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Chapter: Paediatrics: Oncology

Paediatrics: Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood.



Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood. It accounts for 6% of all childhood malignancies (commonly aged <10yrs). The majority of cases are sporadic. Most are either embryo-nal or alveolar (more aggressive) subtypes. Botryoid (good prognosis) and spindle cell types are also recognized. A small number of cases are associ-ated with Li–Fraumeni syndrome.


Presenting features 

Mass, pain and obstruction of:

·  bladder;


·  pelvis;


·  nasopharynx;


·  parameningeal;


·  paratestis;


·  extremity;


·  orbit;


·  intrathoracic.


Lymph node involvement is common. Distant metastases are rare.


Diagnosis and staging


·  Imaging of primary site: CT, or MRI.


·  Biopsy for histological molecular and cytogenetic analysis. Alveolar RMS characterized by the presence of t(2;13) or t(1;13).


·  CT scan of chest.


·  Bone marrow aspirates and trephines.


·  Isotope bone scan.


·  Lumbar puncture (parameningeal primaries).




·  Chemotherapy (6–9 courses) with ifosfamide or cyclophosphamide, actinomycin, vincristine, anthracyclines.

·  Surgery is reserved for accessible sites (paratesticular, peripheral) after 3–6 courses of chemotherapy.

·  Radiotherapy after surgery for residual tumour and alveolar histology.



Ranges from <10% survival for bony metastatic disease to >90% for excised paratesticular tumours. Favourable features are:

·  Younger age at diagnosis.


·  Botryoid or embryonal histology.


·  Paratesticular or superficial head and neck sites.


·  Absence of nodal involvement or distant metastases.


Off treatment monitoring 

Imaging of primary tumour site by US or MRI and CXRs to screen for pulmonary metastatic recurrence.



·  Second-line chemotherapy.


·  Radiotherapy may be employed at sites not previously irradiated.


Outcome for relapse and recurrence is poor


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