What Is a Sarcoma?
A sarcoma is a malignant tumour arising from mesenchymal tissue — the connective tissue that forms muscles, fat, blood vessels, nerves, tendons and cartilage. Sarcomas are rare, representing less than 1% of all adult malignancies, but they are the most common solid tumours in children and young adults. Over 70 histological subtypes exist.
Main Types of Sarcomas
Liposarcoma: Arises from fat tissue. Most common retroperitoneal sarcoma. Subtypes: well-differentiated, dedifferentiated, myxoid, round cell, pleomorphic — with very different behaviours. Leiomyosarcoma: Arises from smooth muscle. Common in the retroperitoneum, uterus, gastrointestinal tract and vascular walls. Gastrointestinal Stromal Tumour (GIST): Arises from interstitial cells of Cajal. Treated with targeted therapy (imatinib) and surgery. Synovial sarcoma: Typically affects young adults in the extremities. Responds to chemotherapy. Rhabdomyosarcoma: Arises from skeletal muscle precursors. Most common soft tissue sarcoma in children. Undifferentiated pleomorphic sarcoma (UPS): High-grade sarcoma of uncertain origin. Ewing sarcoma: Aggressive sarcoma of bone and soft tissue, most common in adolescents. Desmoid tumour (aggressive fibromatosis): Locally aggressive but non-metastasising. Complex management.
Diagnosis
Imaging: MRI is preferred for extremity sarcomas; CT for intra-abdominal. PET-CT for staging. Biopsy: Core needle biopsy (not excisional biopsy) by an experienced team. Biopsy site and technique affect subsequent surgical planning. Pathology review at a sarcoma reference centre is essential for accurate subtype diagnosis. Molecular testing: Increasingly important for subtype classification and targeted treatment decisions (FISH for MDM2, NGS panels).
Treatment Principles
Wide en-bloc surgical resection with clear margins is the cornerstone. Adjuvant or neoadjuvant radiotherapy for selected extremity sarcomas. Chemotherapy for specific subtypes (synovial sarcoma, rhabdomyosarcoma, Ewing). Targeted therapy for GIST (imatinib). Immunotherapy emerging for selected subtypes. Retroperitoneal sarcomas require multivisceral resection at specialised centres.
Have you been diagnosed with a sarcoma? At Quenet Torrent Institute our team specialises in soft tissue and retroperitoneal sarcoma surgery. Request a specialised consultation.