Department of Radiology, Mount Auburn Hospital, Cambridge, USA.
Department of Radiology, Boston Children's Hospital, Boston, USA.
Pediatr Blood Cancer. 2022 Aug;69(8):e29740. doi: 10.1002/pbc.29740. Epub 2022 Apr 29.
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors in childhood. Up to 40% of PPGL are currently thought to be associated with a hereditary predisposition. Nuclear medicine imaging modalities such as fluorodeoxyglucose positron emission tomography ( F-FDG PET), Ga-DOTATATE PET, and I-metaiodobenzylguanidine ( I-MIBG) scintigraphy play an essential role in the staging, response assessment, and determination of suitability for targeted radiotherapy in patients with PPGL. Each of these functional imaging modalities targets a different cellular characteristic and as such can be complementary to anatomic imaging and to each other. With the recent US Food and Drug Administration approval and increasing use of Ga-DOTATATE for imaging in children, the purpose of this article is to use a case-based approach to highlight both the advantages and limitations of DOTATATE imaging as it is compared to current radiologic imaging techniques in the staging and response assessment of pediatric PPGL, as well as other neuroendocrine malignancies.
嗜铬细胞瘤和副神经节瘤（PPGL）是儿童期罕见的神经内分泌肿瘤。目前认为多达 40%的 PPGL 与遗传易感性有关。核医学成像方式，如氟脱氧葡萄糖正电子发射断层扫描（F-FDG PET）、镓- DOTATATE PET 和碘-间碘苄胍（I-MIBG）闪烁显像，在 PPGL 患者的分期、反应评估以及确定是否适合靶向放疗方面发挥着重要作用。这些功能成像方式中的每一种都针对不同的细胞特征，因此可以与解剖成像和彼此互补。随着最近美国食品和药物管理局的批准以及越来越多地在儿童中使用镓-DOTATATE 进行成像，本文旨在通过案例分析的方法，重点介绍 DOTATATE 成像在儿童 PPGL 的分期和反应评估中的优势和局限性，以及在其他神经内分泌恶性肿瘤中的应用。
