Histotype-tailored therapy means choosing treatment by the specific sarcoma subtype (there are more than 70) rather than treating all sarcomas alike.
Examples: imatinib for GIST, trabectedin for leiomyosarcoma and liposarcoma, eribulin for liposarcoma, pazopanib for non-adipocytic STS, nirogacestat for desmoid, vimseltinib for TGCT, tazemetostat for epithelioid sarcoma (withdrawn 2026), afami-cel for synovial sarcoma, larotrectinib for NTRK fusions. ISG-STS 1001 showed histotype-tailored neoadjuvant chemotherapy was not superior to standard epirubicin-ifosfamide, so tailoring applies mainly to targeted agents.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
Showing the organ this term concerns: Sarcomas (soft tissue, bone, GIST).
Shares ISG-STS 1001, A same-week expert second opinion for every rare cancer diagnosis, Sarcomas (soft tissue, bone, GIST).
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Shares ISG-STS 1001, Soft tissue sarcoma of the extremity (localised and advanced), Rare and paediatric cancers without markets, Sarcomas (soft tissue, bone, GIST).
Shares ISG-STS 1001, Soft tissue sarcoma of the extremity (localised and advanced).
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Shares Soft tissue sarcoma of the extremity (localised and advanced), Sarcomas (soft tissue, bone, GIST).
Shares Trabectedin, Soft tissue sarcoma of the extremity (localised and advanced).
Shares Rare and paediatric cancers without markets, Sarcomas (soft tissue, bone, GIST).