Lower Back Epithelioid Malignant Peripheral Nerve Sheath Tumor: A Case Presentation and Review of Literature

Kelly Shortridge, Andrew Fraser, Hugo Araujolino and Raul Lopez

Department of General Surgery, Mountain Vista Medical Center, USA

^*Correspondance to: Kelly Shortridge 

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Abstract:

Epithelioid Malignant Peripheral Nerve Sheath Tumor (EMPNST) is a rare variant of an already rare sarcoma, Malignant Peripheral Nerve Sheath Tumor (MPNST). Approximately 5% of MPNSTs are EMPNSTs. EMPNST is similar to MPNST in that they are both of neural origin. Histologically, EMPNST differs in that its cells are epithelioid in nature. EMPNST has a specific histologic marker profile which is diffusely positive for S100 & SOX-10 with a lack of expression of INI-1. In this study, we present the case of a 30-year-old female with a non-locally invasive EMPNST who underwent wide local surgical excision. Research is limited in this disease process and management of these neoplasms is not structured or standardized. Wide local excision currently remains the mainstay of treatment and chemoradiation is an option for advanced or metastatic disease.

Keywords:

Epithelioid Malignant Peripheral Nerve Sheath Tumor; Malignant Peripheral Nerve Sheath Tumor; Excision

Cite the Article:

Shortridge K, Fraser A, Araujolino H, Lopez R. Lower Back Epithelioid Malignant Peripheral Nerve Sheath Tumor: A Case Presentation and Review of Literature. Ann Clin Case Rep. 2019; 4: 1670.