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Soft tissue sarcoma - Rare malignant tumour of the connective tissue

Soft tissue sarcomas are rare malignant tumours of the connective tissue. Find out more about symptoms, diagnosis, treatment options and new approaches such as the TRAPTRAP study.

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Things worth knowing about "Soft tissue sarcoma"

Soft tissue sarcomas are rare malignant tumours of the connective tissue

soft tissue sarcoma is a malignant tumour that arises from the mesenchymal tissue.strong data-start=""201"" data-end=""225"">mesenchymal tissue of the soft tissue - this includes connective, fatty, muscle, nerve, vascular and tendon sheath tissue. This type of tumour is rare, but accounts for around 1% of all malignant tumours in adults. There are over 50 histological subtypes, which can differ greatly in terms of growth, metastasis and response to treatment.

  • Liposarcoma (fatty tissue)

  • Leiomyosarcoma (smooth muscle)

  • Synovial sarcoma (soft tissue near the joint)

  • Angiosarcoma (vessels)

  • Undifferentiated pleomorphic sarcoma (UPS)

  • recognised late.
    Typical signs are:

    • Painless, progressive swelling or lump

    • Restriction of movement when joints or nerves are affected

    • Pain or discomfort, with increasing spread

    • General symptoms such as fatigue, weight loss or fever are rare

    sporadically, without a clear cause. Possible risk factors:

    • Previous radiotherapy

    • Genetic syndromes (e.g. Li-Fraumeni syndrome, neurofibromatosis type 1)

    • Chronic inflammation or tissue damage

    • CD13 expression, relevant for targeted therapies

  • Imaging (MRI, CT): to assess the size, location and possible metastases

  • Biopsy: for histological and molecular subtyping

  • Staging examinations (chest CT, possibly PET-CT): to rule out lung metastases

  • Surgical resection with a safety margin is the most important therapy

  • Radiation therapy for local control

  • Chemotherapy, especially for high-risk tumours or metastases

  • Targeted Therapy in molecularly genetically defined subtypes

  • Combination therapies in clinical trials (e.g. TRABTRAP study)

    • additional therapy with tTF-NGR, a fusion protein that specifically closes tumour-supplying vessels via CD13

  • Detected early and operated on completely: good chances of recovery

  • Metastasised or aggressive subtypes: poorer prognosis, but increasingly treatable with innovative therapies

  • Literature references:

    • ""Soft tissue sarcomas: ESMO-EURACAN Clinical Practice Guidelines."" Annals of Oncology.

    • ""Patient guide for soft tissue sarcomas.""

    • ""TRAPTRAP trial - Targeted vascular infarction in soft tissue sarcoma."" Journal of Clinical Oncology.

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