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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Undifferentiated pleomorphic sarcoma is the diagnosis for a high-grade soft tissue sarcoma whose cells show no recognisable line of differentiation. It is treated with surgery and radiotherapy, with doxorubicin-based chemotherapy for high-risk or spread disease, and it is one of the few sarcomas in which the immunotherapy drug pembrolizumab has clear activity.
Undifferentiated pleomorphic sarcoma is a diagnosis of exclusion: a genomically complex, high-grade sarcoma with no line of differentiation on histology, immunohistochemistry or molecular testing. It replaced the older label malignant fibrous histiocytoma once many such tumours were reclassified as dedifferentiated liposarcoma, myxofibrosarcoma or pleomorphic leiomyosarcoma. It arises mostly in the deep soft tissues of the limbs in people over 60, less often in the retroperitoneum, and its genome shows widespread copy number change with TP53, RB1, CDKN2A and ATRX loss rather than a driver fusion.
Localised disease is treated with wide resection and radiotherapy, given preoperatively or postoperatively, following the principles established for extremity sarcoma. In the Italian Sarcoma Group ISG-STS 1001 trial, three cycles of neoadjuvant epirubicin and ifosfamide improved relapse-free and overall survival in high-risk limb and trunk sarcomas, a benefit driven partly by the UPS group, and full-dose anthracycline-ifosfamide is offered to fit patients with large, deep, high-grade tumours. Advanced disease is treated with doxorubicin alone or with ifosfamide, gemcitabine-docetaxel, trabectedin or pazopanib.
| Setting | Approach | Guideline |
|---|---|---|
| Localised, limb or trunk | Wide resection with preoperative or postoperative radiotherapy; neoadjuvant anthracycline-ifosfamide for large, deep, high-grade tumours in fit patients (ISG-STS 1001). | not mapped |
| Advanced, first line | Doxorubicin alone or doxorubicin plus ifosfamide (EORTC 62012) when response matters. | not mapped |
| Later lines | Gemcitabine-docetaxel, trabectedin, pazopanib; pembrolizumab in trials or off label after SARC028. | not mapped |