Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect Malignancy
Plexiform neurofibromas are essentially pathognomonic for neurofibromatosis type 1 (NF1), occurring when there is diffuse involvement along a nerve segment and its branches. Transformation into a malignant peripheral nerve sheath tumour (MPNST) is a major cause of mortality in NF1 patients. These tu...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | Case Reports in Radiology |
| Online Access: | http://dx.doi.org/10.1155/2013/493752 |
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| author | Maria Gosein Anthony Ameeral Renee Banfield Murrie Mosodeen |
| author_facet | Maria Gosein Anthony Ameeral Renee Banfield Murrie Mosodeen |
| author_sort | Maria Gosein |
| collection | DOAJ |
| description | Plexiform neurofibromas are essentially pathognomonic for neurofibromatosis type 1 (NF1), occurring when there is diffuse involvement along a nerve segment and its branches. Transformation into a malignant peripheral nerve sheath tumour (MPNST) is a major cause of mortality in NF1 patients. These tumours are highly aggressive and particularly difficult to diagnose in NF1 patients due to the clinical overlap between benign and malignant lesions. We present a case of a plexiform neurofibroma and discuss the typical imaging characteristics on ultrasound, CT, and MRI, including the target sign and continuity with the parent nerve. Certain imaging features should raise suspicion for malignancy however, these modalities may not always reliably differentiate between benign and malignant lesions. Recent studies show a very high negative predictive value for FDG-PET making it quite useful in excluding malignancy. In positive scans, PET/CT aids in guiding biopsy to the most metabolically active area of the tumour. |
| format | Article |
| id | doaj-art-80e1744b3bd247b49545a6d275503dd4 |
| institution | Kabale University |
| issn | 2090-6862 2090-6870 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Radiology |
| spelling | doaj-art-80e1744b3bd247b49545a6d275503dd42025-02-03T05:58:13ZengWileyCase Reports in Radiology2090-68622090-68702013-01-01201310.1155/2013/493752493752Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect MalignancyMaria Gosein0Anthony Ameeral1Renee Banfield2Murrie Mosodeen3San Fernando General Hospital, Independence Avenue, Paradise Pasture, San Fernando, Trinidad and TobagoSan Fernando General Hospital, Independence Avenue, Paradise Pasture, San Fernando, Trinidad and TobagoSan Fernando General Hospital, Independence Avenue, Paradise Pasture, San Fernando, Trinidad and TobagoSan Fernando General Hospital, Independence Avenue, Paradise Pasture, San Fernando, Trinidad and TobagoPlexiform neurofibromas are essentially pathognomonic for neurofibromatosis type 1 (NF1), occurring when there is diffuse involvement along a nerve segment and its branches. Transformation into a malignant peripheral nerve sheath tumour (MPNST) is a major cause of mortality in NF1 patients. These tumours are highly aggressive and particularly difficult to diagnose in NF1 patients due to the clinical overlap between benign and malignant lesions. We present a case of a plexiform neurofibroma and discuss the typical imaging characteristics on ultrasound, CT, and MRI, including the target sign and continuity with the parent nerve. Certain imaging features should raise suspicion for malignancy however, these modalities may not always reliably differentiate between benign and malignant lesions. Recent studies show a very high negative predictive value for FDG-PET making it quite useful in excluding malignancy. In positive scans, PET/CT aids in guiding biopsy to the most metabolically active area of the tumour.http://dx.doi.org/10.1155/2013/493752 |
| spellingShingle | Maria Gosein Anthony Ameeral Renee Banfield Murrie Mosodeen Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect Malignancy Case Reports in Radiology |
| title | Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect Malignancy |
| title_full | Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect Malignancy |
| title_fullStr | Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect Malignancy |
| title_full_unstemmed | Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect Malignancy |
| title_short | Plexiform Neurofibroma of the Wrist: Imaging Features and When to Suspect Malignancy |
| title_sort | plexiform neurofibroma of the wrist imaging features and when to suspect malignancy |
| url | http://dx.doi.org/10.1155/2013/493752 |
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