寂静型副神经节瘤21例临床分析并文献复习

发布时间：2018-08-07 15:24

【摘要】：背景：副神经节瘤（PGL）是神经嵴起源的嗜铬细胞产生的神经内分泌肿瘤，，临床上可以根据临床表现等分为寂静型和功能型。由于寂静型副神经节瘤临床表现不典型，缺乏高儿茶酚胺症群，或因其他系统疾病为首发症状，甚至部分患者无任何临床表现，导致临床医师诊断困难。但本病可在手术、创伤、应激等情况下，引起儿茶酚胺大量释放入血，而引起相关高儿茶酚胺血症，严重者可因严重并发症，如高血压危象、急性左心衰等致死。而近年来随着影像学的发展，寂静型副神经节瘤的检出率不断提高，而目前国内外主要以个例报道为主。目的：总结21例寂静型副神经节瘤患者的临床特征并文献复习，以提高临床医师对本病的认识，减少误诊、漏诊。方法：收集2008年3月2013-12月在重庆医科大学附属第一医院手术病理证实为寂静型副神经节瘤患者21例的临床资料，对其临床表现、影像学表现、病理及免疫组化表现、良恶性、治疗及预后等进行回顾性研究。结果：本组寂静型副神经节瘤21例，其中男性8例，女性13例，平均年龄47.05±12.22岁（从26岁到67岁不等）。寂静组PGL瘤体主要位于右侧，左侧：右侧（2：5），以上数据与症状组PGL相比无统计学差异。寂静型PGL中瘤体直径＜3cm、3～5cm、＞5cm分别有5例、7例、9例，其构成比分别为23.8%、33.3%、42.9%，症状组PGL各构成比分别为9.8%、43.9%、46.3%,有统计学差异。无论是寂静组PGL还是症状组PGL，其CgA、Syn、NSE、S-100均阳性率高，寂静组PASS评分平均分数为：2.57±0.65；症状组中PASS评分平均分数为：2.48±0.58,两组间无统计学差异。无论是寂静组PGL还是症状组PGL，在增强CT各期相可与肾上腺其他肿瘤相鉴别，但两者之间CT值并无统计学差异。21例患者均采用手术治疗，术中有4例发生高血压危象。结论：寂静型副神经节瘤并非少见神经内分泌肿瘤。因其缺乏特异性临床表现，诊断较困难，确诊需要结合解剖影像学特征、生化检查、功能成像和病理等。手术是最有效的治疗，良恶性除病理提示外，长期随访是必须的。
[Abstract]:Background: paraganglioma (PGL) is a neuroendocrine tumor produced by chromaffin cells derived from neural crest. It can be divided into silent type and functional type according to clinical manifestations. Due to the atypical clinical manifestations of silent paraganglioma, the lack of high catecholamine group, or the initial symptoms of other systemic diseases, even some patients without any clinical manifestations, clinicians are difficult to diagnose. However, this disease can cause a large amount of catecholamine to release into the blood under the condition of operation, trauma, stress, etc., and cause related hypercatecholamia. In severe cases, severe complications, such as hypertension crisis, acute left heart failure and so on, can cause death. In recent years, with the development of imaging, the detection rate of silent paraganglioma is increasing. Objective: to summarize the clinical features and literature review of 21 patients with silent paraganglioma in order to improve the understanding of the disease, reduce misdiagnosis and missed diagnosis. Methods: the clinical data of 21 patients with silent paraganglioma confirmed by surgery and pathology in the first affiliated Hospital of Chongqing Medical University from March to December 2008 were collected. The clinical manifestations, imaging findings, pathological and immunohistochemical findings were analyzed. A retrospective study of benign and malignant, treatment and prognosis was carried out. Results: there were 21 cases of silent paraganglioma, including 8 males and 13 females, with an average age of 47.05 卤12.22 years (ranging from 26 to 67 years). The tumor of PGL in silent group was mainly located in right side, left side and right side (2:5). There was no statistical difference between the above data and PGL of symptom group. In silent type PGL, the diameter of tumor was less than 3 cm ~ 3 ~ 5 cm, > 5cm was 5 cases or 7 cases or 9 cases, the constituent ratio was 23. 8% and 33. 3%, respectively. The ratio of PGL composition in symptom group was 9. 8% and 43. 3 cm, respectively. There was statistical difference between them. The positive rate of PGL in both silent group and symptom group was high, the average score of PASS score in silent group was 2. 57 卤0. 65, and the average score of PASS score in symptom group was: 2. 48 卤0. 58. There was no significant difference between the two groups. In both silent group and symptom group, PGL could be distinguished from other adrenal tumors in each phase of enhanced CT, but there was no significant difference in CT value between the two groups. 21 cases were treated by operation, and 4 cases developed hypertension crisis during operation. Conclusion: silent paraganglioma is not uncommon neuroendocrine tumor. Due to its lack of specific clinical manifestations, diagnosis is difficult, the diagnosis needs to be combined with anatomical imaging features, biochemical examination, functional imaging and pathology. Surgery is the most effective treatment and long-term follow-up is necessary except for pathological cues.
【学位授予单位】：重庆医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R739.4

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