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  • Title: Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass.
    Author: Kim JG, Sung WJ, Kim DH, Kim YH, Sohn SK, Lee KB.
    Journal: Korean J Intern Med; 2005 Mar; 20(1):100-4. PubMed ID: 15906964.
    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.
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