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Peripheral nerve sheath tumor (PNST) containing a glandular component, commonly referred to as glandular PNST, represents a rare entity found largely in patients with von Recklinghausen disease. As described in one recent review,[1] most glandular PNSTs are malignant, consisting of a background of spindle cells indistinguishable from that seen in the typical malignant PNST.
Based on these findings, a clinical diagnosis of von Recklinghausen disease was made.
In 1892, Garre[3] described a patient with von Recklinghausen disease and a malignant PNST sciatic nerve.