Long Survival after Resection for Lung Metastasis of Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis 1

A 31-year-old man with neurofibromatosis 1 (NF1) was admitted for the treatment of solitary lung tumor. Nine months earlier he had undergone a large resection for malignant peripheral nerve sheath tumors (MPNSTs) in his back. Surgical resection of the right lower lobe was performed, and the tumor was pathologically diagnosed as a metastasis of MPNST. The survival of patients with pulmonary metastasis of MPNST is extremely poor, especially of those with NF1, but this patient has survived 5 years without recurrence. Based on our knowledge of the literature, a 5-year survival is extremely rare, and select patients have benefited from a resection of pulmonary metastasis. (Ann Thorac Cardiovasc Surg 2008; 14:322-324)