title: Intraneural perineurioma involving the ulnar nerve.
abstract: Intraneural perineurioma is a rare peripheral nerve sheath tumor consisting of intraneural proliferation of neoplastic perineurial cells. Clinical and pathological findings of a perineurioma involving the ulnar nerve is presented. A 7-year-old girl presented with a 2 year history of weakness and atrophy of the right hand muscles. Physical examination and imaging study revealed a pea-sized tumor in the ulnar side of the right forearm. At surgery, a fusiform swelling of the ulnar nerve was found and an excisional biopsy of the lesion was performed. Light microscopy revealed numerous whorls consisting of concentric layers of spindle cells encircling the nerve fibers. The proliferating cells were immunoreactive for vimentin, epithelial membrane antigen and glucose transporter protein 1 (Glut1), but negative for S-100 protein and CD34. Ultrastructural examination revealed features of perineurial cell differentiation. The current study suggests that Glut1 is a useful marker of intraneural perineurioma.
authors.affiliation: Department of Pathology, Okayama University Hospital, Okayama, Japan. shamazak@cc.okayama-u.ac.jp
authors.forename: Shuji
authors.initials: S
authors.lastname: Hamazaki
authors.forename: Kazuo
authors.initials: K
authors.lastname: Fujiwara
authors.forename: Shigeru
authors.initials: S
authors.lastname: Okada
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism*; D002648:Child; D005260:Female; D005542:Forearm / Q000473:pathology; D051272:Glucose Transporter Type 1; D006801:Humans; D007150:Immunohistochemistry; D008854:Microscopy, Electron; D009004:Monosaccharide Transport Proteins / Q000378:metabolism*; D018317:Nerve Sheath Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D010524:Peripheral Nervous System Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D014459:Ulnar Nerve / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D051272:Glucose Transporter Type 1; D009004:Monosaccharide Transport Proteins; C495677:SLC2A1 protein, human