Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed?
Malignant peripheral nerve sheath tumors (MPNSTs) are rare but aggressive neoplasms associated with neurofibromatosis type 1. Specifically, children with deep plexiform neurofibromas are 18 times more likely to develop MPNSTs compared to the general population. However, there is currently no standar...
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| Format: | Article |
| Language: | English English |
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Elsevier
2022
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| Online Access: | https://eprints.ums.edu.my/id/eprint/33806/1/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%20.pdf https://eprints.ums.edu.my/id/eprint/33806/2/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%201.pdf |
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| author | Siti Nurhazwani Kamaludin Marlina Yusuf Warren Erwin Nicholas Aaron Paul Yong, Guang Teh |
| author_facet | Siti Nurhazwani Kamaludin Marlina Yusuf Warren Erwin Nicholas Aaron Paul Yong, Guang Teh |
| author_sort | Siti Nurhazwani Kamaludin |
| collection | UMS |
| description | Malignant peripheral nerve sheath tumors (MPNSTs) are rare but aggressive neoplasms associated with neurofibromatosis type 1. Specifically, children with deep plexiform neurofibromas are 18 times more likely to develop MPNSTs compared to the general population. However, there is currently no standard surveillance imaging protocol for children diagnosed with deep plexiform neurofibromatosis. We present a case of a boy with neurofibromatosis type 1 and scoliosis, who later developed MPNST. This case highlights the need for more frequent surveillance imaging and the challenges of diagnosing MPNST in a patient with scoliosis. In order to facilitate early detection of malignant transformation, we suggest annual surveillance MR imaging for patients known to have deep plexiform neurofibromatosis. |
| first_indexed | 2024-03-06T03:19:15Z |
| format | Article |
| id | ums.eprints-33806 |
| institution | Universiti Malaysia Sabah |
| language | English English |
| last_indexed | 2024-03-06T03:19:15Z |
| publishDate | 2022 |
| publisher | Elsevier |
| record_format | dspace |
| spelling | ums.eprints-338062022-08-16T07:30:55Z https://eprints.ums.edu.my/id/eprint/33806/ Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? Siti Nurhazwani Kamaludin Marlina Yusuf Warren Erwin Nicholas Aaron Paul Yong, Guang Teh RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens Malignant peripheral nerve sheath tumors (MPNSTs) are rare but aggressive neoplasms associated with neurofibromatosis type 1. Specifically, children with deep plexiform neurofibromas are 18 times more likely to develop MPNSTs compared to the general population. However, there is currently no standard surveillance imaging protocol for children diagnosed with deep plexiform neurofibromatosis. We present a case of a boy with neurofibromatosis type 1 and scoliosis, who later developed MPNST. This case highlights the need for more frequent surveillance imaging and the challenges of diagnosing MPNST in a patient with scoliosis. In order to facilitate early detection of malignant transformation, we suggest annual surveillance MR imaging for patients known to have deep plexiform neurofibromatosis. Elsevier 2022 Article PeerReviewed text en https://eprints.ums.edu.my/id/eprint/33806/1/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%20.pdf text en https://eprints.ums.edu.my/id/eprint/33806/2/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%201.pdf Siti Nurhazwani Kamaludin and Marlina Yusuf and Warren Erwin Nicholas and Aaron Paul and Yong, Guang Teh (2022) Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? Radiology Case Reports, 17. pp. 2388-2393. ISSN 1930-0433 https://www.sciencedirect.com/science/article/pii/S1930043322002837 https://doi.org/10.1016/j.radcr.2022.03.111 https://doi.org/10.1016/j.radcr.2022.03.111 |
| spellingShingle | RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens Siti Nurhazwani Kamaludin Marlina Yusuf Warren Erwin Nicholas Aaron Paul Yong, Guang Teh Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? |
| title | Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? |
| title_full | Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? |
| title_fullStr | Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? |
| title_full_unstemmed | Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? |
| title_short | Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed? |
| title_sort | plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis more surveillance imaging needed |
| topic | RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens |
| url | https://eprints.ums.edu.my/id/eprint/33806/1/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%20.pdf https://eprints.ums.edu.my/id/eprint/33806/2/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%201.pdf |
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