Rhabdomyosarcoma (RMS) is a rare, malignant tumor that arises from immature skeletal muscle cells, most commonly affecting children and adolescents. It can occur in various locations, including the head and neck, genitourinary tract, and extremities. RMS is classified into subtypes—embryonal, alveolar, pleomorphic, and spindle cell/sclerosing—based on histological features, with embryonal being the most common in children. Clinical presentation depends on the tumor’s location and may include a painless mass, swelling, obstruction, or functional impairment of nearby organs. Diagnosis relies on imaging studies (MRI, CT), biopsy, and histopathological examination, often supplemented by immunohistochemistry to confirm muscle lineage. Treatment is multimodal, typically combining surgery, chemotherapy, and sometimes radiotherapy, with prognosis influenced by tumor size, location, histologic subtype, and metastasis at diagnosis.
Outline
- Introduction
- Epidemiology
- Associated Syndromes
- Clinical Presentation
- Pathological Classification
- Diagnosis and DDx
- Clinical staging and prognosis
- Treatment
- Soft tissue sarcoma in pediatric divided to:
- Rhabdomyosarcoma (40-50%)
- Non-rhabdomyosarcoma (synovial sarcoma, fibrosarcoma, PNET)
- Soft tissue sarcoma common in adult
- Rhabdomyosarcoma: malignant mesenchymal tumor of striated skeletal muscle
Epidemiology
- 3-4% of all child cancer
- 4 cases per million in Canada
- Conmonest STT in patient <20 Y.O
- M:F(I,5:I)
- Two third of cases diagnosed in first 5 years
- Specific risk factor---unknown and most cases are sporadic
- 20% have lung metastases at time of presentation
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