Definition

Sarcoma are a rare and heterogenous group of malignant tumours of mesenchymal origin
Management strategies are similar for extremities or superficial trunk (chest wall, flank, abdominal wall, paraspinal musculature) therefore are grouped

Treatment options

Resection
- Wide surgical excision of the primary tumour should take place
- The status of the surgical margins is the most important variable a/w recurrence.
- Generally should aim for 1cm margin OR a fascial barrier - often not achieved.
- Biopsy tract needs to be removed at same time.
Limb sparing or not
- Is the patient fit for limb sparing surgery
- Is it possible
  - Tumour needs to be removed, with a good oncological operation, with acceptable risk of complications and functional outcome is better or equivalent to an amputation.
Management of nodes
- Regional nodal dissection is only recommended if there is clinical or radiological disease.
  - Rare for sarcoma to go to nodes
Assessment of margins
- AJCC margin assessment
  - Ro - histologically negative margin
  - R1 - histologically positive margin (tumour on ink)
  - R2 - macroscopically positive margin/known residual disease
- Modified Enneking classification of margin
  - Curative margin - normal cuff of tissue >5cm.
  - Wide margin B - cuff of normal tissue >1cm < 5cm - adequate.
  - Wide margin A - cuff of normal tissues < 1cm (inadequate)
  - Marginal margin - reactive pseudocapsule present at the margin
  - Intralesional margin - tumour entered
Margin taken dictates risk of local recurrence.
- Curative - risk of LR 10%
- Wide Margin B - risk of LR 11%
- Wide Margin A - risk of LR 18%
- Marginal margin - risk of LR 40%
- Intralesion margin - risk of LR - 80%.

Sentinal lymph node biopsy

Most sarcomas don’t go to nodes but for subtypes which do - you should consider performing one.
These include (remember as CLEAR)
- Clear cell sarcoma
- Leiomyosarcoma
- Epithelioid sarcoma
- Angiosarcoma
- Rhabdomyosarcoma

Commonly used for limb sarcomas
- Increases limb-sparing surgery
- Decreases local recurrence
Almost everyone should be given post-operative radiotherapy.
Where RT can be omitted
- <5cm tumour which is low grade, superficial to fascia, where a wide margin (>1cm) can be obtained
Adjuvant vs neoadjuvant RT
- Neoadjuvant
  - Advantages
    - Lower dose required
    - Reduces tumour burden allowing more conservative surgery
    - Good for tumour subtypes which are very sensitive to radiotherapy i.e. myxoid liposarcoma.
    - If tumour is adjacent to critical structures i.e. brachial plexus (as post resection, these would be within the radiation field)
  - Disadvantages
    - Increased wound complications post-operatively.
- Adjuvant
  - Advantages
    - Allows more accurate histological examination of the tumour
    - Reduced wound complications.
- In general - neoadjuvant RT is preferred
  - For patients with predicted poor healing - may prefer to give RT adjuvantly
Definitive RT
- Can be considered for patients who are medically inoperable - due to co-morbidities or amputation is not accepted

Controversial in adults - depends on histological subtype
Is routine for children with Rhabdomyosarcoma and Ewing sarcoma - are both chemosensitive.
- Doxorubicin and Ifosfamide
  - Reduces local recurrence - does not improve survival.
- Angiosarcoma
  - Sensitive to Taxanes - Paclitaxel is often given neoadjuvantly to try and shrink the tumour

Treat with aggressive re-resection, RF and chemotherapy
2/3 of patients who undergo resection for local recurrence will have long-term survival.