[68Ga]DOTANOC PET/CT 和 [131I]MIBG 闪烁显像在嗜铬细胞瘤和副神经节瘤患者治疗中的作用：一项前瞻性研究。
Role of [68Ga]DOTANOC PET/computed tomography and [131I]MIBG scintigraphy in the management of patients with pheochromocytoma and paraganglioma: a prospective study.
机构信息
Department of Nuclear Medicine.
Department of Endocrinology.
出版信息
Nucl Med Commun. 2020 Oct;41(10):1047-1059. doi: 10.1097/MNM.0000000000001251.
PURPOSE
The primary aim of study was to compare role of iodine-131 (I-131)-labeled metaiodobenzylguanidine ([I]MIBG) and gallium-68 (Ga-68)-labeled DOTA-l-Nal3-octreotide ([Ga]DOTANOC) PET/computed tomography (CT) in patients with pheochromocytoma (PCC) and paraganglioma (PGL), subsequent follow-up to see management. The secondary aim was to see association of germline mutation in histopathologically proven patients.
PROCEDURES
We performed [Ga]DOTANOC PET/CT and [I]MIBG in 106 patients (61 men; age: 38.5 ± 16.2 years) of known or suspected PCC/PGL. Following scans, 16 histopathologically proven patients were screened for germline mutations.
RESULTS
[I]MIBG detected 41 lesions in 34 patients and [Ga]DOTANOC PET/CT detected more than 79 lesions in 55 patients. The mean duration of follow-up was 20.6 ± 16.5 months. Management following scans: surgery in 35 patients (positive histopathology in 34 patients, negative in 1 patient); lutecium-177 (Lu-177)-labeled DOTA-0-Tyr-3 octreotate ([Lu]DOTATATE) therapy in 2 patients; chemotherapy in 1 patient; conservative therapy in 34 patients; no therapy in 17 patients; 2 patients have died and 3 were lost to follow-up. Among 12 previously operated, 2 patients showed metastatic disease and 1 showed residual disease. Out of 16 patients who underwent genotypic analysis (15 operated), 8 were positive for germline mutations. Mutations were seen in SDHB, RET, VHL, MDH2 and SDHA genes, including two germline mutations in two patients. Deletion was observed in one patient in SDHB gene and substitution in all other mutations. Four novel mutations in MDH2 (c.1005G>C, c.916G>A, c.580G>A) and SDHB (c.378_380delAAT) were observed (SRA accession: PRJNA551457).
CONCLUSIONS
[Ga]DOTANOC PET/CT should be considered as a first-line investigation in PCC/PGL especially at high risk of metastasis and screening of persons with familial syndrome.
目的
研究的主要目的是比较碘-131（I-131）标记间碘苄胍（[I]MIBG）和镓-68（Ga-68）标记 DOTA-Nal3-奥曲肽（[Ga]DOTANOC）正电子发射断层扫描/计算机断层扫描（PET/CT）在嗜铬细胞瘤（PCC）和副神经节瘤（PGL）患者中的作用，后续随访以观察治疗效果。次要目的是观察组织病理学证实患者中种系突变的相关性。
方法
我们对 106 名已知或疑似 PCC/PGL 的患者（61 名男性；年龄：38.5±16.2 岁）进行了[Ga]DOTANOC PET/CT 和[I]MIBG 检查。在扫描后，对 16 名组织病理学证实的患者进行了种系突变筛查。
结果
[I]MIBG 在 34 名患者中检测到 41 个病灶，[Ga]DOTANOC PET/CT 在 55 名患者中检测到超过 79 个病灶。平均随访时间为 20.6±16.5 个月。扫描后的治疗方案如下：手术治疗 35 例（34 例患者的组织病理学阳性，1 例患者阴性）；钕-177（Lu-177）标记的 DOTA-0-Tyr-3 奥曲肽（[Lu]DOTATATE）治疗 2 例；化疗 1 例；保守治疗 34 例；未治疗 17 例；2 例患者死亡，3 例患者失访。在 12 例既往手术患者中，2 例患者出现转移疾病，1 例患者出现残留疾病。在 16 名接受基因分析的患者中（15 例已手术），8 例存在种系突变。突变发生在 SDHB、RET、VHL、MDH2 和 SDHA 基因中，包括两名患者的两个种系突变。SDHB 基因的一名患者存在缺失，所有其他突变均为替换。在 MDH2 基因中观察到四个新的突变（c.1005G>C、c.916G>A、c.580G>A）和 SDHB 基因（c.378_380delAAT）（SRA 注册号：PRJNA551457）。
结论
[Ga]DOTANOC PET/CT 应被视为 PCC/PGL 的一线检查方法，特别是在转移风险较高和家族性综合征患者的筛查中。
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