Endocr Pract. 2019 Mar;25(3):211-219. doi: 10.4158/EP-2018-0480.
Most of the Indian studies on pheochromocytoma/paraganglioma (PCC/PGL) have focused on PCC, and there is a paucity of information regarding sympathetic paraganglioma (sPGL). Here, we describe the clinical, biochemical, and imaging features of sPGL compared with PCC.
This retrospective study included 75 patients with sPGL and 150 patients with PCC. Diagnosis of PCC/PGL was based on surgical histopathology, and if histopathology was not available, on biochemistry and/or radiology.
sPGL was more frequently detected incidentally ( P = .03), normetanephrine-secreting ( P<.01), and metastatic compared with PCC ( P≤.01). sPGL was most commonly located in the organ of Zuckerkandl (OOZ) (49%) and infradiaphragmatic area above the OOZ (27%). Patients with mediastinal sPGL were significantly older than those with sPGL in the OOZ ( P = .03). Primary tumors of metastatic sPGL were significantly larger than those without metastasis (7.8 ± 4 cm vs. 5.6 ± 3.2 cm; P = .004). Percentage arterial enhancement (PAE) >100% was seen in 98% of sPGLs.
Incidental presentation, normetanephrine-secreting phenotype, and metastatic disease were more frequent in patients with sPGL than those with PCC. sPGL arose most commonly in the OOZ. Tumor size is an independent predictor of malignancy among sPGL patients. PAE >100% is almost a universal finding in sPGL, and its absence is a sensitive parameter to differentiate sPGL from other abdominal masses.
AP = arterial phase; CECT = contrast-enhanced computed tomography; CT = computed tomography; DP = delayed phase; EVP = early venous phase; FDG = fluorodeoxyglucose; fPFMN = fractionated plasma free metanephrine; HU = Hounsfield units; MIBG = metaiodobenzylguanidine; MRI = magnetic resonance imaging; OOZ = organ of Zuckerkandl; PAE = percentage arterial enhancement; PCC = pheochromocytoma; PET = positron emission tomography; PFNMN = plasma free normetanephrine; PGL = paraganglioma; PRRT = peptide receptor radionuclide therapy; PVE = percentage venous enhancement; sPGL = sympathetic paraganglioma; UP = unenhanced phase; VMA = vanillyl mandelic acid.
大多数印度关于嗜铬细胞瘤/副神经节瘤（PCC/PGL）的研究都集中在 PCC 上，关于交感副神经节瘤（sPGL）的信息很少。在这里，我们描述了 sPGL 与 PCC 相比的临床、生化和影像学特征。
这项回顾性研究包括 75 例 sPGL 患者和 150 例 PCC 患者。PCC/PGL 的诊断基于手术组织病理学，如果没有组织病理学，则基于生化和/或影像学。
sPGL 更常被偶然发现（P =.03），与 PCC 相比，sPGL 更常分泌去甲变肾上腺素（P<.01）和转移性（P≤.01）。sPGL 最常见的部位是 Zuckerkandl 器官（OOZ）（49%）和 OOZ 上方的膈下区域（27%）。纵隔 sPGL 患者的年龄明显大于 OOZ 中的 sPGL 患者（P =.03）。转移性 sPGL 的原发性肿瘤明显大于无转移的肿瘤（7.8 ± 4 cm 比 5.6 ± 3.2 cm；P =.004）。98%的 sPGL 出现动脉增强百分比（PAE）>100%。
与 PCC 患者相比，sPGL 患者更常出现偶然发现、去甲变肾上腺素分泌表型和转移性疾病。sPGL 最常见于 OOZ。肿瘤大小是 sPGL 患者恶性肿瘤的独立预测因素。PAE >100%几乎是 sPGL 的普遍发现，其不存在是区分 sPGL 与其他腹部肿块的敏感参数。
AP = 动脉期；CECT = 对比增强 CT；CT = 计算机断层扫描；DP = 延迟期；EVP = 早期静脉期；FDG = 氟脱氧葡萄糖；fPFMN = 血浆游离间甲肾上腺素；HU = 亨氏单位；MIBG = 间碘苄胍；MRI = 磁共振成像；OOZ = Zuckerkandl 器官；PAE = 动脉增强百分比；PCC = 嗜铬细胞瘤；PET = 正电子发射断层扫描；PFNM N = 血浆游离去甲变肾上腺素；PGL = 副神经节瘤；PRRT = 肽受体放射性核素治疗；PVE = 静脉增强百分比；sPGL = 交感副神经节瘤；UP = 未增强期；VMA = 香草扁桃酸。
