应用 (18)F-FDOPA PET 对无转移副神经节瘤和嗜铬细胞瘤进行功能特征分析：关注遗漏病变。
Functional characterization of nonmetastatic paraganglioma and pheochromocytoma by (18) F-FDOPA PET: focus on missed lesions.
机构信息
Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France.
出版信息
Clin Endocrinol (Oxf). 2013 Aug;79(2):170-7. doi: 10.1111/cen.12126. Epub 2013 May 6.
AIMS AND METHODS
To evaluate the clinical value of (18) F-fluorodihydroxyphenylalanine ((18) F-FDOPA) PET in relation to tumour localization and the patient's genetic status in a large series of pheochromocytoma/paraganglioma (PHEO/PGL) patients and to discuss in detail false-negative results. A retrospective study of PGL patients who were investigated with (18) F-FDOPA PET or PET/CT imaging in two academic endocrine tumour centres was conducted (La Timone University Hospital, Marseilles, France and National Institutes of Health (NIH), Bethesda, MD, USA).
RESULTS
One hundred sixteen patients (39·7% harbouring germline mutations in known disease susceptibility genes) were evaluated for a total of 195 PHEO/PGL foci. (18) F-FDOPA PET correctly detected 179 lesions (91·8%) in 107 patients (92·2%). Lesion-based sensitivities for parasympathetic PGLs (head, neck, or anterior/middle thoracic ones), PHEOs, and extra-adrenal sympathetic (abdominal or posterior thoracic) PGLs were 98·2% [96·5% for Timone and 100% for NIH], 93·9% [93·8 and 93·9%] and 70·3% [47·1 and 90%] respectively (P < 0·001). Sympathetic (adrenal and extra-adrenal) SDHx-related PGLs were at a higher risk for negative (18) F-FDOPA PET than non-SDHx-related PGLs (14/24 vs 0/62, respectively, P < 0·001). In contrast, the risk of negative (18) F-FDOPA PET was lower for parasympathetic PGLs regardless of the genetic background (1/90 in SDHx vs 1/19 in non-SDHx tumours, P = 0·32). (18) F-FDOPA PET failed to detect two head and neck PGLs (HNPGL), likely due to their small size, whereas most missed sympathetic PGL were larger and may have exhibited a specific (18) F-FDOPA-negative imaging phenotype. (18) F-FDG PET detected all the missed sympathetic lesions.
CONCLUSIONS
(18) F-FDOPA PET appears to be a very sensitive functional imaging tool for HNPGL regardless of the genetic status of the tumours. Patients with false-negative tumours on (18) F-FDOPA PET should be tested for SDHx mutations.
目的和方法
在两个学术内分泌肿瘤中心（法国马赛的 La Timone 大学医院和美国国立卫生研究院（NIH）），对接受（18）F-氟代二羟苯丙氨酸（（18）F-FDOPA）PET 或 PET/CT 成像检查的嗜铬细胞瘤/副神经节瘤（PHEO/PGL）患者进行了一项大型系列研究，评估（18）F-FDOPA PET 在肿瘤定位和患者遗传状态方面的临床价值，并详细讨论假阴性结果。
结果
对 116 名患者（39.7%携带已知疾病易感性基因的种系突变）进行了总计 195 个 PHEO/PGL 病灶的评估。（18）F-FDOPA PET 在 107 名患者（92.2%）中正确检测到 179 个病灶（91.8%）。副交感神经 PGL（头、颈或前/中胸）、PHEO 和肾上腺外交感神经（腹部或后胸）PGL 的基于病灶的敏感性分别为 98.2%[Timone 为 96.5%，NIH 为 100%]、93.9%[93.8%和 93.9%]和 70.3%[47.1%和 90%]（P<0.001）。交感神经（肾上腺和肾上腺外）SDHx 相关 PGL 的（18）F-FDOPA PET 阴性风险高于非 SDHx 相关 PGL（分别为 14/24 与 0/62，P<0.001）。相比之下，无论遗传背景如何，副交感神经 PGL 的（18）F-FDOPA PET 阴性风险均较低（SDHx 为 1/90，非 SDHx 为 1/19，P=0.32）。（18）F-FDOPA PET 未能检测到两个头颈部 PGL（HNPGL），可能是由于其体积较小，而大多数错过的交感神经 PGL 较大，可能表现出特定的（18）F-FDOPA 阴性成像表型。（18）F-FDG PET 检测到所有错过的交感神经病变。
结论
（18）F-FDOPA PET 似乎是 HNPGL 的一种非常敏感的功能成像工具，与肿瘤的遗传状态无关。（18）F-FDOPA PET 检查呈假阴性的肿瘤患者应进行 SDHx 突变检测。
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