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Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors

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Malignant Peripheral Nerve Sheath Tumor.

Aaron W James¹, Elizabeth Shurell², Arun Singh³

¹Department of Pathology, Johns Hopkins University, 600 North Wolfe Street, Baltimore, MD 21287-6417, USA.

Surgical Oncology Clinics of North America

|September 4, 2016

Summary

This summary is machine-generated.

Malignant peripheral nerve sheath tumors (MPNSTs) are a type of soft tissue sarcoma. Key factors for distinguishing MPNST from benign tumors include size and fludeoxyglucose F 18 avidity, aiding diagnosis.

Keywords:

Atypical neurofibroma Malignant peripheral nerve sheath tumor NF1 Neurofibroma Neurofibromatosis

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Published on: March 7, 2011

Area of Science:

Oncology
Pathology
Radiology

Background:

Malignant peripheral nerve sheath tumor (MPNST) is the sixth most common soft tissue sarcoma.
MPNSTs often develop from peripheral nerves or neurofibromas.
Neurofibromatosis type 1 is the primary risk factor for MPNST development.

Purpose of the Study:

To outline the diagnostic parameters for MPNST.
To discuss the role of imaging and histopathology in MPNST diagnosis.
To describe current management strategies for high-grade MPNST.

Main Methods:

Review of diagnostic criteria for MPNST.
Analysis of imaging features, including tumor size and fludeoxyglucose F 18 avidity.
Evaluation of histopathologic and immunohistochemical findings.
Assessment of current surgical management approaches.

Main Results:

Tumor size and fludeoxyglucose F 18 avidity are crucial for differentiating MPNST from benign tumors.
Histopathology, particularly light microscopy, is the primary diagnostic method.
Immunohistochemical stains are valuable for distinguishing high-grade MPNST from mimics.
Surgical management for high-grade MPNST aligns with that of other high-grade soft tissue sarcomas.

Conclusions:

Accurate diagnosis of MPNST relies on a combination of clinical factors, imaging, and histopathology.
Distinguishing MPNST from benign nerve sheath tumors requires careful evaluation of specific parameters.
Standardized surgical approaches are employed for high-grade MPNST.