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| Introduction |
- A rare malignant histiocytic lesion of the bone
- 25% arise as secondary lesion from bone infarct, Paget disease, or prior radiation
- similar to presentation to fibrosarcoma but histologically different
- Epidemiology
- incidence
- most common soft-tissue sarcoma in adults 55 to 80 years of age
- demographics
- male > female (2:1)
- more common in Caucasians than African Americans or Asians
- location
- often metaphyseal of long bones
- primarily distal femur, proximal tibia, proximal humerus
- Genetics
- Prognosis
- 50% - 60% survival at 5 years
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| Presentation |
- History
- Symptoms
- swelling
- limp
- fever
- can present with pathologic fracture
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| Imaging |
- Radiographs
- recommended views
- AP and lateral of affected area
- findings
- lytic and destructive lesion often in the metaphysis
- variable periosteal reaction
- cortical destruction with soft tissue mass
- radiographs often non-specific
- MRI scan
- indications
- necessary to better characterize lesion
- findings
- deep seated heterogeneous mass
- T1- low signal
- T2- high signal
- Bone scan
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| Studies |
- Labs
- elevated WBC
- hypoglycemia
- Histology
- pleomorphic spindle cells and histiocytic cells in storiform pattern
- malignant multinucleated giant cells with grooved or indented nuclei
- chronic inflammatory cells
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| Treatment |
- Operative (similar to osteosarcoma)
- neadjuntive chemotherapy, wide resection, postoperative chemotherapy +/- radiation
- indications
- chemotherapy
- preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection
- surgical resection
- trend towards limb salvage whenever possible
- radiation
- indications
- comon dossage
- 5000cGy preoperatively
- 1,400 to 2,000 cGy postoperatively or brachytherapy if margins close or positive
- higher doses (6,300 to 6,600 cGy) show equal local control rates and lower postoperative wound complications but have a higher fibrosis incidence
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| Differentials & Groups |
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Malignant destructive lesion in older patient(1)
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Sacral lesions in older patients
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Treated with wide resection and chemotherapy(2)
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| MFH / fibrosarcoma |
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| Chondrosarcoma |
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| Dedifferentiated chondrosarcoma |
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| Metastatic disease |
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| Lymphoma |
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| Myeloma |
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| Secondary sarcoma |
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| Osteosarcoma |
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| Chordoma |
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| Ewings |
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| Rhabdomyosarcoma (soft tissue) |
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ASSUMPTIONS: (1) Younger patient is < 40 yrs; (2) assuming no impending fracture
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| IBank |
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Location |
Xray
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Xray
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CT
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B. Scan
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MRI
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MRI
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Histo(1)
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| Case A |
humerus |
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| Case A |
femur |
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| Case A |
femur |
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(1) - histology does not always correspond to case
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Please Rate Educational Value!
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3.0
t-8030
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Average 3.0 of 2 Ratings
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Qbank (1 Questions)
Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions,
Personal Observations, and Biomechanic Studies)
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Schuetze SM. Utility of positron emission tomography in sarcomas. Curr Opin Oncol. 2006 Jul;18(4):369- 73.
PMID:16721133 (Link to Abstract)
Textbooks
- Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
- AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
- Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
- Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
- Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
- Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Undefined
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Randall RL, Albritton KH, Ferney BJ, Layfield L. Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop (Belle Mead NJ). 2004 Dec;33(12):602-8. Review. PubMed PMID: 15641745.
PMID:15641745 (Link to Abstract)
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