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结节性肺淀粉样变性的影像学及临床特征

Imaging and Clinical Features of Nodular Pulmonary Amyloidosis.

作者信息

Li Fei, Li Junting, Li Yanyan, Shang Danting, Hou Xingyi, He Yanli, Li Gangfeng

机构信息

Department of Radiology, Tangdu Hospital, Air Force Medical University.

Department of Pathology, Tangdu Hospital, Air Force Medical University, Xi'an Shaanxi, People's Republic of China.

出版信息

J Thorac Imaging. 2025 Sep 1;40(5):e0830. doi: 10.1097/RTI.0000000000000830.

Abstract

PURPOSE

To investigate the clinical and computed tomography (CT) features of nodular pulmonary amyloidosis (NPA) to enhance our understanding of the disease and improve the ability to differentiate it from other similar conditions.

MATERIALS AND METHODS

A retrospective analysis was conducted on the clinical data, chest CT imaging findings, and pathologic characteristics of 13 patients with NPA in our hospital from April 2014 to April 2024. All 13 patients underwent chest CT plain scan examination. The basic data, medical history, clinical manifestations, and lung lesion features on chest CT imaging were analyzed and summarized.

RESULTS

Among the 13 patients, there were 3 males (23.08%) and 10 females (76.92%). Their ages ranged from 37 to 68 years, with a mean age of (57.85±8.40) years and a median age of 59 years. Three (23.08%) patients had cough and sputum, while the others (76.92%) had no clinical symptoms. Before surgery, 6 patients underwent chest CT scans, and NPA changes in size, shape, and density were observed. Six cases (46.15%) were located in the left lung (4 in the upper lobe and 2 in the lower lobe), and 7 cases (53.85%) in the right lung (3 in the upper lobe, 2 in the middle lobe, and 2 in the lower lobe). Seven cases (53.85%) of NPA were round or oval, while 6 cases (46.15%) were irregularly shaped. Out of the NPA cases, 11 (84.62%) were solid nodules with well-defined boundaries, including 2 cases of solid nodules with surrounding calcification. In addition, 2 cases presented as solid nodules with cavities. Ten cases (76.92%) had multiple cystic lesions in the bilateral lungs, in which 7 cases had more than 10 cysts with obvious cyst walls, and 1 case showed a solid nodule on the cyst wall. During the postoperative follow-up, 1 patient experienced an increase in the size of the original nodule and the appearance of new solid nodules. Subsequent surgery revealed mucosal-associated lymphoid tissue lymphoma (MALT). The remaining patients were followed up regularly, and their conditions remained stable.

CONCLUSIONS

NPA is more common in middle-aged and elderly people and is more likely to occur in women. Most cases are asymptomatic, and bilateral lungs can be involved. For nodules with multiple pulmonary cysts found by chest CT, the possibility of NPA should be considered, and further histopathologic examination is needed to confirm the diagnosis. Most patients with NPA have a good long-term prognosis after surgical resection, but some patients require further investigation and close follow-up due to underlying causes.

摘要

目的

探讨结节性肺淀粉样变性(NPA)的临床及计算机断层扫描(CT)特征,以加深对该疾病的认识,提高其与其他类似疾病的鉴别能力。

材料与方法

对2014年4月至2024年4月我院收治的13例NPA患者的临床资料、胸部CT影像表现及病理特征进行回顾性分析。13例患者均行胸部CT平扫检查。分析总结其基本资料、病史、临床表现及胸部CT影像上的肺部病变特征。

结果

13例患者中,男性3例(23.08%),女性10例(76.92%)。年龄37~68岁,平均年龄(57.85±8.40)岁,中位年龄59岁。3例(23.08%)患者有咳嗽、咳痰症状,其余患者(76.92%)无临床症状。术前6例患者行胸部CT扫描,观察到NPA在大小、形态及密度上的变化。6例(46.15%)位于左肺(上叶4例,下叶2例),7例(53.85%)位于右肺(上叶3例,中叶2例,下叶2例)。7例(53.85%)NPA呈圆形或椭圆形,6例(46.15%)形态不规则。NPA病例中,11例(84.62%)为实性结节,边界清晰,其中2例实性结节周围有钙化。此外,2例表现为实性结节伴空洞。10例(76.92%)患者双肺有多发囊性病变,其中7例有10个以上囊肿,囊壁明显,1例在囊壁上可见实性结节。术后随访期间,1例患者原结节增大并出现新的实性结节。后续手术病理显示为黏膜相关淋巴组织淋巴瘤(MALT)。其余患者定期随访,病情稳定。

结论

NPA多见于中老年人群,女性更易发生。多数病例无症状,可累及双肺。对于胸部CT发现的伴有多发肺囊肿的结节,应考虑NPA的可能,需进一步行组织病理学检查以明确诊断。多数NPA患者手术切除后长期预后良好,但部分患者因潜在病因需进一步检查及密切随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6cba/12369498/53302c9a51ee/rti-40-e0830-g001.jpg

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