A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: Report of a case
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Malignant peripheral nerve sheath tumor (MPNST) is rare, but its association with neurofibromatosis type 1 (NF1) is well known. A 56-year-old man with NF1 was referred to our hospital for investigation of dyspnea. Computed tomography showed a huge mass occupying the right thorax and invading the inferior vena cava. A salvage resection was performed to alleviate the developing hypoxemia. Histological examination confirmed an MPNST. The tumor regrew rapidly and the patient died 6 weeks after the resection. This case report reinforces the importance of monitoring patients with NF1 carefully because of their increased risk of the development of malignant neoplasms.
Key wordsMalignant peripheral nerve sheath tumor Neurofibromatosis type 1 Von Recklinghausen’s disease Mediastinal tumor
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- 2.DeVita VT Jr, Hellman S, Rosenberg SA, editors. Cancer; principles & practice of oncology. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2001.Google Scholar
- 7.Pediatric malignant peripheral nerve sheath tumor: the Italian and German Soft Tissue Sarcoma Cooperative Group. J Clin Oncol 2005;23:8422–8430.Google Scholar