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.
Shimizu
Katsuhiko
K
Department of Surgical Oncology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan.
Okita
Riki
R
Uchida
Yoko
Y
Hihara
Jun
J
D000328:Adult; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary / Q000601:surgery*; D008297:Male; D018317:Nerve Sheath Neoplasms / Q000556:secondary / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000473:pathology / Q000601:surgery*; D011013:Pneumonectomy; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome