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儿童上颌骨软骨黏液样纤维瘤:免疫组化分析及文献复习。

Chondromyxoid fibroma affecting the maxilla in a 1-year-old child: Immunohistochemical analysis and literature review.

机构信息

Department of Pediatric Dentistry - Riberão Preto Dental School (FORP/USP), University of São Paulo, Ribeirão Preto, São Paulo, Brazil.

Oral Pathology, Department of Stomatology, Public Oral Health, and Forensic Dentistry, Ribeirão Preto Dental School (FORP/USP), University of São Paulo, Ribeirão Preto, São Paulo, Brazil.

出版信息

Oral Oncol. 2022 Jan;124:105467. doi: 10.1016/j.oraloncology.2021.105467. Epub 2021 Jul 24.

Abstract

Chondromyxoid fibroma (CMF) is a benign chondroid/myxoid matrix-producing tumor that often develops in the long bones of young adults. CMF is rarely reported in the craniofacial skeleton, with most cases presenting with bone erosion or destruction, which may lead to a misdiagnosis. To date, approximately 129 cases of CMF in the craniofacial region have been reported, with only three cases in patients aged less than 1 year. Of these 129 cases, only 34 affected the jaws. A 1-year-old boy presented with a mass in the left anterior maxilla, extending and compressing the ipsilateral nasal cavity. After surgical excision of the lesion, microscopy revealed spindle-to-stellate tumor cells surrounded by a predominant myxoid stroma containing focal slit-like vascular channels and hemorrhagic areas. Immunohistochemistry showed positivity for vimentin, CD10, and α-SMA (focal). The Ki-67 labeling index was 6%. CFM should be included in the differential diagnosis when assessing maxillary tumors in pediatric patients.

摘要

软骨黏液样纤维瘤(CMF)是一种良性软骨/黏液样基质产生的肿瘤,常发生于年轻成人的长骨。CMF 在颅面骨骼中很少见报道,大多数病例表现为骨侵蚀或破坏,这可能导致误诊。迄今为止,已报道约 129 例发生于颅面区域的 CMF,其中仅 3 例发生于年龄小于 1 岁的患者。在这 129 例患者中,只有 34 例累及颌骨。1 岁男童因左上颌前份肿物就诊,肿物向同侧鼻腔延伸并压迫。病变切除后显微镜下观察显示梭形至星状肿瘤细胞被以黏液样基质为主的间质包围,其中含有局灶裂隙状血管通道和出血区。免疫组织化学显示波形蛋白、CD10 和 α-SMA(局灶阳性)。Ki-67 标记指数为 6%。在评估儿科患者上颌部肿瘤时,应将 CFM 纳入鉴别诊断。

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