title: Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass.
abstract: A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.
authors.affiliation: Department of Oncology/Hematology, Kyungpook National University Hospital, Daegu, Korea.
authors.forename: Jong Gwang
authors.initials: JG
authors.lastname: Kim
authors.forename: Woo Jin
authors.initials: WJ
authors.lastname: Sung
authors.forename: Dong Hwan
authors.initials: DH
authors.lastname: Kim
authors.forename: Young Hwan
authors.initials: YH
authors.lastname: Kim
authors.forename: Sang Kyun
authors.initials: SK
authors.lastname: Sohn
authors.forename: Kyu Bo
authors.initials: KB
authors.lastname: Lee
mesh_terms: D000008:Abdominal Neoplasms / Q000175:diagnosis*; D000328:Adult; D005260:Female; D006801:Humans; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis*; D009456:Neurofibromatosis 1 / Q000150:complications*; D013899:Thoracic Neoplasms / Q000175:diagnosis*