Division of Neurology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada.
Eur J Nucl Med Mol Imaging. 2010 Jul;37(7):1309-17. doi: 10.1007/s00259-010-1386-4. Epub 2010 Feb 24.
There is little consensus regarding optimal surveillance of optic pathway glioma (OPG) and plexiform neurofibroma (PNF) in childhood neurofibromatosis type 1 (NF1). (18)F-2-Fluoro-2-deoxy-D: -glucose (FDG) positron emission tomography and computed tomography (PET/CT) is employed in the surveillance of adult PNFs; but its utility has neither been specifically studied in children with PNFs nor in children with OPG.
Review of PET/CT studies was performed in NF1 children with OPG or PNF. FDG-avidity of tumours was semi-quantitatively analysed and graded by calculating the maximum standardised uptake value (SUV(max)) [grade 1: <3 (low), grade 2: >3-<4 (intermediate), grade 3: >4 (intense)].
Eighteen children (ten girls; median age: 8.5-years) had PET/CT. Nineteen OPGs were imaged. The SUV(max) could be measured in 16. Ten were grade 1 and three each were grade 2 and grade 3. FDG-avidity reduced from grade 3 to grade 1 in two symptomatic OPGs following chemotherapy and this was associated with clinical improvement. PET/CT diagnosed symptomatic OPGs with a sensitivity of 0.625 [95% confidence interval (CI): 0.259-0.897] and specificity of 0.875 (95% CI: 0.466-0.993). Sixteen PNFs were imaged. Twelve were grade 1 and two each were grade 2 and grade 3. The two grade 3 PNFs were confirmed malignant peripheral nerve sheath tumours. PET/CT diagnosed malignant transformation with a sensitivity of 1.0 (95% CI: 0.197-1.0) and specificity of 0.857 (95% CI: 0.561-0.974).
PET/CT may contribute useful information to the surveillance of OPG in childhood NF1-particularly to identify progressive, symptomatic tumours. As in adults, PET/CT is useful for the detection of malignant transformation in PNFs in children with NF1.
在 1 型神经纤维瘤病（NF1）儿童中，视神经胶质瘤（OPG）和丛状神经纤维瘤（PNF）的最佳监测方法尚未达成共识。（18）F-2-氟-2-脱氧-D：-葡萄糖（FDG）正电子发射断层扫描和计算机断层扫描（PET/CT）用于成人 PNF 的监测；但尚未专门研究其在儿童 PNF 或 OPG 中的应用。
对 NF1 合并 OPG 或 PNF 的儿童进行 PET/CT 研究回顾。通过计算最大标准化摄取值（SUV(max)）[1 级：<3（低），2 级：>3-<4（中），3 级：>4（高）]，对肿瘤的 FDG 摄取进行半定量分析和分级。
18 名儿童（10 名女孩；中位年龄：8.5 岁）进行了 PET/CT 检查。共成像 19 个 OPG。可测量 16 个 OPG 的 SUV(max)。10 个为 1 级，3 个分别为 2 级和 3 级。在 2 例接受化疗的有症状 OPG 中，FDG 摄取从 3 级降至 1 级，与临床改善相关。PET/CT 诊断有症状 OPG 的敏感性为 0.625（95%CI：0.259-0.897），特异性为 0.875（95%CI：0.466-0.993）。共成像 16 个 PNF。12 个为 1 级，2 个分别为 2 级和 3 级。2 个 3 级 PNF 被确认为恶性外周神经鞘肿瘤。PET/CT 诊断恶性转化的敏感性为 1.0（95%CI：0.197-1.0），特异性为 0.857（95%CI：0.561-0.974）。
PET/CT 可能为儿童 NF1 中 OPG 的监测提供有用信息-特别是识别进行性、有症状的肿瘤。与成人一样，PET/CT 对 NF1 儿童 PNF 恶性转化的检测有用。
