A Malignant Peripheral Nerve Sheath Tumor of The Posterior Mediastinum in A Patient with Neurofibromatosis Type 1 : A Case Report
Keywords:
Malignant peripheral nerve sheath tumor, Neurofibromatosis type 1, Von Recklinghausen’s disease.Abstract
Background and Objective : Malignant peripheral nerve sheath tumor (MPNST) is rare, but it commonly associates with neurofibromatosis type1 (NF1). The incidence of MPNST is 0.001% in general population and 0.16% in patients with NF1. NF1 is one of the most significant prognostic factors indicating poor outcome with chemotherapy against MPNST. The prognosis is worse when MPNST appears in patients with NF1 (five year survival rate 16-23% in comparison to 47-53% in patients without NF1.
Material and Method : A-40-year-old female patient presenting with dyspnea for 2 months was reported in this article. Computed tomography (CT) of chest showed a huge mass occupying the left upper thorax and erosion of posterior left 4th rib.
Result : A thoracotomy and tumor excision was performed and MPNST was finally diagnosed based on immunohistological findings. This case report seriously shows the importance of carefully monitoring patients with NF1 because of the increasing risk of the promoting of malignant neoplasms.
Conclusion : MPNST is usually occured in association with neurofibromatosis type 1. The prognosis is worse when MPNST appears in patients with NF1 (five year survival rate 16-23% in comparison to 47-53% in patients without NF1)
