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CASE REPORT
Year : 2017  |  Volume : 4  |  Issue : 2  |  Page : 89-92

Malignant nerve sheath tumor with retroperitoneal extension


1 Resident, Department of Neurosurgery, Medical College and Hospital, Thiruvananthapuram, Kerala, India
2 Head, Department of Neurosurgery, Medical College and Hospital, Thiruvananthapuram, Kerala, India
3 Assistant Professor, Department of Neurosurgery, Medical College and Hospital, Thiruvananthapuram, Kerala, India

Correspondence Address:
Sourabh K Jain
Resident, Department of Neurosurgery, Medical College and Hospital, Thiruvananthapuram, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.5005/jp-journals-10039-1136

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Aim: To discuss a rare entity that was encountered along with its findings and outcome. Background: Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas which originate from peripheral nerves or from cells associated with the nerve sheath, such as Schwann cells, perineural cells, or fibroblasts. These are rare but aggressive neoplasms. The MPNST are frequently seen in the head, neck, and upper extremities. Retroperitoneal cases are fairly rare and clinically difficult to be detected. These tumors have a very aggressive clinical course. The MPNSTs can arise de novo or from malignant transformation of benign nerve sheath tumors. The clinical course is usually short. In most cases, the diagnosis depends on the pathologic and immunohistochemical studies. Case report: Here we report the case of a 42-year-old male patient who presented with swelling over lower back region and paresthesia over anterior aspect of left thigh since last 2 years, which aggravated over last 1 month. It was of pins-and-needle sensation type. Radiologically, the tumor was found extended from retroperitoneum into the spinal canal through the intervertebral foramina of D12–L1 and L1–L2 and compressing dura and its contents. Patient was treated surgically and histopathological examination confirmed MPNSTs. Conclusion: The MPNSTs can arise de novo or from malignant transformation of benign nerve sheath tumors. The clinical course is usually short. In most cases, the diagnosis depends on the pathologic and immunohistochemical studies.


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