Mediastinum
Soft tissue tumors
Malignant peripheral nerve sheath tumor
Author: Hanni Gulwani, M.B.B.S.
Topic Completed: 1 December 2012
Minor changes: 10 June 2020
Copyright: 2003-2019, PathologyOutlines.com, Inc.
PubMed Search: Malignant peripheral nerve sheath tumor mediastinum[TI]
Table of Contents
Clinical features | Prognostic factors | Case reports | Gross description | Microscopic (histologic) description | Microscopic (histologic) imagesCite this page: Gulwani H. Malignant peripheral nerve sheath tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mediastinumMPNST.html. Accessed January 17th, 2021.
Clinical features
- Often associated with neurofibromatosis
- 4% of MPNST show perineurial cell differentiation (Am J Surg Pathol 1998;22:1368)
- Often spreads to pleura or lungs
Prognostic factors
- Extremely poor prognosis if glandular or rhabdomyosarcomatous features
Case reports
- 50 year old man with 13 cm tumor (Singapore Med J 2009;50:e199)
- 51 year old man (Respiration 2000;67:346)
- 56 year old man with neurofibromatosis type 1 (Surg Today 2008;38:945)
Gross description
- Unencapsulated, degenerative changes common (fat, hemorrhage, cysts)
Microscopic (histologic) description
- Bizarre cells and tumor giant cells
- Also atypical mitotic figures and necrosis
- May have areas of uniform bland spindle cells
- Rarely has rhabdomyosarcomatous features ("triton tumor") or glandular differentiation
Microscopic (histologic) images
Images hosted on other servers:
Low grade tumor has
increased cellularity,
nuclear atypia, scattered
mitotic figures
Fascicular proliferation of atypical spindle cells
