The hypocellular area is composed of loosely arranged tumor cells and abundant myxoid stroma.[28] Pancreatic schwannomas demonstrate strong positive immunohistochemical staining for protein S-100 and negative staining for SMA, CD34, CD117, desmin, and smooth muscle myosin.[38,89] Most pancreatic schwannomas are benign and only five malignant cases have been reported so far.[44,79,81–83]. Here, SMN1 is linked to neoplasm.