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舌前部间叶性软骨黏液样肿瘤:病例图像。

Ectomesenchymal Chondromyxoid Tumor of the Anterior Tongue: A Case Image.

机构信息

Department of Pathology, School of Dentistry, Universidade de São Paulo, Av. Prof. Lineu Prestes, 2227 - Cidade Universitária, São Paulo, SP, CEP: 05508-000, Brazil.

Department of Oral Diagnosis and Pathology, School of Dentistry, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.

出版信息

Head Neck Pathol. 2024 Aug 5;18(1):68. doi: 10.1007/s12105-024-01672-z.

Abstract

Ectomesenchymal chondromyxoid tumor (ECT) is a rare soft tissue tumor with peculiar histogenesis, exhibiting a predilection for the dorsum of the tongue. Molecular evidence suggests that it may originate from the migration of ectomesenchymal pluripotent cells from the neural crest to the tongue, where these cells may eventually proliferate and undergo myxoid and chondroid differentiation. This article illustrates a case of a 16-year-old female patient who presented with a nodule on the dorsum of her tongue, which had been present for four years. Surgical excision was performed, and histopathological analysis revealed a myxoid neoplasia composed of polygonal and spindle cells within a loose stroma containing chondroid areas. Tumor cells were positive for GFAP and S-100 proteins on immunohistochemical study, confirming the diagnosis of ECT. After a 5-year follow-up, the patient has shown no evidence of recurrence. Although rare, ECT can be diagnosed straightforwardly due to its distinctive clinical, histopathological, and immunohistochemical features. Clinicians and pathologists should become familiar with this tumor in order to avoid misdiagnosis.

摘要

间叶性软骨黏液样肿瘤(ECT)是一种罕见的软组织肿瘤,具有独特的组织发生学,倾向于发生在舌背。分子证据表明,它可能起源于神经嵴的间充质多能细胞向舌部的迁移,这些细胞可能最终增殖并发生黏液样和软骨样分化。本文介绍了 1 例 16 岁女性患者,其舌背出现结节,已存在 4 年。行手术切除,组织病理学分析显示,黏液样肿瘤由多边形和梭形细胞组成,位于富含软骨样区域的疏松基质中。免疫组织化学研究显示肿瘤细胞阳性表达 GFAP 和 S-100 蛋白,证实了 ECT 的诊断。5 年随访后,患者无复发迹象。尽管罕见,但由于其独特的临床、组织病理学和免疫组织化学特征,ECT 可以直接诊断。临床医生和病理学家应熟悉这种肿瘤,以避免误诊。

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