Clinically, MPNSTs affect young to middle-aged adults, with a slight female predominance. Neurofibromatosis type 1 (NF 1) predispose to the development of MPNSTs. In addition to hereditary factors, exposure to ionizing radiation may also play a role in the development of MPNSTs14 . These tumors are aggressive, locally invasive, and highly metastatic, and cause a variety of symptoms depending on their location and size. Hemothorax is a common complication of MPNSTs because of the characteristic feature of intratumoral hemorrhage and necrosis15,16 .
Complete surgical removal of the tumor is the optimal treatment goal, with adjuvant radiotherapy for invasive and large tumors, as our case. Wide en bloc is the treatment of choice for tumors involving soft tissue3 . Local recurrence rate varied from 40% to 68%2,3,17 . Postoperative radiation therapy of MPNST has led to a significant reduction of local recurrence18 . Combination chemotherapy is recommended for unresectable tumors19 .
The prognosis of patients with MPNST is unfavorable. In 2 major studies with long term follow-up, reported overall 5-year survival rates were 34-52%2,3 . The factors that appear to affect the prognosis of patients with MPNST are tumor location, size, histologic subtype,. Tumor grade, molecular genetics, completeness of resection, recurrence, metastasis and presence of NF1. Reported mean and median ages at death for patients with NF1 are 54.1 and 59 years, respectively, compared with 70.1 and 74 years for the general population. People with NF1 are prone to the development of other disease, and 1.2 times more likely to have malignant neoplasm at the time of death than people without NF11,20 . Thus, a careful follow up plan should be inplace for patients with NF1 to allow for the early diagnosis of MPNST and other neoplasms. A large tumor (> 5 cm), association with NF1, and incomplete resection have been reported as poor prognostic factors2 .
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