine the chances of survival. Diagnosis A doctor will likely first review the child’s symptoms. If they can find no other causes, they will likely order additional testing. To diagnose rhabdoid tumors, a doctor will typically need imaging tests and a biopsy. Imaging tests may include the use of one or more of the following:MRIultrasoundCT scan A biopsy involves taking a small sample of the tumor. The doctor will then send it to a lab to test for the presence of the SMARCB1 gene. Nearly every case of rhabdoid tumor has a mutation in this gene. Financial help People may find financial assistance for treating cancers at the following organizations:CancerCare and their co-payment assistance foundationAmerican Cancer Society (ACS)HealthWell Foundation The ACS notes that childhood cancer is rare, meaning treatment can require travel. Some treatment centers are near a Ronald McDonald House, which provides low cost or free housing. The ACS Hope Lodge program also offers families a free place to stay during the treatment. To help with prescription medications, a person may wish to contact NeedyMeds or the PAN Foundation. A person should also contact the billing departments at the hospitals. They may be able to work out payment plans, help find support for costs, and set someone up with a case manager who may be able to help find cost savings. Coping and support Children and their families may benefit from working with a mental health professional, such as a psychologist. A psychologist can help a family:learn to cope with stressprocess the emotions relating to cancerhelp reduce distress during medical procedureshelp families cope with grief and losssupport survivorshelp manage sleep problems The NCI and the ACS provide resources and information to help people cope with cancer. Summary Rhabdoid tumors are highly aggressive, rare soft tissue tumors that typically develop in young children and infants. It usually develops due to changes to the SMARCB1 gene. Treatments for rhabdoid tumors can include surgery, chemotherapy, and radiation. A doctor may also recommend combining these therapies. The outlook for RT is typically negative, but the survival rates may not be accurate due to the rarity of the disease. Parents and caregivers should speak with a doctor to determine the best course of treatment and likelihood of success. Last medically reviewed on August 30, 2022Cancer / OncologyMedically reviewed by Teresa Hagan Thomas PHD, BA, RN — By Jenna Fletcher on August 30, 2022 Latest newsWhat sets 'SuperAgers' apart? Their unusually large neuronsOmega-3 may provide a brain boost for people in midlifeSeasonal affective disorder (SAD): How to beat it this fall and winterCDC: Monkeypox in the US 'unlikely to be eliminated in the near future'Why are more women prone to Alzheimer's? New clues arise Related CoverageWhat to know about childhood kidney cancer Childhood kidney cancer refers to when cancer develops in the kidneys. Learn about diagnosis, treatment, prognosis, and more.READ MOREWhat to know about childhood melanomaMedically reviewed by Mia Armstrong, MD Childhood melanoma starts in the cells responsible for creating the skin’s color or tone. Read about symptoms, diagnosis, treatment, and outlook.READ MOREWhat to know about Wilm's tumorMedically reviewed by Christina Chun, MPH Wilms' tumor or nephroblastoma is a kidney cancer that generally affects children, and very rarely adults. The tumor was named after Dr. Max Wilms…READ MOREGenetically engineered, cancer-killing herpes virus may be able to fight tumorsREAD MOREWhat are the CAP cancer protocols? The College of American Pathologists (CAP) has a set of protocols that help doctors report on cancer cases. It includes information on different…READ MORE