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Rhabdomyosarcoma, Pediatric

Amit J. Sabnis, MD and Steven G. DuBois, MD, MS Reviewed 10/2018
 


BASICS

DESCRIPTION

A soft tissue cancer with features of skeletal muscle differentiation. Prognostic classification of rhabdomyosarcoma (RMS) currently depends on: 
  • Anatomic site of disease (stage)

  • Exten...

DIAGNOSIS

HISTORY

  • Often presents as a firm, painless mass

  • Additional symptoms relate to location of primary mass:

    • Orbital (10%): unilateral proptosis, ophthalmoplegia, visual changes

    • Head and neck—paramen...

TREATMENT

MEDICATION

  • The backbone of RMS chemotherapy in North America is cycles of vincristine and actinomycin D, with or without cyclophosphamide (a.k.a. “VA” or “VAC”).

    • Can often be given as outpatie...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Patients seen regularly by pediatric oncology

  • After completion of therapy, follow-up with pediatric oncology is every 3 months for the 1st year and then gradually ...

ADDITIONAL READING

  • Hayes-Jordan A, Andrassy R. Rhabdomyosarcoma in children. Curr Opin Ped.  2009; 21(3):373–378. [View Abstract on OvidInsights]

  • Malempati S, Hawkins DS. Rhabdomyosarcoma: review ...

CODES

ICD9

  • 71.9 Malignant neoplasm of connective and other soft tissue, site unspecified

  • 171.0 Malignant neoplasm of connective and other soft tissue of head, face, and neck

  • 171.6 Malignant neoplasm of c...

FAQ

  • Q: Are other children in the family at risk for developing RMS?

  • A: Familial risk for developing RMS is rare, outside of syndromes addressed in “Genetics.”

  • Q: Will this child lose his or her hair?

  • A: ...

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