Malignant transformation in a sciatic plexiform neurofibroma in Neurofibromatosis Type 1 - imaging features that aid diagnosis

Authors

  • Dorinda Chee Yee Chew
  • Daniel Bin Hang Zhao
  • Kesavan Sittampalam
  • S Krishna Kumar

DOI:

https://doi.org/10.3941/jrcr.v14i12.4028

Keywords:

Malignant peripheral nerve sheath tumour, MPNST, neurofibrosarcoma, neurofibromatosis type 1, NF1, plexiform neurofibroma, malignant transformation, MRI, CT, PET

Abstract

A 41-year-old Asian male with NF1 and bilateral sciatic plexiform neurofibromas, presented with unintentional weight loss, increasing size of a left thigh mass associated with increasing pain and radiculopathy. MRI of the left thigh demonstrated imaging features suspicious of malignant transformation. The patient had a new left lung mass, demonstrating avid FDG uptake, raising suspicion for metastasis. Surgical resection of the left thigh mass confirms malignant transformation in a preexisting sciatic plexiform neurofibroma. Diagnosis of malignant transformation in a nerve sheath tumour can be challenging. MRI remains the main preferred imaging modality in the evaluation of these tumours. Imaging features that raise suspicion for malignant transformation are discussed. Although none of these are specific for malignant transformation, studies suggest that the presence of two to four of these features should prompt further investigations.

Author Biographies

Dorinda Chee Yee Chew

Senior Resident, Radiology

Department of Oncology Imaging, National Cancer Center, Singapore

Daniel Bin Hang Zhao

Principle Radiographer

Department of Oncology Imaging, National Cancer Center, Singapore

Kesavan Sittampalam

Senior Consultant, Pathologist 

Department of Pathology, National Cancer Center, Singapore 

S Krishna Kumar

Consultant, Radiologist

Department of Oncology Imaging, National Cancer Center, Singapore

 

Malignant transformation in a sciatic plexiform neurofibroma in Neurofibromatosis Type 1 - imaging features that aid diagnosis by Dorinda Chee Yee Chew et. al.

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Published

2020-12-26

Issue

Vol. 14 No. 12 (2020)

Section

Neuroradiology

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