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以咳嗽为首发症状的心内副神经节瘤

Intracardiac paraganglioma with a cough as the first symptom.

机构信息

Department of Cardiovascular Surgery, Changhai Hospital, Naval Medical University, Shanghai, 200433, People's Republic of China.

Department of Nuclear Medicine, Changhai Hospital, Naval Medical University, Shanghai, 200433, People's Republic of China.

出版信息

J Cardiothorac Surg. 2023 Jan 17;18(1):30. doi: 10.1186/s13019-022-02087-z.

DOI:10.1186/s13019-022-02087-z
PMID:36650563
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9843092/
Abstract

BACKGROUND

Cardiac paragangliomas (PGLs) are clinically rare, with hypertension and metabolic changes as the main symptoms. The tumor is highly related to gene mutation, and surgery is presently the effective treatment. Medical history and clinical manifestations of the patient, routine laboratory examinations and imaging examinations, and pathological examination can help the final diagnosis.

CASE PRESENTATION

The present study presents a 31-year-old male patient with a left atrial mass. The initial symptom was cough. Cardiac enlargement was found during the chest X-ray. The follow-up imaging examination revealed a left atrial occupying lesion, and the possibility of malignant occupying lesions was not ruled out. The patient underwent surgical resection of the mass. The final pathological result revealed paraganglioma. The thoracic computed tomography review two months after the operation revealed that the original occupying lesion disappeared, and no new lesion was found.

CONCLUSIONS

Pheochromocytomas and paragangliomas (PPGLs) are a kind of neuroendocrine tumors. PPGLs can cause secondary hypertension, and lead to a series of clinical syndromes, including myocardial injury, metabolic changes, and so on. The occurrence of PPGIs is related to gene mutation. Biochemical detection, imaging examination, and genetic testing can help diagnose. The tumor should be surgically removed as soon as possible after the diagnosis. As a functional tumor, PPGLs should be fully prepared before surgery to avoid anesthesia and huge fluctuations in blood pressure during and after surgery, or the occurrence of fatal hypertensive crisis and intractable hypotension after tumor resection. Adequate preoperative preparation directly affects the prognosis of patients after surgery. Therefore, multidisciplinary cooperation before, during, and after the operation is extremely important.

摘要

背景

心脏副神经节瘤(PGL)临床少见,以高血压、代谢改变为主要表现,肿瘤与基因突变更为密切,目前手术是有效的治疗方法。患者的病史、临床表现、常规实验室检查和影像学检查以及病理学检查有助于最终诊断。

病例介绍

本研究报道了 1 例 31 岁男性患者,以左心房肿块为首发症状,初始症状为咳嗽,胸部 X 线检查发现心脏增大,随访影像学检查提示左心房占位性病变,不排除恶性占位性病变可能,行肿块切除术,最终病理结果提示为副神经节瘤。术后 2 个月复查胸部 CT 提示原占位性病变消失,未发现新发病灶。

结论

嗜铬细胞瘤和副神经节瘤(PPGL)是一种神经内分泌肿瘤,可引起继发性高血压,并导致一系列临床综合征,包括心肌损伤、代谢改变等。PPGL 的发生与基因突变更为密切,生化检测、影像学检查和基因检测有助于诊断,诊断后应尽快手术切除肿瘤。由于肿瘤为功能性肿瘤,PPGL 术前应充分准备,避免术中、术后麻醉及血压剧烈波动,或肿瘤切除后发生致命性高血压危象和难治性低血压。充分的术前准备直接影响患者术后的预后,因此手术前后的多学科合作至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac79/9843926/93b9f92600ed/13019_2022_2087_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac79/9843926/dd79316f2988/13019_2022_2087_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac79/9843926/f9a0e5554a6e/13019_2022_2087_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac79/9843926/93b9f92600ed/13019_2022_2087_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac79/9843926/dd79316f2988/13019_2022_2087_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac79/9843926/f9a0e5554a6e/13019_2022_2087_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac79/9843926/93b9f92600ed/13019_2022_2087_Fig3_HTML.jpg

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