Pleomorphic Rhabdomyosarcoma
Synonyms: Adult-type rhabdomyosarcoma, pleomorphic type
Adult variant - generally poor prognosis
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
High
Synonyms
- Adult-type rhabdomyosarcoma
- pleomorphic type
Category
Soft tissue
Behaviour
Malignant
Grade
High
Gender
M = F
Tissue of Origin
Skeletal muscle
Epidemiology
- Rare adult variant of rhabdomyosarcoma
- Peak incidence in older adults
- Lower extremity predominance
Clinical Features
- Large, rapidly growing mass
- Often presents with advanced disease
Location
- Lower extremity most common
- Trunk
- Soft tissues
Imaging
- MRI: large heterogeneous mass
- CT chest - metastatic staging
Pathology
- Pleomorphic myogenic cells
- High mitotic rate, extensive necrosis
- Markedly atypical
Genetics
- Variable molecular features
- No specific fusion requirement
Treatment
- Wide surgical excision
- Chemotherapy and/or radiotherapy
Prognosis
- Poor - High-grade malignancy
- Often metastatic at presentation
Key Points
- Adult variant - generally poor prognosis
- Aggressive behaviour requiring multimodal therapy
Workup - Blood Tests
FBC, U&E, LFTs, LDH - baseline
Workup - Local Imaging
- MRI primary site - local staging
- CT chest/abdomen/pelvis - metastatic staging
Workup - Biopsy
Core needle biopsy - confirm diagnosis
Workup - Staging
CT chest/abdomen/pelvis - metastases
Follow-up Summary
- 1
Year 1
2-monthly clinical examination, CXR, plain films of primary bony site; soft tissue tumours - baseline end-of-treatment MRI/CT primary site, thereafter at clinician's discretion; radiotherapy as definitive local treatment - baseline end-of-treatment MRI/CT, then at 6 and 12 months; end of Year 1 - gonadal function and renal function
- 2
Years 2–3
3-monthly clinical examination, CXR, plain films of bony primary site; MRI of soft tissue primary site at clinician's discretion; radiotherapy patients - MRI/CT at 18 and 24 months; MUGA/ECHO 2 years post-diagnosis; annual renal function
- 3
Year 4
6-monthly clinical examination, CXR, plain films of primary site; MRI of soft tissue primary at clinician's discretion; MUGA/ECHO 4 years post-diagnosis; annual renal function
- 4
Year 5
6-monthly clinical examination, CXR, plain films of primary site; annual renal function
- 5
Years 6–10
Annual clinical examination, CXR, plain films of primary site; MUGA/ECHO 6 years post-diagnosis; annual renal function
- 6
Discharge at 10 years after surgery
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