Radical resection of bronchoscopically diagnosed lung metastasis from recurrent malignant peripheral never sheath tumor on the back

Abstract

　Neurofibromatosis Type I (NF1) is known to cause multiple neurofibromas, however it rarely causes malignant peripheral nerve sheath tumor (MPNST). We experienced a patient with MPNST and single lung metastasis, therefore, I report this case. A 71-year-old woman, who had been clinically diagnosed with NF1, developed MPNST in July 2013. She received tumor resection three times (July 2013, March 2017, and March 2018). She was admitted to our hospital because of the enlarging lung tumor over 4 months. Bronchoscopic biopsy revealed the tumor was MPNST. Complete resection of the lung metastasis and the recurrence tumor on the back were performed consecutively.We experienced a case of lung metastasis from MPNST on the back which was diagnosed with bronchoscope and resected completely. As MPNST is resistant to both chemotherapy and radiotherapy, it was suggested that the radical resection of metastasis might be most effective treatment based on our case who has achieved long survival due to the early detection and the repeated operation. Early detection and radical resection of metastatic tumor may attribute to the long survival for the treatment of recurrent MPNST.