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唾液腺孤立性胸膜外纤维瘤:我们的经验——病例系列及文献综述

Solitary Extrapleural Fibrous Tumor in Salivary Glands: Our Experience-Case Series and Literature Review.

作者信息

Boschetti Ciro Emiliano, Vitagliano Rita, Imola Gianmaria, Cornacchini Nicola, Colella Maria Luisa, Tartaro Gianpaolo, Colella Giuseppe

机构信息

Oral and Maxillofacial Surgery Unit, Multidisciplinary Department of Medical-Surgical and Dental Special- 8 ties, University of Campania "Luigi Vanvitelli", Via Luigi de Crecchio, 6, 80138 Naples, Italy.

出版信息

Diagnostics (Basel). 2022 Nov 4;12(11):2688. doi: 10.3390/diagnostics12112688.

Abstract

(1) Background: Extrapleural solitary fibrous tumors (ESFTs) are rare oncological entities occurring in the head and neck, and even more so in the salivary glands. The clinical presentation and histologic features are usually unspecific, resulting in frequent misclassification. As an unusual tumor, ESTFs have an unpredictable clinical behavior. (2) Methods: We present two clinical cases referred to our Maxillofacial Surgery Unit for the onset of a symptomless mass involving, in one case, the parotid gland, and in the other case, the sublingual gland. (3) Results: Solitary fibrous tumors could be considered as neoplasms with intermediate biological behavior that are not entirely predictable on the basis of morphological features, as these are mostly still unknown. However, a few histologic, immunohistochemical, and imaging features, such as a hypodense signal at the T1 sequence in an MRI, or positivity for CD34, bcl2, and CD99, and the NAB2-STATS6 fusion gene, could be useful for an early differential diagnosis of ESTFs. (4) Conclusions: All patients were alive at follow-up with no evidence of disease. Surgical management should always be considered as the first choice for oncological radicality, and clinical behavior should always be defined with the help of the study of radiological and anatomopathological features.

摘要

(1) 背景:胸膜外孤立性纤维瘤(ESFTs)是发生于头颈部的罕见肿瘤实体,在唾液腺中更为少见。其临床表现和组织学特征通常不具有特异性,常导致分类错误。作为一种不常见的肿瘤,ESFTs具有不可预测的临床行为。(2) 方法:我们报告两例临床病例,均因无症状肿块就诊于我们的颌面外科,一例累及腮腺,另一例累及舌下腺。(3) 结果:孤立性纤维瘤可被视为具有中间生物学行为的肿瘤,基于形态学特征无法完全预测其行为,因为这些特征大多仍不明确。然而,一些组织学、免疫组化和影像学特征,如MRI的T1序列低信号,或CD34、bcl2和CD99阳性以及NAB2-STATS6融合基因,可能有助于ESFTs的早期鉴别诊断。(4) 结论:随访时所有患者均存活,无疾病证据。手术治疗应始终被视为肿瘤根治的首选方法,且应借助放射学和解剖病理学特征研究来明确临床行为。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/03fe/9688987/81e38c5833ff/diagnostics-12-02688-g001.jpg

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