PDF(Pubmed)
Abstract：
Schwannomatosis is a rare autosomal dominant hereditary syndrome disease characterized by multiple schwannomas throughout the body, without bilateral vestibular schwannoma or dermal schwannoma. The most common location of schwannomatosis is the head and neck, as well as the limbs, while multiple schwannomas in the lumbosacral canal and lower extremities are relatively rare. In this study, we report a 79-year-old woman diagnosed with schwannomatosis. MRI and contrast-enhanced imaging revealed multiple schwannomas in both lower extremities. An 18F-FDG PET/CT examination revealed that in addition to multiple tumors with increased 18F-FDG uptake in both lower extremities, there was also an increased 18F-FDG uptake in a mass in the lumbosacral canal. These masses were confirmed to be schwannomas by pathology after surgery or biopsy. 18F-FDG PET/CT findings of schwannomas were correlated with MRI and pathological components. Antoni A area rich in tumor cells showed significant enhancement on contrast-enhanced T1WI, and PET/CT showed increased uptake of 18F-FDG in the corresponding area, while Antoni B region rich in mucus showed low enhancement on contrast-enhanced T1WI, accompanied by a mildly increased 18F-FDG uptake.
摘要：
神经鞘瘤病是一种罕见的常染色体显性遗传综合征疾病，其特征是全身多发性神经鞘瘤，没有双侧前庭神经鞘瘤或真皮神经鞘瘤。神经鞘瘤病最常见的部位是头颈部,以及四肢，而腰骶管和下肢的多发性神经鞘瘤相对罕见。在这项研究中,我们报告了一名79岁的女性，被诊断患有神经鞘瘤病。MRI和对比增强成像显示下肢多发神经鞘瘤。18F-FDGPET/CT检查显示，除了两个下肢18F-FDG摄取增加的多个肿瘤外，腰骶管肿块中18F-FDG的摄取也增加。手术或活检后病理证实这些肿块为神经鞘瘤。神经鞘瘤的18F-FDGPET/CT表现与MRI和病理成分相关。AntoniA区富含肿瘤细胞，在对比增强的T1WI上有显著增强，PET/CT显示相应区域18F-FDG的摄取增加，而富含粘液的安东尼B区在对比增强的T1WI上显示出低增强，伴随着轻度增加的18F-FDG摄取。
