A Case of Intrathoracic Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I / 대한소아신경학회지

ABSTRACT

Malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm and the main cause of the mortality in neurofibromatosis type 1 (NF 1). MPNSTs have been found mostly in the head and neck and the upper or lower extremities with intrathoracic MPNSTs being uncommon. PET has been a useful diagnostic modality of MPNSTs in NF 1. We present a 17-year-old girl patient with NF 1. She was admitted with chronic cough and shortness of breath caused by a huge mediastinal mass. An 18FDG-PET study revealed intense uptake at the mediastinal mass. She underwent surgery to lessen respiratory symptoms, and the mass was histologically diagnosed as an intrathoracic MPNST.