Risks for rhabdomyosarcoma

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Certain behaviours, substances or conditions can affect your risk, or chance, of developing cancer. Some things increase your risk and some things decrease it. Most cancers are the result of many risks. But sometimes cancer develops in people who don't have any risks.

Rhabdomyosarcoma (RMS) is most common in children younger than 10 years old. It can also occur in teens and adults. It is slightly more common in boys than in girls.

People who have certain genetic conditions have a higher than average risk for RMS. A genetic condition is a disease caused by a change (mutation) in one or more genes.

The genetic conditions listed below can increase your child's risk for RMS. Talk to your child's doctor about the risk. Your child may need to visit the doctor more often to check for RMS. The doctor will recommend the tests your child should have and how often they should have them.

Li-Fraumeni syndrome

Neurofibromatosis type 1

Beckwith-Wiedemann syndrome

Costello syndrome

Noonan syndrome

Li-Fraumeni syndrome

Li-Fraumeni syndrome is an inherited condition that is associated with an increased risk of developing certain types of cancers, including breast cancer, brain tumours, acute leukemia, bone and soft tissue sarcomas (such as RMS), and adrenal cortical carcinomas.

People with Li-Fraumeni syndrome tend to develop several different types of cancer before the age of 45.

Neurofibromatosis type 1

Neurofibromatosis type 1 is an inherited condition that affects the nervous system. It affects the development and growth of neurons (nerve cells), causes tumours (neurofibromas) to grow on nerves and may produce other abnormalities in muscles, bones and skin. Sometimes cancerous tumours called malignant peripheral nerve sheath tumours may grow along the nerves.

Neurofibromatosis type 1 increases the risk for neuroendocrine tumours, soft tissue sarcoma, such as RMS, brain tumours, leukemia and neuroblastoma.

Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome is an inherited condition that affects how different parts of the body grow. Signs include large body size, large tongue, large organs, a defect in the abdominal wall and low blood sugar in newborns. Beckwith-Wiedemann syndrome is associated with an increased risk of developing cancer, including RMS, Wilms tumour, neuroblastoma and adrenal gland tumours.

Costello syndrome

Costello syndrome is a condition that affects many parts of the body. Children with Costello syndrome often have delayed physical development, intellectual disability, unique facial features including a large mouth with full lips, loose folds of extra skin especially on the hands and feet and unusually flexible joints. They also have a higher risk of developing certain types of cancers, including RMS and neuroblastoma.

Noonan syndrome

Noonan syndrome is an inherited condition that affects many parts of the body. Children with Noonan syndrome have a unique facial appearance including widely spaced eyes and a deep groove in the area between the nose and the mouth, short height, heart defects and bleeding problems. They have a higher risk for RMS.

Expert review and references

  • Canadian Cancer Society | Société canadienne du cancer
  • American Cancer Society . Risk Factors for Rhabdomyosarcoma . 2018 : https://www.cancer.org/.
  • American Society of Clinical Oncology (ASCO). Cancer.net: Rhabdomyosarcoma - Childhood: Risk Factors. 2018: https://www.cancer.net/.
  • Burningham Z, Hasibe M, Spector L & Schiffman JD . The epidemiology of sarcoma. Clinical Sarcoma Research. BioMed Central; 2012.
  • Cripe TP . Medscape Reference: Pediatric Rhabdomyosarcoma. WebMD LLC; 2018: https://www.medscape.com/.
  • Grufferman S, Ruymann F, Ognjanovic S, Erhardt EB, & Maurer HM . Prenatal X-ray exposure and rhabdomyosarcoma in children: a report from the children's oncology group. Cancer Epidemiology, Biomarkers & Prevention. American Association for Cancer Research; 2009.
  • Grufferman S, Schwartz AG, Ruymann FB & Maurer HM . Parents' use of cocaine and marijuana and increased risk of rhabdomyosarcoma in their children . Cancer Causes & Control . Springer ; 1993 .
  • Macmillan Cancer Support. Rhabdomyosarcoma. 2019: https://www.macmillan.org.uk/.
  • PDQ Pediatric Treatment Editorial Board. Childhood Rhabdomyosarcoma Treatment (PDQ®) – Health Professional Version. Bethesda, MD: National Cancer Institute; 2021: https://www.cancer.gov/.
  • PDQ Pediatric Treatment Editorial Board. Childhood Rhabdomyosarcoma Treatment (PDQ®) – Patient Version. Bethesda, MD: National Cancer Institute; 2020: https://www.cancer.gov/.
  • National Institutes of Health. MedlinePlus Genetics: Costello Syndrome. Bethesda, MD: US Department of Health & Human Services; 2020: https://medlineplus.gov/genetics/.
  • National Institutes of Health. MedlinePlus Genetics: Noonan Syndrome. Bethesda, MD: US Department of Health & Human Services; 2018: https://medlineplus.gov/genetics/.
  • Ognjanovic S, Carozza SE, Chow EJ, et al . Birth characteristics and the risk of childhood rhabdomyosarcoma based on histological subtype. British Journal of Cancer (BJC). Nature Publishing Group; 2010.
  • Roman E, Lightfoot T, Picton S, Kinsey S. Childhood cancers. Thun MJ, Linet MS, Cerhan JR, Haiman CA Schottenfeld D, eds.. Schottenfeld and Fraumeni Cancer Epidemiology and Prevention. 4th ed. New York, NY: Oxford University Press; 2018: Kindle version, 59 https://read.amazon.ca/?asin=B0777JYQQC&language=en-CA.
  • Singer S, Tap WD, Kirsch DG, Crago AM. Soft tissue sarcoma. DeVita VT Jr., Lawrence TS, Rosenberg SA, eds. DeVita, Hellman, and Rosenberg's Cancer: Principles & Practice of Oncology. 11th ed. Philadelphia, PA: Wolters Kluwer; 2019: 88:1400–1450.
  • Wexler LH, Skapek SX, Helman LJ. Rhandomyosarcoma. Pizzo PA, Poplack DG, eds.. Principles and Practice of Pediatric Oncology. 7th ed. Philadelphia, PA: Wolters Kluwer; 2016: 31:798–826.