0
Images in Neurology |

Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1

John P. Rossiter, MB, BCh, PhD1; Paul V. Fenton, MD2
[+] Author Affiliations
1Department of Pathology and Molecular Medicine, Queen’s University and Kingston General Hospital, Kingston, Ontario, Canada
2Department of Diagnostic Radiology, Queen’s University and Kingston General Hospital, Kingston, Ontario, Canada
JAMA Neurol. 2014;71(2):242. doi:10.1001/jamaneurol.2013.2048.
Text Size: A A A
Published online

Extract

A young adult man with neurofibromatosis type 1 (NF1) presented with a several-month history of an increasingly painful mass in the posteroinferior region of the right thigh. Imaging studies (Figure) showed a relatively well-circumscribed mass, 15 cm in length and 7 cm in diameter, arising on a background of very extensive involvement of the peripheral nervous system by plexiform neurofibromas. A biopsy specimen of the mass showed a high-grade (World Health Organization grade IV) malignant peripheral nerve sheath tumor (MPNST). An above-knee amputation was subsequently performed and the MPNST (Figure, inset A) was found to arise in continuity with one of the many preexisting low-grade (World Health Organization grade I) plexiform neurofibromas (Figure, inset B shows an example of such in the leg). For approximately 3.5 years following surgery and radiotherapy, there was no evidence of local tumor recurrence. The patient then was seen with a large left-sided retroperitoneal mass that was histopathologically confirmed to also be an MPNST, possibly a metastasis from the original thigh tumor but more likely a de novo primary tumor. He subsequently underwent several major debulking surgeries, radiotherapy, and rounds of chemotherapy, including palliative doxorubicin, for increasingly disseminated metastatic disease. He died approximately 7 years after his initial presentation.

Figures in this Article

Topics

Sign In to Access Full Content

Don't have Access?

Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more

Subscribe for full-text access to content from 1998 forward and a host of useful features

Activate your current subscription (AMA members and current subscribers)

Purchase Online Access to this article for 24 hours

First Page Preview

View Large
/>
First page PDF preview

Figures

Place holder to copy figure label and caption
Figure.
Coronal T1-Weighted Magnetic Resonance Images of the Pelvis/Upper Thighs and the Lower Thighs/Legs Show Extensive Bilateral Plexiform Neurofibromas and a 15 × 7 cm Mass in the Lower Right Thigh

(The upper and lower magnetic resonance images are from closely adjacent, but not identical, coronal planes. This is because the plexiform neurofibromas in the pelvis and upper thighs are more clearly demonstrated in the slice shown. As a consequence of the different planes, there is a partial mismatch in the alignment of the thighs between the 2 images and the lower image has been slightly reduced in size to minimize this.) Microscopic image A shows a representative area of the highly cellular and mitotically active malignant peripheral nerve sheath tumor and image B shows low-density spindle-shaped tumor cells in a stroma comprising bundles of wavy collagen fibers, characteristic of neurofibroma (insets A and B, hematoxylin phloxine saffron, original magnification ×40).

Graphic Jump Location

Tables

References

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

Related Content

Customize your page view by dragging & repositioning the boxes below.

Related Topics
Jobs
brightcove.createExperiences();