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Non-Rhabdomyosarcoma (Non-RMS)

 

 

Investigations

 

 

If a soft tissue sarcoma is suspected, the most important initial investigation is always a complete history and physical:

Complete history and physical:

History

The history should include:

Specific information regarding primary tumor:

  • Tumor mass:
    • When was the mass first noticed?
    • How quickly is the mass changing in size (e.g. rapidly or slowly)?
    • Are there any overlying skin changes?
    • Is there associated pain/tenderness/redness or warmth?
    • If pain is present, does the pain radiate anywhere?
  • Is there any functional abnormality of the affected limb?
  • Is there any enlargement of regional lymph nodes?
  • Is there any distal lymphedema/swelling of limb?

 

General health:

Is there any previous history of:

  • Other illnesses such as TB?
  • Previous radiation therapy?

 

  • Are there any systemic symptoms?
    • Appetite change, weight loss, appetite, fevers, night sweats?
  • Review of symptoms:
    • General questions about lung, cardiac, GI and GU symptoms
  • Is there any pain elsewhere?
  • Are there any pulmonary symptoms (such as shortness of breath, cough or chest pain)

 

Family history:

  • Is there any relevant family history (previous history of cancers in relatives)

 

Physical examination

Specific to region of tumor:

  • Size of tumor in cm
  • Mobile or attached (fixed) to deep tissues
  • Any breakthrough of skin (fungating tumor)
  • Are any regional lymph nodes enlarged?
  • Any distal edema involving limb where tumor is located?
  • Any evidence of a deep vein thrombosis?

General examination:

  • Vital observations
  • Weight, height, blood pressure, pulse and temperature
  • Examination of chest (respiratory and cardiovascular systems) and abdomen

 

Other Investigations

Blood work:

General including CBC, liver function tests and electrolytes, creatinine and BUN.

Blood coagulation studies prior to biopsy

 

Radiology

To assess extent of primary tumor (local disease)

  • MR scan of affected limb

To exclude distant metastatic disease:

  • CXR
  • CT of chest to exclude pulmonary metastases
  • For liposarcoma; CT of chest, abdomen and pelvis to exclude metastatic disease in these areas
  • PET- CT scan: Very sensitive for tumors such as extra-osseous Ewing sarcoma

 

Biopsy

  • Needs to be carefully planned by surgeon and treatment team

 

N.B.

PRIOR TO THERAPY in any young person there should be a discussion about any potential side effects of treatment on fertility.

 

 

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