Not recommended: malignant schwannoma, neurogenic sarcoma, malignant neurilemoma, neurofibrosarcoma
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Ultrasonographic Findings
Angiographic Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
Immunohistochemistry
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Hassan A et al: Systemic options for malignant peripheral nerve sheath tumors. Curr Treat Options Oncol. 22(4):33, 2021
Yun JS et al: Peripheral nerve sheath tumor: differentiation of malignant from benign tumors with conventional and diffusion-weighted MRI. Eur Radiol. 31(3):1548-57, 2021
Ahlawat S et al: Current status and recommendations for imaging in neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis. Skeletal Radiol. 49(2):199-219, 2020
Tovmassian D et al: The role of [18F]FDG-PET/CT in predicting malignant transformation of plexiform neurofibromas in neurofibromatosis-1. Int J Surg Oncol. 2016:6162182, 2016
Schaefer IM et al: Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 39(9):1234-41, 2015
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
Karabatsou K et al: Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1. Neurosurgery. 65(4 Suppl):160-70, 2009
Grobmyer SR et al: Malignant peripheral nerve sheath tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 97(4):340-9, 2008
Weiss SW et al: Malignant tumors of the peripheral nerve. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 903-25, 2008
Park SK et al: Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature. Surg Neurol. 68(1):79-84; discussion 84, 2007
Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 337-8, 2006
Lee YJ et al: Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures. J Pediatr Surg. 41(2):e19-22, 2006
Keel SB et al: Orthopaedic implant-related sarcoma: a study of twelve cases. Mod Pathol. 14(10):969-77, 2001
Navarro O et al: Malignant peripheral nerve-sheath tumor arising in a previously irradiated neuroblastoma: report of 2 cases and a review of the literature. Pediatr Radiol. 30(3):176-80, 2000
Khan RJ et al: Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of the literature. Pathology. 30(3):237-41, 1998
Terry DG et al: Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis. Skeletal Radiol. 27(6):346-9, 1998
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Malignant peripheral nerve sheath tumor (MPNST)
Synonyms
Not recommended: malignant schwannoma, neurogenic sarcoma, malignant neurilemoma, neurofibrosarcoma
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Ultrasonographic Findings
Angiographic Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
Immunohistochemistry
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Hassan A et al: Systemic options for malignant peripheral nerve sheath tumors. Curr Treat Options Oncol. 22(4):33, 2021
Yun JS et al: Peripheral nerve sheath tumor: differentiation of malignant from benign tumors with conventional and diffusion-weighted MRI. Eur Radiol. 31(3):1548-57, 2021
Ahlawat S et al: Current status and recommendations for imaging in neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis. Skeletal Radiol. 49(2):199-219, 2020
Tovmassian D et al: The role of [18F]FDG-PET/CT in predicting malignant transformation of plexiform neurofibromas in neurofibromatosis-1. Int J Surg Oncol. 2016:6162182, 2016
Schaefer IM et al: Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 39(9):1234-41, 2015
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
Karabatsou K et al: Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1. Neurosurgery. 65(4 Suppl):160-70, 2009
Grobmyer SR et al: Malignant peripheral nerve sheath tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 97(4):340-9, 2008
Weiss SW et al: Malignant tumors of the peripheral nerve. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 903-25, 2008
Park SK et al: Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature. Surg Neurol. 68(1):79-84; discussion 84, 2007
Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 337-8, 2006
Lee YJ et al: Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures. J Pediatr Surg. 41(2):e19-22, 2006
Keel SB et al: Orthopaedic implant-related sarcoma: a study of twelve cases. Mod Pathol. 14(10):969-77, 2001
Navarro O et al: Malignant peripheral nerve-sheath tumor arising in a previously irradiated neuroblastoma: report of 2 cases and a review of the literature. Pediatr Radiol. 30(3):176-80, 2000
Khan RJ et al: Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of the literature. Pathology. 30(3):237-41, 1998
Terry DG et al: Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis. Skeletal Radiol. 27(6):346-9, 1998
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