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经支气管镜切除治疗支气管内肺副神经节瘤

Management of an Endobronchial Pulmonary Paraganglioma via Bronchoscopic Resection.

作者信息

Hoang Khuyen, Brown Michael V, Badiei Arash, Nguyen Phan

机构信息

Adelaide Medical School, Faculty of Health and Medical Sciences University of Adelaide Adelaide Australia.

Department of Thoracic Medicine Royal Adelaide Hospital Adelaide Australia.

出版信息

Respirol Case Rep. 2025 Jul 4;13(7):e70265. doi: 10.1002/rcr2.70265. eCollection 2025 Jul.

Abstract

Paragangliomas are rare neuroendocrine tumours sharing embryological origins with phaeochromocytomas but differ in that they are found outside the adrenal medulla. The prevalence in literature ranges from 2 to 8 cases per million people. Pulmonary paragangliomas account for approximately 2% of this. Paragangliomas can cause symptoms due to the secretion of catecholamines. Other 'non-functional' paragangliomas may cause symptoms relating to mass effect or endobronchial obstruction. Once a diagnosis is made, the most common reported modality of management are lobectomy and endobronchial sleeve resection. Only a limited number of case reports describe other management modalities such as endobronchial ablation. We present the case of 48-year-old female with a right main bronchus endobronchial lesion identified on positron emission tomography (PET) dotatate scan on a background of succinic dehydrogenase B gene mutation (SDHB) familial paraganglioma. Bronchoscopic management with argon plasma coagulation (APC) probe ablation was used to debulk the lesion which resulted in excellent symptomatic improvement and stability on follow-up.

摘要

副神经节瘤是一种罕见的神经内分泌肿瘤,与嗜铬细胞瘤有着共同的胚胎学起源,但不同的是,它们位于肾上腺髓质之外。文献报道的发病率为每百万人中有2至8例。肺副神经节瘤约占其中的2%。副神经节瘤可因儿茶酚胺分泌而引起症状。其他“无功能”副神经节瘤可能会引起与占位效应或支气管内阻塞相关的症状。一旦确诊,最常见的治疗方式是肺叶切除术和支气管袖状切除术。只有少数病例报告描述了其他治疗方式,如支气管内消融。我们报告一例48岁女性患者,在琥珀酸脱氢酶B基因突变(SDHB)家族性副神经节瘤背景下,正电子发射断层扫描(PET)钇[90Y] DOTATATE扫描发现右主支气管内病变。采用氩等离子体凝固(APC)探头消融进行支气管镜治疗,以减少病变体积,症状得到显著改善,随访期间病情稳定。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/242e/12231209/33849403981c/RCR2-13-e70265-g001.jpg

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