Intrathoracic Malignant Peripheral Nerve Sheath Tumors: Imaging Features and Implications for Management

Authors

  • Sophia C Kamran Department of Imaging, Dana-Farber Cancer Institute, 450 Brookline Ave, Boston, MA 02115 Department of Radiology, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA 02115 Harvard Medical School, Boston MA 02115
  • Atul Bhanudas Shinagare
  • Stephanie Anne Holler Howard
  • Mizuki Nishino
  • Jason Laurence Hornick
  • Katherine Margaret Krajewski
  • Nikhil Himmatsinh Ramaiya

Abstract

Purpose: To study the clinical and imaging characteristics of primary intrathoracic malignant peripheral nerve sheath tumors (MPNSTs).

Materials and Methods: In this 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 FDG avid [mean SUVmax 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.

Conclusion: 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.

Author Biographies

Atul Bhanudas Shinagare

Stephanie Anne Holler Howard

Mizuki Nishino

Jason Laurence Hornick

Katherine Margaret Krajewski

Nikhil Himmatsinh Ramaiya

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Published

2013-08-27

How to Cite

Kamran, S. C., Shinagare, A. B., Holler Howard, S. A., Nishino, M., Hornick, J. L., Krajewski, K. M., & Ramaiya, N. H. (2013). Intrathoracic Malignant Peripheral Nerve Sheath Tumors: Imaging Features and Implications for Management. Radiology and Oncology, 47(3). Retrieved from https://www.radioloncol.com/index.php/ro/article/view/1516

Issue

Section

Radiology