Rhabdomyosarcoma

Rhabdomyosarcoma is a malignant tumor arising from the striated muscle.

Etiology and epidemiology

Rhabdomyosarcoma is the most common malignant mesechymal tumor in children (but it is rare compared to other childhood malignancies).

Pathology

Histologically, there are 4 basic subunits:

• embryonic - includes sarcoma bothyroides;
• alveolar;
• pleomorphic;
• mixed.

thumb|right|300px|CT hlavy bez kontrastu – izodenzní masa bez intrakraniální progrese. Jedná se o postaurikulární vrozený alveolární rhabdomyosarkom.

Location

• head and neck are most often affected - 35%;
• 

  CT of the head without contrast - isodense mass without intracranial progression. It is a postauricular congenital alveolar rhabdomyosarcoma.
  torso and limbs - 35%;
• genitourinary area - 30%.

Metastasis

Tumors have a marked tendency to develop local recurrences. They metastasize early, both hematogenously and lymphogenically. Any authority can be affected.

Diagnosis

• Rtg,
• CT,
• MRI,
• US,
• scintigraphy skeleton.

Therapy

The combination of effective cytostatics has made it possible to dispense with radical, often mutilating surgical procedures.

• chemotherapy - initial neoadjuvant treatment after biopsy;
• surgery - after the tumor has shrunk, a definitive surgical procedure follows;
• radiotherapy - the residue of the tumor and the affected lymph nodes must be treated with radiotherapy.

Prognosis

It depends on the stage of the disease.

• Localized forms - healing is possible up to 100%.
• Metastatic spread - 40%.

Overall survival is reported to be 70%.

Links

Related articles

• Mesenchymal tumors
• Rhabdomyom