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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.
UPS stands out among sarcomas for immunotherapy: in the SARC028 phase 2 trial pembrolizumab produced objective responses in undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma but almost none in leiomyosarcoma or synovial sarcoma, and the randomised SU2C-SARC032 trial found that pembrolizumab added to preoperative radiotherapy and surgery improved disease-free survival in stage III UPS and dedifferentiated liposarcoma of the limbs. Tertiary lymphoid structures and B-cell-rich immune classes predict who benefits.
One of the commonest soft tissue sarcomas of older adults, usually a deep, fast-growing mass in the thigh or upper arm; about a third of patients with high-grade limb tumours develop lung metastases despite surgery and radiotherapy.
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, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, 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)
Wide resection with preoperative or postoperative radiotherapy; neoadjuvant anthracycline-ifosfamide for large, deep, high-grade tumours in fit patients (ISG-STS 1001).
Doxorubicin alone or doxorubicin plus ifosfamide (EORTC 62012) when response matters.
Gemcitabine-docetaxel, trabectedin, pazopanib; pembrolizumab in trials or off label after SARC028.
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The general principles on the sarcoma subtype pages (reference centre surgery, radiotherapy for high-grade deep tumours, doxorubicin first line, histology-directed later lines) follow this guideline.
Three cycles of neoadjuvant anthracycline-ifosfamide is a reasonable standard for fit patients with high-risk limb or trunk sarcoma, particularly undifferentiated pleomorphic sarcoma.
PD-1 blockade is an off-label or trial option specifically for undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma, not for sarcoma in general.
Single-agent doxorubicin is the standard first-line palliative treatment for most advanced sarcomas, with doxorubicin-ifosfamide reserved for fit patients in whom tumour shrinkage matters.
Query for this cancer: (TITLE:"Undifferentiated pleomorphic sarcoma" OR ABSTRACT:"Undifferentiated pleomorphic sarcoma" OR TITLE:"UPS" OR ABSTRACT:"UPS" OR TITLE:"Malignant fibrous histiocytoma historic" OR ABSTRACT:"Malignant fibrous histiocytoma historic" OR TITLE:"MFH" OR ABSTRACT:"MFH" OR TITLE:"Pleomorphic sarcoma, not otherwise specified" OR ABSTRACT:"Pleomorphic sarcoma, not otherwise specified") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Undifferentiated pleomorphic sarcoma (UPS), not a curated reading list.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Take on an empty stomach (1 hour before or 2 hours after food).
See all on the product pages:DocetaxelDoxorubicinGemcitabineIfosfamidePazopanibPembrolizumabTrabectedin·Printable cards in the navigator
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