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中胚叶性软骨黏液样肿瘤:与不对称性软腭裂的罕见关联。

Ectomesenchymal Chondromyxoid Tumor: A Rare Association With an Asymmetrical Soft Palate Cleft.

机构信息

Mediclinic Midstream Hospital, Pretoria, Gauteng, South Africa.

Facial Cleft Deformity Clinic, Department of Maxillofacial and Oral Surgery, 56410University of Pretoria, Pretoria, Gauteng, South Africa.

出版信息

Cleft Palate Craniofac J. 2022 Jul;59(7):932-937. doi: 10.1177/10556656211035029. Epub 2021 Aug 30.

Abstract

Ectomesenchymal chondromyxoid tumor (ECT) is a rare oral lesion first described by Smith et al. in 1995. These tumors are typically painless, slow growing and benign masses occurring predominantly on the anterior tongue dorsum. Prior to this seminal report, many ECTs may have been misdiagnosed due to the histological similarities with other lesions. Immunohistochemical stains aid in definitive diagnosis of an ECT. A total of 39 papers since published have reported 96 patients with ECT. Most lesions involve the anterior aspect of the tongue, with only 6 occurring in the posterior tongue and 2 involving the hard palate. ECTs are considered to develop from ectomesenchymal cells of neural crest cells that have migrated to the tongue during embryological development. This paper is of a rare case of ECT of the posterolateral tongue occurring in association with an unusual asymmetrical soft palate cleft. It is postulated that since the tongue develops before the formation of the soft palate, an ECT lesion occurring on the posterior aspect may have a causal contribution to the development of the soft palate cleft.

摘要

间叶性软骨黏液样肿瘤(ECT)是一种罕见的口腔病变,最初由 Smith 等人于 1995 年描述。这些肿瘤通常为无痛、生长缓慢的良性肿块,主要发生在前舌背。在此开创性报告之前,由于与其他病变的组织学相似,许多 ECT 可能被误诊。免疫组织化学染色有助于明确 ECT 的诊断。自那时以来,总共发表了 39 篇论文,报告了 96 例 ECT 患者。大多数病变涉及舌前部分,只有 6 例发生在舌后部分,2 例涉及硬腭。ECT 被认为是从中胚层细胞的神经嵴细胞迁移到舌的间叶细胞发展而来的。本文报道了一例罕见的舌后外侧 ECT 病例,与不寻常的不对称软腭裂有关。据推测,由于舌在软腭形成之前发育,因此发生在后舌的 ECT 病变可能对软腭裂的发展有因果关系。

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