제 1형 신경섬유종증에 동반된 흉강 내악성 말초신경초종 1례

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

악성 말초신경초종은 드문 악성 종양이나 신경섬유종증 제 1형 환자에서 호발하며, 말초신경이나 심부에 발생한 신경섬유종에서 발생할 수 있다. 18FDG-PET는 신경섬유종증 제 1형 환자에서 발생한 악성말초신경초종을 진단하는데 유용하다. 저자들은 신경섬유종증 제 1형을 가진 17세 여아에서 18FDG-PET 등의 검사를 사용하여 흉강 내에 발생한 악성말초신경초종을 진단하고 치료한 경험을 보고하는 바이다.

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.