Background: Malignant peripheral nerve sheath tumors (MPNSTs) refer to malignant tumors in the cells of the peripheral nerve or nerve sheath. This study was conducted to report the incidence and provide an overview of MPNSTs in Saudi Arabia due to the rarity and lack of studies on MPNSTs in the country.
Methods: This retrospective cohort study of MPNSTs included all patients diagnosed with MPNSTs in Saudi Arabia between 2010 and 2020. The data were collected from the Saudi Cancer Registry and were analyzed using John’s Macintosh Project statistical software.
Results: Twenty-five total with 25 MPNST cases were included in this study. The annual incidence in Saudi Arabia is 0.076 per million people. Of the patients, 15 were males (60.00%), and the median age was 36 (1547) years. In this study, all 25 cases were diagnosed based on histopathology of the primary lesions (100.00%). For the sites of the lesions, the trunk and lower limbs were the most reported sites with six cases (24.00%) each. Regarding tumor grading, the majority had an unknown grade (18 cases, 72.00%), followed by grade II (moderately differentiated) in four cases (16.00%). Finally, for tumor extension, 12 cases (48.00%) were localized, whereas only one case (4.00%) was distally metastasized.
Conclusion: This study revealed a low incidence of MPNSTs in Saudi Arabia, providing information on gender distribution, age, geographical distribution, tumor site, grading, and tumor extension.
Key words: Malignant peripheral nervous sheath tumor, peripheral nerve tumor, peripheral nerve surgery, epidemiology, Saudi Arabia
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