Soft tissue sarcoma: specialists and information

Leading Medicine Guide Editors
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Leading Medicine Guide Editors

Soft tissue sarcoma covers a broad spectrum of malignant tumors (sarcomas) of various soft tissues. Soft tissue includes connective, fatty and muscle tissue as well as blood and lymph vessels and peripheral nerve tissue.

Below you will find further information and selected special sites for the treatment of soft tissue sarcomas.

ICD codes for this diseases: C49

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Article overview

Forms and classification of soft tissue sarcomas

Cancer means that the body's own tissue changes and then proliferates uncontrollably.

Soft tissue sarcomas are divided into different subtypes depending on their tissue of origin.

The frequently occurring liposarcoma (10 percent of cases) develops from fat cells, for example . The tumor cells have fine tissue characteristics of fat cells. The more similar the cancer cells are to the original tissue, the less aggressive they are.

Liposarkom

Liposarcoma is a slow-growing, painless tumor that is usually located in the extremities @ timonina /AdobeStock

There are over 50 different soft tissue sarcomas in total. With around one to two cases per 100,000 inhabitants per year, soft tissue sarcomas in adults are very rare tumors. The peak incidence in adults is between the ages of 45 and 55.

In children and adolescents, soft tissue tumors are the fourth most common group of solid tumors. Most children are under the age of 15 when they develop soft tissue sarcoma.

Symptoms of soft tissue sarcoma

The symptoms and their severity depend greatly on the location and extent of the soft tissue sarcoma . Some soft tissue sarcomas - particularly of the lower limbs - are initially characterized by painless swelling.

In around 60 percent of cases, soft tissue sarcoma is localized in the upper limbs (arms and legs). 40 percent of soft tissue sarcomas develop in the lower limbs.

Less frequently, soft tissue sarcomas develop in the following areas:

  • in the trunk of the body
  • the abdomen or
  • in the neck and head region

Depending on its location and size, the tumor can restrict the function of neighboring structures. For example, tumors close to joints lead to restricted movement of the joint.

Tumors in the urinary and reproductive tract lead to

Less common are general symptoms such as

  • Loss of performance
  • tiredness
  • fatigue or
  • weight loss

In around one in ten people affected, the tumor has already formed metastases (metastases) at the time of diagnosis. Soft tissue sarcomas often spread to the lungs and cause symptoms such as a chesty cough and shortness of breath, especially in stressful situations.

Causes and risk factors for the development of soft tissue sarcoma

The development and cause of soft tissue sarcomas is still largely unclear.

A genetic component has so far only played a role in a very small proportion of soft tissue sarcomas. People with Recklinghausen's disease therefore have a greatly increased risk of a malignant tumor of the nerve sheath tissue. Recklinghausen's disease is a hereditary neurofibromatosis type 1 and can cause nerve tumors.

Some chemicals, especially in combination with previous radiation therapy, can also increase the risk of soft tissue sarcoma.

Hazardous chemicals include:

  • thermoplastic polyvinyl chloride (PVC)
  • asbestos fibers and
  • arsenic
AsbestfaserAsbestos fibers increase the risk of developing soft tissue sarcoma @ ALEXEY /AdobeStock

    Finally, as with all types of cancer, the risk of developing soft tissue sarcoma increases with age.

    Treatment of soft tissue sarcoma

    The treatment of soft tissue sarcomas is less standardized than for other types of tumours. The optimal strategy is currently still the subject of research.

    The following specialists should be involved in planning the treatment:

    The treatment of soft tissue sarcoma is aimed at the surgical removal of the tumor (resection of the primary tumor) and its metastases.

    The surgical technique and strategy depend on

    • Size
    • Localization and
    • Growth behavior of the sarcoma
    • Surgery of the primary tumor

    The majority of soft tissue sarcomas remain confined to the natural spatial boundaries of the body for a long period of time. For this reason, doctors often remove the tumor together with all the tissue of the affected body region. This also includes the tissue envelope (compartment). This is therefore referred to as a compartment resection.

    This procedure is not always possible for tumors in the neck and head area or on the trunk due to their unfavorable location and extent. Alternatively, doctors often remove the sarcoma with a margin of healthy tissue.

    The affected regions are then irradiated to kill any remaining tumor cells (adjuvant radiotherapy).

    For patients with localized tumours in the early stages, surgical removal of the tumour is often sufficient.

    If the tumor is extensive and metastases are present, additional chemotherapy and/or radiotherapy is usually required.

    • Neoadjuvant therapy to reduce the size of the sarcoma

    In most cases, the metastases of soft tissue sarcomas are confined to the lungs. This applies in particular to the early stages of the disease.

    As the cancer progresses, metastases can also occur outside the lungs. In these cases, systemic chemotherapy is recommended as initial therapy.

    It is given before the operation. Doctors refer to this as neoadjuvant therapy. Neoadjuvant therapy can also help if the tumor can only be removed by amputation.

    The aim is to reduce the size of the sarcoma so that doctors can remove it completely without amputating the affected limb.

    The following methods are available to shrink the tumor:

    • Systemic chemotherapy with or without additional overheating of the tumor cells(hyperthermia)
    • Radiotherapy or
    • Isolated hyperthermic limb perfusion (ILP)

    Isolated hyperthermic limb perfusion leads to preservation of the affected limb in around 80 percent of cases. This does not worsen the overall prognosis.

    The doctors first isolate the circulation of the affected limb from the rest of the body's circulation by means of a surgical procedure. A heart-lung machine takes over the heart function.

    They then administer cancer drugs (cytostatics) to this isolated circulation in order to kill the tumor cells. In most cases, the cancer drug melphalan is used in perfusion treatment.

    This avoids serious systemic side effects(affecting the whole body).

    In addition, doctors heat the tissue of the limb to enhance the effect of the cancer drugs.

    Infusion bei ChemotherapieTreatment for soft tissue sarcoma includes surgery, radiotherapy and chemotherapy @ Seventyfour /AdobeStock

    • Reconstructive surgery

    In some cases, doctors have to remove such large areas of tissue that reconstructive surgery is necessary. This surgery is used to compensate for large tissue defects and restore the functionality of the affected body part as far as possible.

    Reconstructive surgery can be performed directly after the removal of the sarcoma. If further treatment measures such as chemotherapy or radiotherapy are planned after the operation, the reconstructive surgery is only performed afterwards.

    Doctors move or transplant the body's own tissue such as muscle flaps to cover skin and soft tissue defects.

    Specialists can now transplant the following structures to compensate for tissue loss:

    Doctors can also compensate for possible bone defects with so-called endoprostheses. Endoprostheses are implants that replace natural structures in the body.

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