Silveira Heitor Albergoni, Silva Andréia Aparecida, Dias Rafael Rodrigues, Martins Karina Helen, da Silva Anderson Tangerino Ferreira, Cardoso Camila Lopes, León Jorge Esquiche
Centro Universitário Estácio Ribeirão Preto, Ribeirão Preto, SP Brazil.
Oral Pathology, Department of Stomatology, Public Oral Health, and Forensic Dentistry, School of Dentistry of Ribeirão Preto, University of São Paulo, Avenida do Café, S/N, Ribeirão Preto, SP 14040-904 Brazil.
J Maxillofac Oral Surg. 2025 Jun;24(3):839-843. doi: 10.1007/s12663-025-02480-w. Epub 2025 Feb 9.
Mesenchymal chondrosarcoma (MC) is a rare neoplasm representing about 3% of all chondrosarcomas and 0.1% of all head and neck cancers. To date, about 94 MCs affecting the jaws have been reported. Of them, 28 cases affected pediatric patients, which appear to have a favorable prognosis. Herein, we report an additional maxillary MC.Case Description: A 9-year-old boy was referred presenting an asymptomatic and expansive mass in the left maxilla with six months of evolution. CT scans revealed an expansile osteolytic lesion with granular irregularly shaped calcifications invading the nasal and orbital cavity and infiltrating the maxillary sinus with destruction of its posterolateral wall. Microscopic examination revealed dense sheets of small to medium-sized, poorly differentiated round cells arranged in hemangioperycitoma-like vascular pattern surrounded by nodules of mature cartilage. Immunopositivity for vimentin, S100, and CD99 were observed.Diagnosis and Treatment: The final diagnosis was MC. After neoadjuvant chemotherapy, partial maxillectomy was performed. Currently, with 13 years of follow-up, the patient remains well, with no evidence of recurrence or metastasis.
The current case emphasizes that MC affecting pediatric patients with maxillary location appears to be associated with an indolent course and favorable prognosis.
The online version contains supplementary material available at 10.1007/s12663-025-02480-w.
间叶性软骨肉瘤（MC）是一种罕见的肿瘤，约占所有软骨肉瘤的3%，占所有头颈癌的0.1%。迄今为止，已报道约94例累及颌骨的MC。其中，28例为儿科患者，预后似乎良好。在此，我们报告1例额外的上颌骨MC。
一名9岁男孩因左侧上颌骨出现无症状性膨胀性肿块6个月前来就诊。CT扫描显示一个膨胀性溶骨性病变，伴有颗粒状不规则钙化，侵犯鼻腔和眼眶，并浸润上颌窦，破坏其后外侧壁。显微镜检查显示密集的中小等大小、低分化圆形细胞片，呈血管外皮细胞瘤样血管模式排列，周围有成熟软骨结节。观察到波形蛋白、S100和CD99免疫阳性。
最终诊断为MC。新辅助化疗后，进行了部分上颌骨切除术。目前，经过13年的随访，患者情况良好，无复发或转移迹象。
本病例强调，累及上颌骨的儿科患者的MC似乎病程惰性，预后良好。
在线版本包含可在10.1007/s12663-025-02480-w获取的补充材料。
