Introduction: Malignant peripheral nerve sheath tumors represent all the neoplasias developed at the expense of cells located in the peripheral nerve sheaths. Complete surgical excision with microscopically healthy edges remains the only curative treatment. Through a clinical case, and a review of the literature, we will report our experience in the management of a tumor of the peripheral nerve sheaths. Observation: Madam K.L, 34 years old, admitted for management of a spontaneously painful left axillary mass, and in whom the clinical examination found a patient, in good general condition, with the presence of a 8cm left axillary mass hard to fixed palpation in relation to the 2 planes, painless on palpation. Thoracic computed tomography revealed an encapsulated left axillary tissue mass compressing the axillary pedicle measuring 84mm*67mm with areas of necrosis. MRI had objectified the presence of a fusiform mass extended to the inter-scalene space most likely evoking a tumor of the malignant peripheral nerve sheaths. The patient had undergone complete tumor excision with section of the left subclavian artery and end-to-end anastomosis, with simple postoperative consequences. Conclusion: MPNSTs are rare tumors. Surgery occupies an essential place in the treatment of this disease, it must be planned in order to avoid vascular and nervous risks. Radiotherapy may be indicated as an adjuvant or in the event of an unhealthy margin.
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