Molecular Basis of Synovial Sarcoma and the Rare Case of its Localization in Palatine Tonsil: A Review of the Literature

N. Chrysanthakopoulos, E. Vryzaki, K. Karkoulias
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Abstract

Synovial sarcomas (SS) are rare soft tissue malignant tumors that mainly arise from the lower extremities, especially the lower thigh-knee region. The literature has shown that this tumor may be observed in uncommon regions such as the abdominal wall and the head and neck region which are non-synovium-lined spaces. SS rarely occurs in the head and neck region, as only 3-10% of these neoplasms appear in this region. The parapharyngeal space is the most common location, whereas primary SS of the palatine tonsil is extremely rare as only a few cases of primary tonsillar SS have been described. It is considered as a high grade malignant tumor, however current investigation regarding its clinical and histopathological features revealed in all these cases a biphasic morphology. Molecular analyses detected a typical SYT gene t(X;18) (p11; q11) translocation and a representative SYT/SSX1fusion type. Immunohistochemical analysis recorded cytokeratin OSCAR, Bcl-2, EMA, vimentin, TLE1 and PGP 9.5, were diffusely positive. The aim of the current review was to present the molecular basis of SS and SS rare location in palatine tonsil.
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滑膜肉瘤的分子基础及其定位于腭扁桃体的罕见病例:文献综述
滑膜肉瘤(SS)是一种罕见的软组织恶性肿瘤,主要发生在下肢,特别是小腿-膝盖区域。文献表明,这种肿瘤可能出现在不常见的区域,如腹壁和头颈部,这些区域是非滑膜排列的间隙。SS很少发生在头颈部,因为只有3-10%的此类肿瘤出现在该区域。咽旁间隙是最常见的位置,而腭扁桃体的原发性SS极为罕见,因为只有少数病例被描述为原发性扁桃体SS。它被认为是一种高度恶性肿瘤,然而目前对其临床和组织病理学特征的调查显示,所有这些病例都有双相形态。分子分析检测到典型的SYT基因t(X;18) (p11;q11)易位和典型的SYT/ ssx1融合型。免疫组化分析显示细胞角蛋白OSCAR、Bcl-2、EMA、vimentin、TLE1、PGP 9.5弥漫性阳性。本文的目的是介绍SS的分子基础和SS在腭扁桃体的罕见位置。
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