. High-Grade Pleomorphic Undifferentiated Sarcoma of Bone. PORTNotes. In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created Feb 16, 2009 16:36. Last modified Oct 26, 2016 09:55 ver.6. Retrieved 2018-01-21, from https://www.orthopaedicsone.com/x/zQAjAQ.
DEFINITION AND PATHOGENESIS
- 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
- 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
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Secondary MFH (30% reported cases) (arising in bone infarcts, Paget disease, irradiated tissue, fibrous dysplasia, enchondroma, GCT, chronic osteomyelitis)
DISEASE COURSE AND TREATMENT
- 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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