Articles Vol. 63 No. 5 (2022) 29/09/2022

MALIGNANT PERIPHERAL NERVE SHEATH TUMORS SPINE METASTASES - DOCUMENT REVIEW A CASE REPORTS

Cung Van Cong1,2
1 National Lung Hospital
2 Bệnh viện Phổi Trung ương
Corresponding author: vancong13071964@gmail.com
DOI: 10.52163/yhc.v63i5.419
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Keywords
Malignant Peripheral Nerve Sheath Tumor, malignant neurilemmoma, malignants chwannoma, neurofibrosarcoma.
Abstract

Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare, high-grade soft tissue sarcomas
of extranodal origin and often metastasize early. The World Health Organization has coined the
term MPNST to replace previously heterogeneous and often confusing terms, such as malignant
schwannoma, malignant neurilemmoma, neurogenic sarcoma and neurofibrosarcoma. MPNSTs
arise from the sheaths of peripheral nerve fibers, either from Schwann cells or from neural crestderived pluripotent cells. Schwann cells are believed to be responsible for the formation of benign as
well as malignant tumors in the nerve fiber sheath. MPNST can appear in many places in the body,
but in the lungs is extremely rare. Imaging is usually nonspecific. The diagnosis was confirmed
by immunohistochemistry. We report a case of MPNST in the lung, with spinal metastases; was
confirmed by biopsy of metastatic lesions and immunohistochemistry at the National Lung Hospital.

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