The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 601 North Wolfe Street, Baltimore, MD 21287, USA.
Skeletal Radiol. 2013 Sep;42(9):1317-22. doi: 10.1007/s00256-013-1626-3. Epub 2013 May 7.
Neurofibromatosis type 1 (NF1) is a neurocutaneous syndrome characterized by the development of multiple peripheral nerve sheath tumors, the majority of which are benign neurofibromas. However, malignant peripheral nerve sheath tumors (MPNSTs) occur with a 10 % lifetime risk in patients with NF1, often developing within a neurofibroma. When clinical suspicion for an MPNST arises, imaging with FDG PET and MRI is performed to characterize a peripheral tumor for potential malignancy. In this report, we describe a patient with NF-1 who had two peripheral tumors with similar features by PET, both suspicious for MPNST, but differing features by MRI, one of which was subsequently determined to be an MPNST and the second to be a schwannoma.
神经纤维瘤病 1 型(NF1)是一种神经皮肤综合征,其特征是多发性周围神经鞘瘤的发展,其中大多数为良性神经纤维瘤。然而,在 NF1 患者中,恶性外周神经鞘瘤(MPNST)的发生风险为 10%,其常在神经纤维瘤内发生。当临床怀疑为 MPNST 时,会使用 FDG PET 和 MRI 进行成像以对潜在恶性的外周肿瘤进行特征描述。在本报告中,我们描述了一位 NF-1 患者,其两个具有相似 PET 特征的外周肿瘤均疑似为 MPNST,但 MRI 特征不同,其中一个随后被确定为 MPNST,另一个为神经鞘瘤。