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Soft-tissue tumours (STT) are a heterogeneous group of benign and malignant diseases accounting for <4% of all tumours in adults and <8% of all tumours in children. Soft-tissue sarcomas rare malignant tumours derived from mesenchymal cells at all body sites. These rare tumours comprise approximately 1% of all newly diagnosed cancers. The incidence of soft tissue tumours is approximately 2000 cases per annum in the UK.
Patients may present with a painful or painless swelling that is growing insidiously. Almost 50% arise in the lower limbs (most commonly the adductor compartment of the thigh) and the median age for presentation is 65 years. Concern at to the biological activity of a lesion should arise if the size is >5cm, it is painful, deep to fascia, increasing in size, or recurrence of a previously excised lesion. Each of these five characteristics corresponds to an approximately 20% risk of malignancy. Any lesion presenting with features that are suggestive of STT require referral to specialist centres, where appropriate evaluation and staging will take place.

INDICATIONS:
Surgical resection of soft tissue sarcomas remains the mainstay of treatment and has been shown to improve overall and disease free survival when compared to oncological management alone. The mode of surgical resection is dependant on the extent of the tumour and the likelihood in achieving clear margins at surgical resection. Broadly speaking, surgery comprises either limb salvage, where the tumour is removed en masse without removal of the affected limb, or limb sacrifice whereby the tumour is removed with the affected limb. The decision on limb salvage versus limb sacrifice is made following careful assessment of the structures involved by the tumour, particularly the involvement of critical nerves and vessels, and the presence of metastatic disease at diagnosis. Also broadly speaking, limb sacrifice would not be offered to a patient with metastatic disease (Grier et al, Sarcoma, 2010).
The use of adjuvant or neo-adjuvant therapies (predominantly radiotherapy) is assessed on a case by case basis and decisions are made through a disease specific multi-disciplinary team. Certain histological subsets, for example myxoid liposarcomas, are exquisitely sensitive to radiotherapy and this is often given prior to surgical resection. Radiotherapy should be considered for all high grade lesions and whether this is given before surgery or after surgery is at the discretion of the MDT and based on the pre operative extent of the tumour and likelihood of achieving clear surgical margins at surgery.
SYMPTOMS & ASSESSMENT:
Patients may present with a painful or painless swelling that is growing insidiously. Almost 50% arise in the lower limbs (most commonly the adductor compartment of the thigh) and the median age for presentation is 65 years. There are, however, age-related variations; embryonal rhabdomyosarcomas occur exclusively in children, synovial sarcomas in young adults and liposarcomas in older people. Concern at to the biological activity of a lesion should arise if the size is >5cm, it is painful, deep to fascia, increasing in size, or recurrence of a previously excised lesion. Each of these five characteristics corresponds to an approximately 20% risk of malignancy. Any lesion presenting with features that are suggestive of STT require referral to specialist centres, where appropriate evaluation and staging will take place. A clinical assessment and examination are mandated, with attention required to regional lymphadenopathy, distal neurovascular deficit and localised skin changes at the tumour site.
INVESTIGATION:
Plain radiographs of soft-tissue lesions are helpful to exclude bone lesions with soft-tissue extension and to assess bone invasion from extra-osseous tumours with a risk of fracture. Radiographs should be obtained as the location, presence of any periosteal reaction, erosion (e.g. glomus tumour, PVNS) soft-tissue mineralisation (e.g. synovial sarcoma) is useful for characterisation.[46] Soft-tissue tumours are more difficult to diagnose specifically from imaging, however there may be characteristics which can help to determine aggressiveness or identifiable tissues such as fat, calcification or haemorrhage. Matrix calcification or ossification may be found in synovial sarcomas and in the rarer mesenchymal chondrosarcomas or soft-tissue osteosarcomas.
MRI sequencing of the affected limb is valuable to define extent of the lesion and local invasion of critical structures and to characterise the lesion prior to biopsy. If surgery is planned this will define the margins to neurovascular structures and the involved musculature, joints or tendons. There is extensive overlap of the soft-tissue sarcoma appearances with MRI; the majority have a low signal T1 and heterogeneous high signal T2 appearances. Exceptions to this may include high T1 signals in some liposarcomas and low T2 signal in desmoid fibromatosis and PVNS. The characteristics of particular tumours may be emphasised, such as a serpiginous pattern in vascular tumours, dystrophic calcification in synovial sarcomas or blooming artefact caused by the haemosiderin content of diffuse-type PVNS. MRI should be performed before biopsy as the tissue characteristics may alter. Contrast is reserved for targeting the biologically active areas of tumours for biopsy, although it risks nephrogenic systemic fibrosis in patients with chronic renal impairment.
Systemic staging will requires chest CT or PET CT to identify other biologically active lesions. Bone scintigraphy is not normally required. Systemic staging for soft-tissue sarcomas principally involves chest radiographs and chest CT for pulmonary metastases. In pelvic or retroperitoneal primaries, CT of the abdomen and pelvis would be included; PET CT is useful to identify other biologically active lesions in neurofibromatosis type 1. WBMRI is reliable to detect extra-pulmonary metastases in myxoid liposarcomas which rarely metastasise to the chest.
OPERATIVE ALTERNATIVES:
The options of planned marginal resection following radiotherapy versus en bloc resection with excision of the distal femur and reconstruction with a distal femoral endoprosthetic replacement were discussed. The latter option is felt not to confer a survival advantage in terms of local recurrence or the development of metastatic disease and carries a high risk of periprothetic infection in the presence of radiotherapy.
NON-OPERATIVE ALTERNATIVES:
In the presence of significant comorbidities, the patient may be offered radiotherapy alone as local control of the tumour. There is a disease free survival advantage conferred by surgical resection in combination with radiotherapy.
CONTRAINDICATIONS: Sufficient comorbidities to preclude surgical intervention.

The procedure should be performed by a surgeon designated by the local sarcoma multidisciplinary team (MDT). All cases are discussed in a disease specific MDT with careful scrutiny of the radiology, histology and discussion of the appropriate surgical and oncological management.
This patient had received neo-adjuvant radiotherapy
At the time of surgery, the procedure is performed in a laminar flow theatre.
Pre operative antibiotics, as designated by the local hospital protocol, are given at induction of anaesthesia.
The use of a tourniquet is at the discretion of the surgeon but is often not used as this allows visualisation of the vessels which often require dissection free from the tumour.
The patient is positioned supine with a sandbag behind the buttock to maintain the limb in a neutral position with the patella pointing directly upwards. The limb can therefore be manipulated during surgery to allow access to the entire quadriceps compartment.
The anaesthetic technique is at the discretion of the anaesthetist but should include regional or spinal anaesthesia, often with a combined epidural, for post operative analgesia.
The instrument sets should include an orthopaedic oncology set, including vascular instruments, and a vascular set, including non crushing clamps. Appropriate sized suture material should be available before commencing the case in the event of vascular injury requiring repair.

Antibiotics are given in line with the local hospital protocol. In this case, a further 24 hours of intravenous antibiotics wee given.
The drains are removed when less than 100ml total drainage in a 24 hour period.
The knee is immobilised in a straight position until the wound has healed at which point gentle knee flexion can commence, usually after a period of 2 weeks.
Physiotherapy is imperative given the extent of the quadriceps dissection.
The wound is reviewed at 5 days post operation and if no concerns, the vacuum dressing is removed and a barrier dressing applied.
The skin clips are removed no sooner than 2 weeks after operation. In light of the pre operative radiotherapy, the clips are often left in situ for longer than would normally be advised due to the risk of wound dehiscence.
The patient is discharged home when there are no longer concerns about the wound but is reviewed regularly due to the high incidence of late wound complications on the background of neo-adjuvant radiotherapy.
The tumour os processed by a specialist musculoskeletal pathologist. The response to neo-adjuvant radiotherapy is quantified as the percentage of viable cells seen in the resection specimen. The margins achieved at resection are carefully assessed and quantified in millimetres from viable tumour.
Following successful removal of the tumour, the patient embarks on standard sarcoma surveillance which in the UK comprises clinical assessment and chest radiography every 3 months for the first 2 years, every 6 months until 5 years post operation, and annually thereafter until 10 years.

Success following treatment for extremity soft tissue sarcoma is defined in terms of overall and disease free survival. This is quantified by the risk of local recurrence of the tumour or the development of metastases. The risk of disease recurrence is affected by the pathological grade of the tumour, the size of the tumour, the depth, location, histologcal subtype and the margin acheived at surgical resection (Collin et al, J Clin Oncol, 1987; Eiber et al., Ann Surg, 2003). Wide excision with the addition of neo-adjuvant or adjuvant radiotherapy form the mainstay of treatment. Radiotherapy, either given prior to resection or after resection, has been shown to reduce the development of local recurrence but does not improve overall survival (Zagars et al., Cancer, 2003). Pre operative radiotherapy is associated with a slight improvement in overall survival but an increased incidence of wound complications (O’Sullivan et al, Lancet, 2003). The addition of neo adjuvant radiotherapy can allow a closer margin of resection without a detrimental effect on the risk of local recurrence or overall survival when compared to such a margin in the non radiotherapy setting. This planned positive margin allows resection of large tumours in close association to vital structures without detriment to function through sacrifice of these structures, or increase in the risk of local recurrence (Gerrand et al, JBJS-B, 2001).
Overall survival for extremity sarcomas varies widely but averages between 60 and 75%. This is dependant on a number of variables. The risk of local recurrence is dependant on the margin achieved at surgical resection, the grade of the tumour and the histological variant of the tumour (eg soft tissue Ewings sarcoma, synovial sarcoma, angiosarcoma).
Reference
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