Soft tissue sarcoma unit

Sarcomas are rare malignant tumours that develop from the body’s supporting tissue.

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Sarcomas

Sarcomas most often occur as a deep localised mass in the limbs or abdomen, regardless of the age and sex of the patient. There are many types of sarcomas and dozens of subtypes with levels of aggressiveness that vary greatly from one another.

Thus, their rarity, their pathological diversity and their ubiquitarity in the body make the management of these tumours complex. Particular expertise of trained professionals is therefore necessary from the diagnosis to the treatment of this pathology.

Surgery is the cornerstone of treatment for non-metastatic sarcomas. It must be performed by a “sarcoma expert surgeon”, a pledge of quality essential for the removal of these tumours, which will optimise the survival of patients and reduce their risk of recurrence.

J. Torrent Institute has surgeons who are experts in the treatment of these tumours and is closely linked to an international collaborative network that applies the most innovative treatments.

Epidemiology

Sarcomas account for 1% of all cancers, which corresponds to an incidence of 6/100000 inhabitants per year. They affect men as well as women and their frequency increases with age, especially from the age of fifty. Children and young adults are not spared since they are affected in about 10% of cases.

Sarcomas can be classified according to their origin. In that respect, the most common are “soft tissue sarcomas”, including visceral sarcomas, which take their name from the tissue they resemble, for example:

  • Fatty tissue, liposarcoma.
  • Smooth muscle tissue, leiomyosarcoma.
  • Fibrous tissue (tendon and ligament), fibrosarcoma.
  • Blood vessels, angiosarcoma.
  • Wall of the digestive tract, gastrointestinal stromal tumour (GIST).

Soft tissue sarcomas develop from the limbs in 50% of the cases, in the abdomen, thorax and trunk wall in 45% and in the head and neck in 5% of cases. Depending on the location of the sarcoma, patient care presents very different problems.

Hard tissue sarcomas” account for 10% of sarcomas and preferentially affect children. They develop from bones and cartilage. These are osteosarcomas, Ewing’s sarcomas and chondrosarcomas.

Diagnostic

Any clinical suspicion of soft tissue sarcoma must lead to magnetic resonance imaging (MRI) and/or CT scan for retroperitoneal locations. This makes it possible to specify the nature of the swelling, its size, its topography, its anatomical relations, and to eliminate differential diagnoses.

After that, it is necessary to characterise the sarcoma by performing a biopsy under radiological control (ultrasound or CT scan). This biopsy should be performed by a trained radiologist and discussed in advance with the surgeon at a dedicated consultation meeting.

This biopsy will then be analysed by a pathologist specialised in the pathology, which will make it possible to obtain the most precise diagnosis before any therapeutic management.

Treatments

Surgery is the main treatment for soft tissue sarcomas when they are localised. Surgical excision consists of removing the tumour without breaking it with a margin of healthy tissue wide enough in the surroundings in order to minimise the risk of local recurrence.

It must be performed by a surgeon with the expertise of the problems related to the management of sarcomas (prevention of the risk of tumour fragmentation, experience in multi-visceral excision, competence in reconstruction surgery).

Radiation therapy may be offered in addition to soft tissue sarcoma surgery, especially when the tumour is large, deep-seated and high-grade. The last approach called intensity-modulated radiation therapy (IMRT) can be performed when the sarcoma is close to certain organs (lung, gastrointestinal tract, spinal cord) to preserve their integrity.

Chemotherapy may precede the surgical procedure of localised sarcoma. It is proposed when the tumour is difficult to operate. The most common situations are high tumour volume and/or adverse localisation (nerve or vessels in the proximity) and/or a more aggressive type of sarcoma. The goal is to obtain tumour size reduction or necrosis of the tumour which will facilitate surgical excision.

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