Updated: 10/6/2016

Malignant Peripheral Nerve Sheath Tumor

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Introduction
  • Overview
    • arises from a peripheral nerve or neurofibroma
    • also known as neurofibrosarcoma or malignant schwannoma
  • Epidemiology
    • demographics
      • 30 to 55 years for solitary neurofibromas
      • 20 to 40 years for neurofibromatosis type 1 (NF-1)
  • Genetics
    • most cases associated with NF-1
      • 4% incidence in these patients
  • Prognosis
    • survival for solitary lesion is 75% (5-year survival)
    • survival associated with NF-1 is 30% (5-year survival) 
Presentation
  • Presentation
    • soft-tissue mass
    • most arise from large nerves (sciatic, brachial plexus)
    • motor and sensory deficit of the affected nerve
Imaging
  • Radiograph
    • usually normal or non-specific soft-tissue mass
  • MRI
    • low-intensity on T1-weighted images
    • high intensity on T2-weighted images 
    • serial MRI may show enlargement of previous benign nerve sheath lesion suggesting malignant transformation
  • Bone scan
    • mildly positive
Studies
  • Histology
    • classic characteristics are
      • spindle cells with wavy nuclei resembling fibrosarcoma  
    • Immunohistochemistry
      • positive S100 stain
      • keratin staining is negative
Treatment
  • Operative
    • wide surgical resection + radiation
      • indications
        • standard of care in most patients
        • in general, treated as high-grade sarcoma
      • technique
        • wide resection should include entire affected nerve
      • radiation
        • perform preoperative adjuvant radiation
      • chemotherapy not useful
 

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(OBQ04.178) A 45-year-old man presents for evaluation of 6 months of radicular buttock pain. A MRI is shown in Figure A. The resected mass stains positive for S100 and the histology is shown in Figures B. What is the most likely diagnosis? Review Topic

QID: 1283
FIGURES:
1

Schwannoma

33%

(562/1711)

2

Liposarcoma

4%

(73/1711)

3

Lipoma

0%

(2/1711)

4

Malignant peripheral nerve sheath tumor

61%

(1037/1711)

5

Giant cell tumor of tendon sheath

2%

(29/1711)

L 4

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