Updated: 10/4/2016

Malignant Fibrous Histiocytoma (Pleomorphic Sarcoma)

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https://upload.orthobullets.com/topic/8030/images/Case A - tibia - xray - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8030/images/Case B - femur  -xray - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8030/images/Case B - femur  - CT - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8030/images/Histology A - high power - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8030/images/mfht1.jpg
https://upload.orthobullets.com/topic/8030/images/mfht2.jpg
Introduction
  • A rare malignant histiocytic lesion of the bone
    • similar in presentation to osteosarcoma, but histologically different (lacks osteoid formation)
    • also known as undifferentiated pleomorphic sarcoma
  • Epidemiology
    • incidence
      • comprises < 2% of all primary malignant bone tumors
      • second most common bone sarcoma in adults after chondrosarcoma
    • demographics
      • affects individuals 20 to 80 years of age
        • highest rates of affected patients in 2nd to 4th decades
      • male > female (3:2)
      • more common in Caucasians than African Americans or Asians
    • body location
      • 75% of cases from appendicular skeleton
      • often metaphysis of long bones
        • primarily distal femur, proximal tibia, proximal humerus
    • risk factors
      • 25% arise as secondary lesion from bone infarct, Paget disease, or prior radiation
  • Genetics
    • mutations of the tumor suppressor gene p53 have been implicated in tumorgenesis of secondary MFH of bone
  • Prognosis
    • 50% - 60% survival at 5 years
    • worse prognosis with secondary MFH compared to primary lesions
    • pulmonary metastasis common in up to 30%
    • good response (> 90% tumor necrosis) to neoadjuvant chemotherapy found to have a much better prognosis
    • age younger than 40 years associated with improved disease-free survival rate
Presentation
  • History
    • new, painful mass
    • can present with pathologic fracture
  • Symptoms
    • pain
    • constitutional symptoms
      • fevers, chills, night sweats and weight loss
  • Physical exam
    • inspection
      • swelling
      • limp
    • motion
      • decreased range of motion
    •  
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 extension
      • radiographs often non-specific
  • MRI
    • indications
      • necessary to better characterize lesion
    • findings
      • deep seated heterogeneous mass
      • often associated with internal hemorrhage
      • T1- low signal
      • T2- high signal
  • CT scan
    • indications
      • to assess the amount and character of bone destruction
  • Bone scan
    • indications
      • may be ordered as part of pre-treatment staging
    • findings
      • shows increased uptake in adjacent cortex
Studies
  • Labs
    • elevated WBC
    • eosinophilia
    • abnormal LFTs
    • hypoglycemia
  • Histology
    • pleomorphic spindle cells and histiocytic cells in storiform pattern 
    • malignant multinucleated giant cells with grooved or indented nuclei 
    • areas of chronic inflammatory cells
    • variable collagen production
    • fibrous fascicles radiate from focal hypocellular areas
    • hemorrhagic and necrotic regions not infrequent; suggest high-grade lesion
Treatment
  • Operative (similar to osteosarcoma)
    • neoadjunctive chemotherapy, wide resection, postoperative chemotherapy +/- radiation 
      • indications
        • standard of care
      • chemotherapy
        • preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection
      • surgical resection
        • correct surgical option is based on the location of the tumor and the feasibility of obtaining a wide surgical margin
          • wide excision or amputation have been found to have a higher 5-year survival rate than those who received intralesional or marginal excision
        • trend towards limb salvage whenever possible
          • options include arthroplasty, resection arthrodesis, allograft reconstruction and rotationplasty
      • radiation
        • indications
          • incomplete or questionable margins in order to reduce risk of local recurrence
          • adjunct to traditional chemotherapy and surgical regimens
Differentials & Groups
 
 
Malignant destructive lesion in older patient(1)
 
Sacral lesions in older patients
 
Treated with wide resection and chemotherapy(2)
MFH / fibrosarcoma
 
 
Chondrosarcoma
 
 
 
Dedifferentiated chondrosarcoma
 
 
Metastatic disease
 
 
 
Lymphoma
 
 
 
Myeloma
 
 
 
Secondary sarcoma
 
 •
 
Osteosarcoma
 
 
 
 
Chordoma
 
 
 
 
Ewings
 
 
 
 
Rhabdomyosarcoma (soft tissue)
 
 
 
 
ASSUMPTIONS: (1) Younger patient is < 40 yrs; (2) assuming no impending fracture




 
IBank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A humerus
Case A femur
Case A femur
(1) - histology does not always correspond to case

 

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(OBQ12.25) A 75-year-old male presents with a slowly enlarging, painless thigh mass. Physical exam demonstrates a mass measuring approximately 7 cm in size. Figure A and B demonstrate a T1-weighted MRI and T2-weighted MRI respectively. The histologic findings from a biopsy are shown in Figure C. Which of the following is the most likely diagnosis. Review Topic

QID: 4385
FIGURES:
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1

Liposarcoma

7%

(329/4410)

2

Desmoid Tumor

15%

(663/4410)

3

Undifferentiated Pleomorphic Sarcoma

63%

(2799/4410)

4

Synovial sarcoma

8%

(352/4410)

5

Schwanoma

5%

(235/4410)

L 3

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