Fact-sheet: Cndifferentiated pleomorphic sarcoma
Updated on 06/24/2025 at 6:21 AM
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Definition
Aggressive soft-tissue sarcoma arising from primitive mesenchymal cells
May be primary or secondary (irradiated field, bone infarct, Paget disease, fibroxanthoma, fibrous dysplasia, chondroma, chronic osteomyelitis)
Clinical features
Soft-tissue mass or bone lesion occurring most often between the 5th and 7th decades
Male-to-female ratio = 3
Mass, pain, pathologic fracture
Radiography
Bone lesion: moth-eaten or permeative osteolysis, more rarely geographic, with erosion and/or cortical breakthrough. Soft-tissue involvement. Little or no periosteal reaction. Possible pathologic fracture.
CT
Staging with chest-abdomen-pelvis CT to look for lymph node, pulmonary, hepatic, or bone metastases.
MRI
Reference examination.
Aggressive, heterogeneous soft-tissue mass, with heterogeneous enhancement after contrast injection. Precisely describe the muscles, compartments, and neurovascular structures involved.
Aggressive bone lesion of nonspecific appearance, which may be large and invade the soft tissues
Management
Management at a sarcoma reference center with Netsarc+ multidisciplinary tumor board.
En bloc resection with chemotherapy and sometimes radiotherapy.