Ren Jiazhong, Yang Guoren, Zhou Jing, Fu Zheng
Department of Radiology, Jining No. 1 People's Hospital, Jining Shandong Department of Nuclear Medicine Department of Radiotherapy Department of Imaging, Shandong Cancer Hospital Affiliated to Shandong University Shandong Academy of Medical Sciences, Jinan, People's Republic of China.
Medicine (Baltimore). 2018 May;97(20):e10648. doi: 10.1097/MD.0000000000010648.
Neurofibromatosis type one (NF1) is characterized by cutaneous and nervous lesions, and the tendency to form plexiform neurofibromas (PNFs). PNFs may undergo malignant transformation into a malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs often carry an significant morbidity and mortality.
A 17-year-old man with gradually increased multiple subcutaneous soft lesions. He also presented with numerous lentigines and multiple café-au-lait macules on his body.
These were collagen neurofibroma, which were definitively diagnosed by pathology. NF1 was eventually diagnosed.
These lesions were abnormal uptake of radiotracer, when he underwent positron emission tomography (PET) with fluorine-18-fluorodeoxyglucose (FDG) scanning. Standard uptake value (SUV) and other parameters can help to distinguish benign and malignant lesions in patient with NF1. He was underwent serials F-FDG PET/CT examinations to followed up, in order to monitor these lesions malignant transformation.
So far, these subcutaneous soft lesions were not malignant transformation.
F-FDG PET/CT is being increasingly used as an imaging modality to discover the systemic lesions and to discriminate between benign and malignant plexiform neurofibromas.
1型神经纤维瘤病（NF1）的特征是皮肤和神经病变，以及形成丛状神经纤维瘤（PNF）的倾向。PNF可能会恶变成为恶性外周神经鞘瘤（MPNST）。MPNST通常具有较高的发病率和死亡率。
一名17岁男性，多发性皮下软组织肿物逐渐增多。他身上还出现了大量雀斑样痣和多个咖啡牛奶斑。
这些病变经病理确诊为胶原性神经纤维瘤。最终确诊为NF1。
当他接受18F-氟脱氧葡萄糖（FDG）正电子发射断层扫描（PET）时，这些病变表现为放射性示踪剂摄取异常。标准摄取值（SUV）和其他参数有助于区分NF1患者的良性和恶性病变。他接受了一系列F-FDG PET/CT检查进行随访，以监测这些病变的恶变情况。
到目前为止，这些皮下软组织肿物尚未恶变。
F-FDG PET/CT越来越多地被用作一种成像方式，以发现全身病变并区分良性和恶性丛状神经纤维瘤。
