Plexiform neurofibromatosis with peripheral malignant nerve sheath tumor and scoliosis - more surveillance imaging needed?

• Main Authors: Siti Nurhazwani Kamaludin, Marlina Yusuf, Warren Erwin Nicholas, Paul, Aaron, Teh, Yong Guang
• Format: Article
• Language: English; English
• Published: Elsevier 2022
• Subjects: RC254-282 Neoplasms. Tumors. Oncology Including cancer and carcinogens
• Online Access: https://eprints.ums.edu.my/id/eprint/33757/1/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%20-%20more%20surveillance%20imaging%20needed.pdf; https://eprints.ums.edu.my/id/eprint/33757/2/Plexiform%20neurofibromatosis%20with%20peripheral%20malignant%20nerve%20sheath%20tumor%20and%20scoliosis%20-%20more%20surveillance%20imaging%20needed%20_ABSTRACT.pdf; https://eprints.ums.edu.my/id/eprint/33757/; https://www.sciencedirect.com/science/article/pii/S1930043322002837?via%3Dihub; https://doi.org/10.1016/j.radcr.2022.03.111
• Institution: Universiti Malaysia Sabah

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...