A Case of Intrathoracic Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I.
- Author:
Young Mi KIM
1
;
So Eun JEON
;
Byung Ki LEE
;
Yoon Jin LEE
;
Sang Ook NAM
;
Young Tak LIM
Author Information
1. Department of Pediatrics, College of Medicine, Pusan National University Hospital, Busan, Korea. pink2129@naver.com
- Publication Type:Case Report
- Keywords:
Malignant peripheral nerve sheath tumors;
Neurofibromatosis 1;
Intrathoracic
- MeSH:
Adolescent;
Cough;
Dyspnea;
Head;
Humans;
Lower Extremity;
Neck;
Nerve Sheath Neoplasms;
Neurofibromatoses;
Neurofibromatosis 1;
Peripheral Nerves
- From:
Journal of the Korean Child Neurology Society
2011;19(2):165-168
- CountryRepublic of Korea
- Language:Korean
-
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.
