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伴有卵巢样间质的多灶性肺黏液性囊性肿瘤:极为罕见病例报告

Multifocal pulmonary mucinous cystic neoplasm with ovarian-like stroma: Once in a blue moon-case report.

作者信息

Shawash Sarah, Abdeljalil Riad, Haddad Hussam

机构信息

Department of Pathology and Laboratory Medicine, King Hussein Cancer Center, Jordan.

Department of Surgery, King Hussein Cancer Center, Jordan.

出版信息

Respir Med Case Rep. 2019 Feb 16;26:276-280. doi: 10.1016/j.rmcr.2019.02.014. eCollection 2019.

DOI:10.1016/j.rmcr.2019.02.014
PMID:30828544
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6383178/
Abstract

Mucinous cystic neoplasm of the lung is a rare well known entity in the WHO classification of lung tumors. It is defined as "a localized cystic mass filled with mucin and surrounded by a fibrous wall lined by well-differentiated columnar mucinous epithelium." The presence of ovarian-like stroma is not mentioned in this definition. Reviewing the literature, we have found only one reported case mentioning this finding, described by Geramizadeh et al., in 2014. Here we present a similar case of a patient who was initially thought to have lung metastases. Hence, our case is the second one to be reported in the literature. After gross dissection of the received specimens, microscopic examination was performed. The H&E slides revealed that the lesions are composed of non-infiltrative biphasic proliferation of benign mucious epithelial cells forming cysts and gland-like structures with intervening cellular ovarian-like stroma. Differential diagnosis included hamartoma and sclerosing pneumocytoma. Additionally, ancillary studies were performed including cytokeratin AE1/AE3, EMA, TTF-1, MUC5AC, ER, CD10, SMA, CD34, S100, Inhibin, PAS, PAS/D and Mucicarmine. Immunohistochemistry showed Cytokeratin AE1/AE3, EMA and TTF-1 are positive in the epithelial cells and negative in the stromal cells. MUC5AC is weakly positive at the surface of the epithelial cells. The cells of the ovarian-like stroma are positive for ER and CD10 and focally positive for SMA. PAS, PAS/D and Mucicarmine highlighted mucin in the cystic spaces and the surface of epithelial cells. CD34, S100 and Inhibin are negative. Mucinous cystic neoplasm with ovarian-like stroma is a well-known entity in the pancreas and liver. Its occurrence in the lung has been reported in a single case in the English-written literature. We believe that it should be considered in the differential diagnosis of benign biphasic pulmonary lesions. To our knowledge, our case is the second one to be ever reported.

摘要

肺黏液性囊性肿瘤是世界卫生组织肺肿瘤分类中一种罕见但为人熟知的实体。它被定义为“一个充满黏液的局限性囊性肿块,周围有一层由高分化柱状黏液上皮衬里的纤维壁”。该定义中未提及卵巢样间质的存在。查阅文献后,我们仅发现1例报告提及这一发现,由Geramizadeh等人于2014年描述。在此,我们报告1例类似病例,该患者最初被认为患有肺转移瘤。因此,我们的病例是文献中报道的第二例。对接收到的标本进行大体解剖后,进行了显微镜检查。苏木精-伊红(H&E)切片显示,病变由良性黏液上皮细胞的非浸润性双相增殖组成,形成囊肿和腺样结构,其间有细胞性卵巢样间质。鉴别诊断包括错构瘤和硬化性肺细胞瘤。此外,还进行了辅助研究,包括细胞角蛋白AE1/AE3、上皮膜抗原(EMA)、甲状腺转录因子-1(TTF-1)、黏液蛋白5AC(MUC5AC)、雌激素受体(ER)、CD10、平滑肌肌动蛋白(SMA)、CD34蛋白、S100蛋白、抑制素、过碘酸雪夫反应(PAS)、PAS/淀粉酶消化反应(PAS/D)和黏液卡红染色。免疫组织化学显示,细胞角蛋白AE1/AE3、EMA和TTF-1在上皮细胞中呈阳性,在间质细胞中呈阴性。MUC5AC在上皮细胞表面呈弱阳性。卵巢样间质细胞ER和CD10呈阳性,SMA局灶性阳性。PAS、PAS/D和黏液卡红染色突出显示了囊性间隙和上皮细胞表面的黏液。CD34、S100蛋白和抑制素呈阴性。伴有卵巢样间质的黏液性囊性肿瘤在胰腺和肝脏中是一种为人熟知的实体。其在肺中的发生在英文文献中仅报道过1例。我们认为,在良性双相性肺病变的鉴别诊断中应考虑到它。据我们所知,我们的病例是有史以来报道的第二例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/55e588d0641a/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/5a13d2da1e6a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/9a205419e6a4/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/0646e7389950/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/55e588d0641a/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/5a13d2da1e6a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/9a205419e6a4/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/0646e7389950/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5146/6383178/55e588d0641a/gr4.jpg

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本文引用的文献

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Multifocal pulmonary mucinous cystic neoplasm with ovarian type stroma, a new finding in an extremely rare case report.伴有卵巢型间质的多灶性肺黏液性囊性肿瘤,一项极其罕见病例报告中的新发现。
Indian J Pathol Microbiol. 2014 Jan-Mar;57(1):92-3. doi: 10.4103/0377-4929.130910.
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Ovarian-type stroma in hepatobiliary cystadenomas and pancreatic mucinous cystic neoplasms: an immunohistochemical study.肝胆管囊腺瘤和胰腺黏液性囊性肿瘤中的卵巢型间质:一项免疫组织化学研究
Am J Clin Pathol. 2008 Feb;129(2):211-8. doi: 10.1309/U2BBP4EMBAHCM6E6.
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Cystadenomas with ovarian stroma in liver and pancreas: an evolving concept.
肝脏和胰腺中伴有卵巢间质的囊腺瘤:一个不断演变的概念。
Dig Surg. 2006;23(3):186-91. doi: 10.1159/000094488. Epub 2006 Jul 11.