【摘要】：背景： 副神經(jīng)節(jié)瘤（PGL）是神經(jīng)嵴起源的嗜鉻細(xì)胞產(chǎn)生的神經(jīng)內(nèi)分泌腫瘤，，臨床上可以根據(jù)臨床表現(xiàn)等分為寂靜型和功能型。由于寂靜型副神經(jīng)節(jié)瘤臨床表現(xiàn)不典型，缺乏高兒茶酚胺癥群，或因其他系統(tǒng)疾病為首發(fā)癥狀，甚至部分患者無任何臨床表現(xiàn)，導(dǎo)致臨床醫(yī)師診斷困難。但本病可在手術(shù)、創(chuàng)傷、應(yīng)激等情況下，引起兒茶酚胺大量釋放入血，而引起相關(guān)高兒茶酚胺血癥，嚴(yán)重者可因嚴(yán)重并發(fā)癥，如高血壓危象、急性左心衰等致死。而近年來隨著影像學(xué)的發(fā)展，寂靜型副神經(jīng)節(jié)瘤的檢出率不斷提高，而目前國內(nèi)外主要以個(gè)例報(bào)道為主。 目的： 總結(jié)21例寂靜型副神經(jīng)節(jié)瘤患者的臨床特征并文獻(xiàn)復(fù)習(xí)，以提高臨床醫(yī)師對本病的認(rèn)識(shí)，減少誤診、漏診。 方法： 收集2008年3月2013-12月在重慶醫(yī)科大學(xué)附屬第一醫(yī)院手術(shù)病理證實(shí)為寂靜型副神經(jīng)節(jié)瘤患者21例的臨床資料，對其臨床表現(xiàn)、影像學(xué)表現(xiàn)、病理及免疫組化表現(xiàn)、良惡性、治療及預(yù)后等進(jìn)行回顧性研究。 結(jié)果： 本組寂靜型副神經(jīng)節(jié)瘤21例，其中男性8例，女性13例，平均年齡47.05±12.22歲（從26歲到67歲不等）。寂靜組PGL瘤體主要位于右側(cè)，左側(cè)：右側(cè)（2：5），以上數(shù)據(jù)與癥狀組PGL相比無統(tǒng)計(jì)學(xué)差異。寂靜型PGL中瘤體直徑＜3cm、3～5cm、＞5cm分別有5例、7例、9例，其構(gòu)成比分別為23.8%、33.3%、42.9%，癥狀組PGL各構(gòu)成比分別為9.8%、43.9%、46.3%,有統(tǒng)計(jì)學(xué)差異。無論是寂靜組PGL還是癥狀組PGL，其CgA、Syn、NSE、S-100均陽性率高，寂靜組PASS評(píng)分平均分?jǐn)?shù)為：2.57±0.65；癥狀組中PASS評(píng)分平均分?jǐn)?shù)為：2.48±0.58,兩組間無統(tǒng)計(jì)學(xué)差異。無論是寂靜組PGL還是癥狀組PGL，在增強(qiáng)CT各期相可與腎上腺其他腫瘤相鑒別，但兩者之間CT值并無統(tǒng)計(jì)學(xué)差異。21例患者均采用手術(shù)治療，術(shù)中有4例發(fā)生高血壓危象。 結(jié)論： 寂靜型副神經(jīng)節(jié)瘤并非少見神經(jīng)內(nèi)分泌腫瘤。因其缺乏特異性臨床表現(xiàn)，診斷較困難，確診需要結(jié)合解剖影像學(xué)特征、生化檢查、功能成像和病理等。手術(shù)是最有效的治療，良惡性除病理提示外，長期隨訪是必須的。
[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 鹵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 鹵0. 65, and the average score of PASS score in symptom group was: 2. 48 鹵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.
【學(xué)位授予單位】：重慶醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2014
【分類號(hào)】：R739.4

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