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一名来自埃塞俄比亚的12岁儿童下颌牙槽黏膜原发性平滑肌肉瘤:病例报告

Primary Leiomyosarcoma of the Mandibular Alveolar Mucosa of a 12-Year-Old Child from Ethiopia: A Case Report.

作者信息

Kenea Tewodros Tefera, Kebede Betel Abebe, Gozjuze Fekadu Mesele, Kiros Hagos, Wilde Frank

机构信息

Department of Cranio-Maxillofacial and Oral Surgery, Yekatit 12 Hospital Medical College, Addis Ababa, Ethiopia.

Department of Pediatrics and Child Health, Addis Ababa University, Addis Ababa, Ethiopia.

出版信息

Craniomaxillofac Trauma Reconstr. 2017 Mar;10(1):56-59. doi: 10.1055/s-0036-1582459. Epub 2016 Apr 27.

Abstract

Leiomyosarcomas (LMSs) are rare malignant mesenchymal tumors which show smooth-muscle differentiation. Most LMSs involving the oral tissues primarily affect the maxillary sinus, the maxillary or mandibular bone. We present a case of LMS of the mandibular alveolar mucosa, arising in a 12-year-old male child from Ethiopia. A malignant spindle cell-like neoplasm was diagnosed on clinical and radiographic findings as well as on incisional biopsy. The tumor was resected with wide margins. The following histopathologic examination with additional immunohistochemical studies secured the diagnosis LMS. Microscopically, the spindle-shaped tumor cells were arranged in an interlacing fascicular pattern and contained oval to elongated, blunt-ended (cigar-shaped) nuclei. The immunohistochemical examination showed immunoreactive tumor cells for vimentin, actin, desmin, and H-caldesmon, which is pathognomonic for LMS. Immunohistochemical studies are mandatory to differentiate the LMS from other similar spindle cell neoplastic lesions. Radical resection with safety margins and a lifelong periodic follow-up has to be recommended.

摘要

平滑肌肉瘤(LMSs)是一种罕见的具有平滑肌分化的恶性间叶组织肿瘤。大多数累及口腔组织的LMSs主要影响上颌窦、上颌骨或下颌骨。我们报告一例发生于一名12岁埃塞俄比亚男童下颌牙槽黏膜的LMS。根据临床、影像学表现以及切开活检诊断为恶性梭形细胞样肿瘤。肿瘤行广泛边缘切除。随后的组织病理学检查及额外的免疫组化研究确诊为LMS。显微镜下,梭形肿瘤细胞呈交错束状排列,细胞核呈椭圆形至细长形、钝端(雪茄形)。免疫组化检查显示肿瘤细胞对波形蛋白、肌动蛋白、结蛋白和H-钙调蛋白呈免疫反应性,这是LMS的特征性表现。免疫组化研究对于鉴别LMS与其他类似的梭形细胞肿瘤性病变至关重要。建议行带安全边缘的根治性切除并进行终身定期随访。

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