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巨大多房囊性化生性胸腺瘤:一例报告

Giant Multilocular-Cystic Metaplastic Thymoma: A Case Report.

作者信息

Han Lihua, Gao Bo, Wang En-Hua, Wang Liang

机构信息

The Central Laboratory of Morphology, Shenyang Medical College, Shenyang, People's Republic of China.

Department of Pathology, Cancer Hospital of China Medical University, Shenyang, People's Republic of China.

出版信息

Pharmgenomics Pers Med. 2023 May 19;16:463-466. doi: 10.2147/PGPM.S413757. eCollection 2023.

DOI:10.2147/PGPM.S413757
PMID:37229516
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10204752/
Abstract

The metaplastic thymoma with giant multilocular-cyst formation had not been documented. The metaplastic thymoma is an extremely rare primary thymic epithelial tumor with an indolent clinical course. It is characterized by a histologic biphasic appearance, which is consisted of solid epithelial areas and spindle cells as the background. This specific pattern can be easily mistaken as the type A thymoma or the type A components of type AB thymoma. When cystic change occurs in metaplastic thymoma, it will bring more challenges for both imaging and pathological diagnosis. Herein, we reported an extremely rare case of a 14.9-cm giant tumor located in the anterior mediastinum of an elderly female. The tumor is consisted of both multilocular cysts and solid components, with the largest cyst measuring 6 cm in diameter. The multilocular cyst contained hemorrhage, calcification, and cholesterol crystal cracks without cell lining. In the solid area, the epithelial cell nests were surrounded by spindle cells with scattered lymphocytes. With immunostains, neither type of cells was CD20 positive. The epithelial cells were positive for CK and P63, while the spindle cells expressed vimentin and EMA. Fluorescence in situ hybridization analysis revealed that the tumor harbored gene fusions. Accordingly, although the multilocular cystic pattern set a diagnostic challenge, the diagnosis of metaplastic thymoma was rendered due to the immunohistochemistry staining and gene rearrangement detection.

摘要

伴有巨大多房囊肿形成的化生型胸腺瘤此前未见报道。化生型胸腺瘤是一种极为罕见的原发性胸腺上皮肿瘤,临床病程呈惰性。其组织学特征为双相外观,由实性上皮区域和梭形细胞构成背景。这种特殊模式易被误诊为A型胸腺瘤或AB型胸腺瘤的A型成分。当化生型胸腺瘤发生囊性变时,会给影像学和病理诊断带来更多挑战。在此,我们报道了一例极为罕见的病例,一名老年女性前纵隔出现一个14.9厘米的巨大肿瘤。肿瘤由多房囊肿和实性成分组成,最大囊肿直径达6厘米。多房囊肿内有出血、钙化及胆固醇结晶裂隙,无细胞内衬。在实性区域,上皮细胞巢被散在淋巴细胞的梭形细胞包绕。免疫组化染色显示,两种细胞均不表达CD20。上皮细胞CK和P63呈阳性,而梭形细胞表达波形蛋白和EMA。荧光原位杂交分析显示肿瘤存在基因融合。因此,尽管多房囊性模式带来了诊断挑战,但通过免疫组化染色和基因重排检测,仍诊断为化生型胸腺瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b6/10204752/b896c7469949/PGPM-16-463-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b6/10204752/3a7aa5a180ed/PGPM-16-463-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b6/10204752/f36d5e34bd1c/PGPM-16-463-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b6/10204752/b896c7469949/PGPM-16-463-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b6/10204752/3a7aa5a180ed/PGPM-16-463-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b6/10204752/f36d5e34bd1c/PGPM-16-463-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b6/10204752/b896c7469949/PGPM-16-463-g0003.jpg

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本文引用的文献

1
Unique correlation between GTF2I mutation and spindle cell morphology in thymomas (type A and AB thymomas).胸腺瘤(A型和 AB 型胸腺瘤)中 GTf2i 突变与梭形细胞形态之间的独特相关性。
J Clin Pathol. 2023 Jul;76(7):463-466. doi: 10.1136/jclinpath-2021-207837. Epub 2022 Jan 17.
2
Fusion as a Diagnostic Biomarker for Metaplastic Thymoma.融合作为化生性胸腺瘤的诊断生物标志物
Front Oncol. 2021 Jul 20;11:692283. doi: 10.3389/fonc.2021.692283. eCollection 2021.
3
Metaplastic thymoma: a distinctive thymic neoplasm characterized by YAP1-MAML2 gene fusions.
化生性胸腺瘤:一种具有特征性的胸腺肿瘤,其特征在于存在 YAP1-MAML2 基因融合。
Mod Pathol. 2020 Apr;33(4):560-565. doi: 10.1038/s41379-019-0382-x. Epub 2019 Oct 22.
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Resected case of giant cystic thymoma with spontaneous intracystic hemorrhage.巨大囊性胸腺瘤伴自发性囊内出血的切除病例
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Am J Clin Pathol. 2012 Feb;137(2):261-9. doi: 10.1309/AJCP0T1JFYLMPHMI.
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Thymoma arising in the wall of a thymic cyst.起源于胸腺囊肿壁的胸腺瘤。
Pathologica. 2008 Dec;100(6):476-7.
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Cystic thymoma.囊性胸腺瘤
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