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目的进一步提高血浆游离甲氧基肾上腺素(MN)、甲氧基去甲肾上腺素(NMN)正常的嗜铬细胞瘤(Pheos)和副神经节瘤(PGLs)患者的诊断水平。方法本研究组回顾性分析了本中心2004年到2012年电子数据库中病理确认的Pheos和PGLs(PPGLs)患者的临床症状与体征、内分泌激素水平、遗传综合征特征、增强CT表现和核素显像特点。根据血浆游离MNs的水平将患者分为MNs正常组与MNs增高组,比较两组患者的上述临床特点。结果病理证实的PPGLs患者共189例,其中MNs正常组24例,增高组165例。MNs正常组患者高血压、阵发性高血压、阵发性的头痛、心悸、大汗三联征症状≥2项、体重下降发生率明显低于增高组(P<0.05);MNs正常组患者24 h尿肾上腺素、去甲肾上腺素、多巴胺的水平相对增高组明显降低,尤其24 h尿去甲肾上腺素仅是后者平均水平的1/6(P=0.001)。虽然增强CT上MNs正常组PPGLs肿块明显不均匀强化发生率较增高组低14.8%(P=0.101),但是它仍占该组PPGLs肿块CT增强形式的主要部分;两组中患者肿瘤增强CT上所有增强形式的发生率无明显统计学差异(P>0.05)。核素显像对两种肿瘤的发现率相似(P=1.000)。结论血浆游离MNs正常的PPGLs患者的临床症状与体征不典型,儿茶酚胺水平降低;增强CT上肿块明显不均匀强化的特征以及核素扫描阳性比内分泌激素检测更有助于血浆游离MNs正常PPGLs的诊断。
Objective To further improve the diagnostic value of plasma free methionine (MN), normal norepinephrine (NMN) patients with pheos and paraganglioma (PGLs). Methods The study group retrospectively analyzed the clinical signs and symptoms, the level of endocrine hormones, the features of hereditary syndrome, the manifestations of enhanced CT and the appearance of radionuclide in patients with pathologically confirmed Pheos and PGLs (PPGLs) from 2004 to 2012 in our center. Like features. Patients were divided into MNs normal group and MNs increased group according to the level of plasma free MNs. The clinical features of the two groups were compared. Results A total of 189 pathologically confirmed PPGLs patients, including 24 cases of normal MNs group, 165 cases of increasing group. Patients with normal MNs had hypertension≥2, paroxysmal hypertension, paroxysmal headache, palpitations and hyperhidrosis symptoms≥2, and the incidence of weight loss was significantly lower than those in the normal group (P <0.05) h, norepinephrine, norepinephrine and dopamine levels were significantly lower, especially 24 h urinary norepinephrine was only 1/6 of the latter (P = 0.001). Although the incidence of significant inhomogeneous enhancement of the PPGLs mass in the normal MNs on enhanced CT was 14.8% lower than that in the elevated group (P = 0.101), it still accounted for the major part of the enhanced form of CT in the group of PPGLs; in both groups, There was no significant difference in the incidence of all forms of enhancement (P> 0.05). Radionuclide imaging was similar for both tumors (P = 1.000). Conclusions The clinical symptoms and signs of typical PPGLs with plasma free MNs are not typical and the levels of catecholamines are reduced. The obvious heterogeneous enhancement of the mass on the enhanced CT and the positive scintigraphy are more helpful than the detection of endocrine hormone in the diagnosis of normal PPGLs in plasma-free MNs .