Malignant Peripheral Nerve Sheath Tumor Arising in Schwannomatosis with Multiple Lung Metastases

Yuhui Ding,Hongtao Rong,Tong Liu,Yi Wang,Jinhao Zhang,Sipeng Li,Tao Zhu

World Neurosurgery（2018）

Cited 5|Views7

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Abstract

•The incidence of MPNST due to the malignant transformation of schwannomatosis was extremely rare.•The splicing site of the SMARCB1 gene mutation was first found in this MPNST patient.•MPNSTs arising from schwannomatosis may have different biological behavior and genetic pathways than other MPNSTs.

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Key words

Intraspinal,Lung metastasis,Malignant peripheral nerve sheath tumor,MPNST,Schwannomatosis,SMARCB1

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