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Undifferentiated High-grade Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma ) Occurring in the Nerve Root: A Rare Case Report and Review of the Literature

Tantan Liu, Qing Li, Zhaoxiang Zhang


Introduction: undifferentiated pleomorphic sarcoma (UPS) represents a group of pleomorphic mesenchymal neoplasms without any defined cell differentiation, occurs more commonly in the extremities. However, we report a rare case of UPS, not malignant peripheral nerve sheath tumor (MPNST) in which the nerve root of the forth cervical vertebrae and adjacent tissues were involved.

Presentation of Case: Histopathologically, this tumor was composed of highly atypical spindle cells, pleomorphic cells and multinucleated giant cells. Nuclear mitoses were frequently observed. Immunohistochemistrical results showed that the tumor cells stained positively for vimentin but negatively for all the other immunomarkers.

Conclusion: We here reported an extremely rare case of UPS arising from the nerve root of the forth cervical vertebrae and proposed a hypothesis “tumors without any expression of neural markers should be diagnosed as UPSs, not MPNSTs, even though which may arise from peripheral nerve branches”.


Undifferentiated pleomorphic sarcoma; Malignant fibrous histiocytoma; Nerve root.

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