Malignant peripheral nerve sheath tumors (MPNST) are defined as any tumor arising from a peripheral nerve or showing nerve sheath differentiation. The majority of these tumors arise on the trunk, extremities, or head and neck region. The literature to date has fewer than 14 cases of MPNST arising in the gastrointestinal tract, and only two cases were ever reported in the small intestine, one of which was a recurrent disease. In this paper, we report the first US case of an MPNST arising in the small intestine and presenting as intussusception.
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