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High-Grade Pleomorphic Undifferentiated Sarcoma of Bone


  • High grade malignancy with histiocytic mesenchymal cell of origin
  • Previously known as Malignant Fibrous Histiocytoma


  • May be primary (70%) or secondary (30%)
  • Diagnosis should be made after excluding other sarcomas with specific matrix production (eg, bone)


  • Most commonly in middle aged patients (45-55)
  • M:F = 3:2
  • Can be associated with preexisting Paget's disease, XRT, bone infarcts (most common malignancy associated with bone infarcts (idiopathic, sickle-cell, caisson's disease, use of steroids, Gaucher's disease as underlying etiologies) (less common secondary tumors are osteosarcoma, fibrosarcoma, angiosarcoma, poorly differentiated sarcoma), FD, NOF, ABC, hereditary bone dysplasia (up to 50% reported)
  • Pain ± swelling
  • Has been reported in brothers, one with multiple lesions
  • 10% with pathologic fx
  • ↑ alkφ with Paget's disease or osteosarcoma


  • There is benign variant histologically similar in the cervical spinous processes
  • Aggressive osteolysis, premeative, destructive tumor growth centrally located in the metaphysis of long bones
  • Sequestrum may be seen
  • Most common site is distal femoral and proximal tibial metaphysis
  • Diaphyseal involvement in < 10%
  • Periosteal reaction is reported rare to frequent, cortical expansion infrequent
  • May appear as a cold defect on bone scan
  • MRI: Inhomogeneous, nodular pattern with peripheral Gd-DTPA enhancement on T1- and T2-weighted images
  • ± extraosseous extension (80-100% reported)
  • ± joint invasion (up to 50%)


  • Extensive permeation of host bone
  • ± soft tissue mass


  • Subtypes
    • Storiform-pleomorphic (50-60%)
    • Myxoid (25%)
  • Spindle cells in a storiform pattern
  • Little collagen present with the fibroblast having abundant cytoplasm
  • Nuclear pleomorphism
  • Anaplastic giant cells can distinguish this lesion from fibrosarcoma
  • Malignant foamy cells
  • 50% with necrosis and hemosiderin
  • Most with chronic inflammatory cells
  • 1/3 with giant cells


  • Osteosarcoma
  • Fibrosarcoma
  • Secondary MFH (30% reported cases) (arising in bone infarcts, Paget disease, irradiated tissue, fibrous dysplasia, enchondroma, GCT, chronic osteomyelitis)


  • Wide resection
  • Adriamycin the only chemotherapeutic agent found effective (50% 5 year survival vs 20-30% with surgery alone) and adjuvant chemotx may be better than neoadjuvant to ? drug resistance but neoadjuvant better to ? safety for limb salvage
  • Higher rate of disease free survival (94 vs 61%) if a good histologic response to chemotx
  • XRT — different opinions re: pre- or post-resection doses; used for unresectable lesions, or those presenting with wide spread metastases
  • Lymph node metastases may occur rarely
  • LR reported in up to 51%


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