Rhabdomyosarcoma



Rhabdomyosarcoma


Courtland Polley





  • Rhabdomyosarcomas (RMSs) are primary malignant soft tissue tumors that arise from embryonic mesenchyme with the potential to differentiate into skeletal muscle.1


  • They are rare overall but account for over half of all soft tissue sarcomas in children.


  • This is the third most common solid tumor in infants and children, behind neuroblastoma and Wilms tumor.1



    • 250 to 300 new cases per year


    • 75% of cases are diagnosed in children younger than 6 years


EPIDEMIOLOGY AND ETIOLOGY



  • RMSs can occur in any anatomical location in the body except for bone, even in places where there is no skeletal muscle (eg, the bladder, biliary tree) (Figure 58.1).


  • Most cases of RMS appear to be sporadic, but they have been associated with familial syndromes such as neurofibromatosis, Li-Fraumeni, Beckwith-Wiedemann, and Costello syndromes).2


CLINICAL PRESENTATION



  • The presenting signs and symptoms are variable and influenced by the site of origin, age of the patient, and presence or absence of distant metastases.


  • In general, the primary lesion has the appearance of a nontender mass, occasionally with overlying skin erythema3 (Figures 58.2 and 58.3).


May 5, 2019 | Posted by in PEDIATRICS | Comments Off on Rhabdomyosarcoma
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