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特发性多中心 Castleman 病中囊肿形成的放射学和病理学特征。

Radiological and Pathological Features of Cyst Formation in Idiopathic Multicentric Castleman Disease.

机构信息

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan.

Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan.

出版信息

Adv Respir Med. 2023 Apr 19;91(2):164-173. doi: 10.3390/arm91020014.

Abstract

INTRODUCTION

Idiopathic multicentric Castleman disease (MCD) has been reported to form lung cysts at a relatively high rate. However, the radiological and pathological features of cystic formation in MCD are unclear.

METHODS

To clarify these questions, we retrospectively investigated the radiological and pathological findings of cysts in MCD patients. Eight consecutive patients who underwent surgical lung biopsies in our center from 2000 to 2019 were included.

RESULTS

The median age was 44.5 years, with three males and five females. On the initial computed tomography, cyst formation was found in seven patients (87.5%). All of the cysts were multiple, round, and thin walled, accompanying ground-glass attenuation (GGA) around cysts. In six patients (75%), cysts increased during their clinical courses, and the new cysts had emerged from GGA, although GGA was improved by treatment. In all four cases, whose pulmonary cysts could be pathologically evaluated, a marked plasma cell infiltration around the cyst wall, and loss of elastic fibers of the alveolar wall were observed.

CONCLUSIONS

Pulmonary cysts emerged in the area of GGA pathologically consistent with plasma cell infiltration. Cysts in MCD may be formed by the loss of elastic fibers due to marked plasma cell infiltration and may be considered irreversible changes.

摘要

简介

特发性多中心 Castleman 病(MCD)已被报道以相对较高的比率形成肺囊肿。然而,MCD 中囊肿形成的放射学和病理学特征尚不清楚。

方法

为了阐明这些问题,我们回顾性调查了 MCD 患者的囊肿的放射学和病理学发现。纳入了 2000 年至 2019 年在我们中心接受手术肺活检的 8 例连续患者。

结果

中位年龄为 44.5 岁,男性 3 例,女性 5 例。在初始 CT 上,7 例(87.5%)发现囊肿形成。所有囊肿均为多发、圆形、薄壁,伴有囊肿周围磨玻璃影(GGA)。在 6 例(75%)患者中,囊肿在其临床过程中增加,新的囊肿从 GGA 出现,尽管治疗后 GGA 有所改善。在所有 4 例可以进行肺囊肿病理学评估的病例中,均观察到囊肿壁周围明显的浆细胞浸润和肺泡壁弹性纤维丧失。

结论

肺囊肿出现在与浆细胞浸润一致的 GGA 区域,病理上。MCD 中的囊肿可能是由于明显的浆细胞浸润导致弹性纤维丧失而形成的,并且可能被认为是不可逆的改变。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5ec9/10135672/ca0d9ff29c4b/arm-91-00014-g001.jpg

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