Soft tissue sarcoma that arises from or differentiates toward cells of peripheral nerve sheath
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Widemann BC et al: Biology and management of undifferentiated pleomorphic sarcoma, myxofibrosarcoma, and malignant peripheral nerve sheath tumors: state of the art and perspectives. J Clin Oncol. 36(2):160-7, 2018
Kolberg M et al: Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 15(2):135-47, 2013
Wasa J et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74, 2010
Benz MR et al: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer. 116(2):451-8, 2009
Ferner RE et al: [18F.] 2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol. 19(2):390-4, 2008
Gupta G et al: Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am. 19(4):533-43, v, 2008
Amin A et al: Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina. Spine (Phila Pa 1976). 29(21):E506-9, 2004
Baehring JM et al: Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology. 61(5):696-8, 2003
Kluwe L et al: Constitutional NF1 mutations in neurofibromatosis 1 patients with malignant peripheral nerve sheath tumors. Hum Mutat. 22(5):420, 2003
Su W et al: Malignant peripheral nerve sheath tumor cell invasion is facilitated by Src and aberrant CD44 expression. Glia. 42(4):350-8, 2003
Asavamongkolkul A et al: Malignant peripheral nerve sheath tumor with neurofibromatosis type 1: a 2-case report and review of the literature. J Med Assoc Thai. 84(2):285-93, 2001
Stark AM et al: Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien). 143(4):357-63; discussion 363-4, 2001
Weiss SW et al: Enzinger and Weiss's soft tissue tumors. 4th ed. St. Louis: CV Mosby. 1209-63, 2001
Ferner RE et al: Evaluation of (18)fluorodeoxyglucose positron emission tomography ((18)FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry. 68(3):353-7, 2000
Woodruff JM: Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. Am J Med Genet. 89(1):23-30, 1999
Sangüeza OP et al: Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms. Am J Dermatopathol. 20(1):89-102, 1998
Kransdorf MJ et al: Imaging of soft tissue tumors, 1st ed. Philadelphia: W.B. Saunders. 240-54, 1997
Celli P et al: Primary spinal malignant schwannomas: clinical and prognostic remarks. Acta Neurochir (Wien). 135(1-2):52-5, 1995
Bass JC et al: Retroperitoneal plexiform neurofibromas: CT findings. AJR Am J Roentgenol. 163(3):617-20, 1994
Suh JS et al: Peripheral (extracranial) nerve tumors: correlation of MR imaging and histologic findings. Radiology. 183(2):341-6, 1992
Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991
Riccardi VM et al: Neurofibrosarcoma as a complication of von Recklinghausen neurofibromatosis. Neurofibromatosis. 2(3):152-65, 1989
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Malignant peripheral nerve sheath tumor (MPNST)
Synonyms
Neurofibrosarcoma
Malignant schwannoma
Malignant neurilemmoma
Neurogenic sarcoma
Definitions
Soft tissue sarcoma that arises from or differentiates toward cells of peripheral nerve sheath
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Widemann BC et al: Biology and management of undifferentiated pleomorphic sarcoma, myxofibrosarcoma, and malignant peripheral nerve sheath tumors: state of the art and perspectives. J Clin Oncol. 36(2):160-7, 2018
Kolberg M et al: Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 15(2):135-47, 2013
Wasa J et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74, 2010
Benz MR et al: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer. 116(2):451-8, 2009
Ferner RE et al: [18F.] 2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol. 19(2):390-4, 2008
Gupta G et al: Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am. 19(4):533-43, v, 2008
Amin A et al: Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina. Spine (Phila Pa 1976). 29(21):E506-9, 2004
Baehring JM et al: Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology. 61(5):696-8, 2003
Kluwe L et al: Constitutional NF1 mutations in neurofibromatosis 1 patients with malignant peripheral nerve sheath tumors. Hum Mutat. 22(5):420, 2003
Su W et al: Malignant peripheral nerve sheath tumor cell invasion is facilitated by Src and aberrant CD44 expression. Glia. 42(4):350-8, 2003
Asavamongkolkul A et al: Malignant peripheral nerve sheath tumor with neurofibromatosis type 1: a 2-case report and review of the literature. J Med Assoc Thai. 84(2):285-93, 2001
Stark AM et al: Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien). 143(4):357-63; discussion 363-4, 2001
Weiss SW et al: Enzinger and Weiss's soft tissue tumors. 4th ed. St. Louis: CV Mosby. 1209-63, 2001
Ferner RE et al: Evaluation of (18)fluorodeoxyglucose positron emission tomography ((18)FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry. 68(3):353-7, 2000
Woodruff JM: Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. Am J Med Genet. 89(1):23-30, 1999
Sangüeza OP et al: Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms. Am J Dermatopathol. 20(1):89-102, 1998