title: Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management.
abstract: BACKGROUND
  The aim of the study was to analyze the clinical and imaging characteristics of primary intrathoracic malignant peripheral nerve sheath tumors (MPNSTs).
  
  
  METHODS
  In this institutional review board (IRB)-approved retrospective study, clinical and imaging features of 15 patients (eight men; mean age 50 years [range 18-83)] with pathologically proven malignant peripheral nerve sheath tumors seen from January 1999 to December 2011 were analyzed. Imaging features (CT in 15, MRI in 5 and PET/CT in 4) of primary tumors were evaluated by three radiologists and correlated with clinical management.
  
  
  RESULTS
  Of the 15 tumors, six were located in the mediastinum (two each in anterior, middle and posterior mediastinum), four in chest wall, two were paraspinal, and three in the lung. Four patients had neurofibromatosis-1 (NF1); four tumors had heterologous rhabdomyoblastic differentiation (malignant triton tumor). Masses typically were elongated along the direction of nerves, with mean size of 11 cm. The masses were hypo- or isodense to muscles on CT, isointense on T1-weighted images, hyperintense on T2-weighted images and intensely fluorodeoxyglucose (FDG) avid (mean standardized uptake value [SUV]max of 10.5 [range 4.4-23.6]). Necrosis and calcification was seen in four tumors each. Finding of invasion of adjacent structures on imaging led to change in management in seven patients; patients with invasion received chemoradiation.
  
  
  CONCLUSIONS
  Intrathoracic MPNSTs appear as large elongated masses involving mediastinum, lung or chest wall. Radiological identification of invasion of adjacent structures is crucial and alters therapy, with patients with invasion receiving neoadjuvant or adjuvant chemoradiation.
authors.affiliation: Department of Imaging, Dana-Farber Cancer Institute, 450 Brookline Ave, Boston, MA 02115, USA ; Department of Radiology, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA ; Harvard Medical School, Boston MA 02115, USA.
authors.forename: Sophia Constance
authors.initials: SC
authors.lastname: Kamran
authors.forename: Atul Bhanudas
authors.initials: AB
authors.lastname: Shinagare
authors.forename: Stephanie Anne Holler
authors.initials: SA
authors.lastname: Howard
authors.forename: Mizuki
authors.initials: M
authors.lastname: Nishino
authors.forename: Jason Laurence
authors.initials: JL
authors.lastname: Hornick
authors.forename: Katherine Margaret
authors.initials: KM
authors.lastname: Krajewski
authors.forename: Nikhil Himmatsinh
authors.initials: NH
authors.lastname: Ramaiya
keywords: chest; imaging; malignant peripheral nerve sheath tumors; malignant triton tumor; neurofibromatosis