title: An intraosseous malignant peripheral nerve sheath tumor of the lumbar spine without neurofibromatosis: Case report and review of the literature.
abstract: A malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor that develops or differentiates from cells in the peripheral nerve sheath. This tumor is commonly associated with neurofibromatosis type 1 (NF1) and previous radiotherapy treatment. Primary intraosseous MPNSTs are extremely rare and a case of the lumbar spine in a patient without NF1 is reported in the present study, with a review of the intraosseous MPNST literature. A 45-year-old female presented with a 1-month history of severe lower back pain and pain radiating to the left leg. A totalen blocspondylectomy of L4 was performed. The postoperative histopathological diagnosis was MPNST with deletion ofNF1, confirmed by dual-color fluorescencein situhybridization (FISH) analysis. The tumor recurred 1 month following the surgery. Although adjuvant chemotherapy was administered, the patient succumbed due to intramedullary dissemination and carcinomatous meningitis 8 months following the initial consultation.NF1deletion by FISH analysis may be particularly useful in distinguishing MPNST from other high-grade malignancies with overlapping morphological features.
authors.affiliation: Department of Orthopaedic Surgery, Faculty of Medicine, University of Toyama, Toyama City, Toyoma 939-0194, Japan.
authors.forename: Kayo
authors.initials: K
authors.lastname: Suzuki
authors.affiliation: Department of Orthopaedic Surgery, Faculty of Medicine, University of Toyama, Toyama City, Toyoma 939-0194, Japan.
authors.forename: Taketoshi
authors.initials: T
authors.lastname: Yasuda
authors.affiliation: Department of Orthopaedic Surgery, Iiyama Red Cross Hospital, Iiyama City, Nagano 389-2295, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Hori
authors.affiliation: Department of Orthopaedic Surgery, Faculty of Medicine, University of Toyama, Toyama City, Toyoma 939-0194, Japan.
authors.forename: Kenta
authors.initials: K
authors.lastname: Watanabe
authors.affiliation: First Department of Human Science, Faculty of Medicine, University of Toyama, Toyama City, Toyoma 939-0194, Japan.
authors.forename: Masahiko
authors.initials: M
authors.lastname: Kanamori
authors.affiliation: Department of Orthopaedic Surgery, Faculty of Medicine, University of Toyama, Toyama City, Toyoma 939-0194, Japan.
authors.forename: Tomoatsu
authors.initials: T
authors.lastname: Kimura
keywords: intraosseous malignant peripheral nerve sheath tumor; spine; total en bloc spondylectomy