Background:

malignant peripheral nerve sheath tumors are soft tissue sarcomas with a low incidence.

Objective:

to present a case of malignant peripheral nerve sheath tumor of the left sciatic nerve who underwent surgical treatment at the University Hospital Manuel Ascunce Domenech in the province of Camagüey in June, 2018.

Presentation of the case:

a 45-year-old male patient, personal history of neurofibromatosis type 1 with a truncal left sciatic pain that turned permanent. He referred gait difficulties and increase of the volume in the left buttock. A tumor mass of seven centimeters was palpated, firm, fix to deep planes and painful at deep palpation. Soft tissue ultrasonography showed: complex oval hypoechoic image of deep location of 45 X 9 X 20 millimeters of regular and well defined contours at 22 millimeters depth from the skin. Surgical treatment was decided base on the tumor resection and radiotherapy. Definitive biopsy after immune histochemistry, informed a high degree malignant peripheral nerve sheath tumor of a previous neurofibroma.

Conclusions:

malignant peripheral nerve sheath tumors are weird neoplasias and even more its proximal location. The presented patient despite the radical surgical resection associated to radiotherapy, the size superior to ten centimeters, the association to neurofibromatosis type 1 and the high histological degree, worsen the forecast. The precocious diagnosis and accurate treatment are important.

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