Kunz W G, Auernhammer C J, Nölting S, Pfluger T, Ricke J, Cyran C C
Klinik und Poliklinik für Radiologie, Klinikum der Universität München, Marchioninistr. 15, 81377, München, Deutschland.
Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, München, Deutschland.
Radiologe. 2019 Nov;59(11):975-981. doi: 10.1007/s00117-019-0569-7.
If pheochromocytoma (PC) or paraganglioma (PGL) is diagnosed based on serologic studies, imaging is required to locate the adrenal mass for further management. Besides pathognomonic hormonal findings, PC/PGL can exhibit typical imaging features. However, PC/PGL can also show morphological overlap with other pathologies.
The modality of choice for evaluation of PC is CT. In case of extra-adrenal location, MRI is superior to CT. Imaging with PET-CT provides complementary information in the differentiation of PC/PGL and is recommended as the imaging modality of choice for malignant PC/PGL. Ga-DOTATATE (or Ga-DOTATOC/ Ga-DOTANOC) PET-CT has high sensitivity for SDHx-mutated PC/PGL and serves for planning of radioreceptor therapy with somatostatin analogues. In contrast, I-metaiodobenzylguanidine (MIBG) scintigraphy is important in assessing the potential efficacy of radioreceptor therapy with MIBG.
The CT protocol for PC evaluation should include non-enhanced, arterial, portal-venous and late phases; the latter for the evaluation of wash-out. Recent studies indicate non-enhanced CT alone may be sufficient to rule out PC. For MRI, in- and opposed-phase sequences should be additionally acquired.
A relevant proportion of PC is diagnosed incidentally. Therefore, imaging of PC will gain further importance. Recent studies show better response rates of PC/PGL after radioreceptor therapy with somatostatin analogues (Lu-DOTATATE) than with MIBG. Therefore, Ga-DOTATATE PET-CT gains further importance-for diagnostic imaging and therapy planning.
如果基于血清学研究诊断出嗜铬细胞瘤（PC）或副神经节瘤（PGL），则需要进行影像学检查以定位肾上腺肿块，以便进一步治疗。除了具有诊断意义的激素检查结果外，PC/PGL还可表现出典型的影像学特征。然而，PC/PGL也可能与其他病理情况在形态学上存在重叠。
评估PC的首选检查方式是CT。对于肾上腺外的病变，MRI优于CT。PET-CT成像在PC/PGL的鉴别诊断中可提供补充信息，推荐作为恶性PC/PGL的首选成像方式。镓标记的奥曲肽（或镓标记的托曲肽/镓标记的那曲肽）PET-CT对琥珀酸脱氢酶亚基（SDHx）突变的PC/PGL具有高敏感性，可用于生长抑素类似物放射受体治疗的规划。相比之下，碘代间位苄胍（MIBG）闪烁扫描在评估MIBG放射受体治疗的潜在疗效方面很重要。
用于评估PC的CT方案应包括平扫、动脉期、门静脉期和延迟期；延迟期用于评估洗脱情况。最近的研究表明，仅平扫CT可能足以排除PC。对于MRI，应额外采集同相位和反相位序列。
相当一部分PC是偶然诊断出来的。因此，PC的影像学检查将变得更加重要。最近的研究表明，与MIBG相比，生长抑素类似物（镥标记的奥曲肽）放射受体治疗后PC/PGL的缓解率更高。因此，镓标记的奥曲肽PET-CT在诊断成像和治疗规划方面变得更加重要。
