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腺纤维瘤样孤立性纤维性肿瘤:发生于鼻窦的一种不常见的形态学变异型。

Adenofibromatous Solitary Fibrous Tumor: An Unusual Morphologic Variant Occurring in the Sinonasal Tract.

机构信息

Department of Pathology, Queen Elizabeth Hospital, 30 Gascoigne Road, Kowloon, Hong Kong, China.

Department of Pathology, Queen Mary Hospital, Hong Kong, China.

出版信息

Head Neck Pathol. 2023 Mar;17(1):165-171. doi: 10.1007/s12105-022-01490-1. Epub 2022 Sep 27.

Abstract

BACKGROUND

Solitary fibrous tumor can exhibit a broad morphologic spectrum, such as presence of epithelioid tumor cells, adipose cells and multinucleated giant cells. This report describes an unusual morphologic variant characterized by adenofibromatous features, all occurring in the sinonasal region.

METHODS

Four cases of the adenofibromatous variant of solitary fibrous tumor were retrieved from the surgical pathology and consultation files in Queen Elizabeth Hospital, Hong Kong. Histologic examination, immunohistochemical study and reverse-transcription polymerase chain reaction (RT-PCR) were performed.

RESULTS

The patients were adults who presented with an obstructive mass of the nasal septum, nasal cavity or nasolacrimal sac. Histologic examination showed a circumscribed biphasic tumor with intermingling of glandular structures and spindle cells, reminiscent of mammary fibroadenoma. Bland-looking spindle cells formed short, irregularly oriented fascicles, admixed with variable amount of collagen fibers. The glandular component comprised ducts and seromucinous acini with a lobular architecture, indicating that it represented exuberant hyperplasia of indigenous glands rather than part of the neoplastic process. Demonstration of CD34 and STAT6 immunoreactivity in the spindle cells and NAB2::STAT6 gene fusion by polymerase chain reaction supports the diagnosis of solitary fibrous tumor.

CONCLUSION

This study reports four cases of sinonasal solitary fibrous tumor with adenofibromatous features, furthermore expanding the morphologic spectrum of this tumor.

摘要

背景

孤立性纤维瘤可表现出广泛的形态学谱,例如存在上皮样肿瘤细胞、脂肪细胞和多核巨细胞。本报告描述了一种发生于鼻-鼻窦部位的不常见的腺纤维瘤样形态变异型。

方法

从香港伊利沙伯医院外科病理学和咨询文件中检索到 4 例孤立性纤维瘤的腺纤维瘤样变异型。进行了组织学检查、免疫组织化学研究和逆转录聚合酶链反应(RT-PCR)。

结果

患者均为成年人,表现为鼻中隔、鼻腔或鼻泪管的阻塞性肿块。组织学检查显示为界限清楚的双相性肿瘤,有腺体结构和梭形细胞交织在一起,类似于乳腺纤维腺瘤。外观温和的梭形细胞形成短而不规则排列的束状,混杂有不同数量的胶原纤维。腺体成分包括导管和浆液黏液性腺泡,具有小叶结构,表明这代表了固有腺体的过度增生,而不是肿瘤过程的一部分。梭形细胞中 CD34 和 STAT6 免疫反应性以及聚合酶链反应中 NAB2::STAT6 基因融合的显示支持孤立性纤维瘤的诊断。

结论

本研究报告了 4 例发生于鼻-鼻窦的具有腺纤维瘤样特征的孤立性纤维瘤,进一步扩大了该肿瘤的形态学谱。

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