Maxillary Mesenchymal Chondrosarcoma Affecting A Pediatric Patient: Case Report Showing Long-Term Follow-Up and Detailed Literature Review

Author(s)

Silveira, Heitor Albergoni ; Silva, Andreia Aparecida ; Dias, Rafael Rodrigues ; Martins, Karina Helen ; da Silva, Anderson Tangerino Ferreira ; Cardoso, Camila Lopes ; Leon, Jorge Esquiche

• Document type: Journal article
• Source: JOURNAL OF MAXILLOFACIAL & ORAL SURGERY; v. N/A, p. 5-pg., 2025-02-09.

Abstract

IntroductionMesenchymal 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.IntroductionMesenchymal 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.IntroductionMesenchymal 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. ConclusionThe current case emphasizes that MC affecting pediatric patients with maxillary location appears to be associated with an indolent course and favorable prognosis. (AU)

• FAPESP's process: 18/12734-2 - Comparative immunohistochemical characterization of dendritic cell subgroups and viral oncogenesis in oral and oropharyngeal squamous cell carcinoma
• Grantee: Heitor Albergoni da Silveira
• Support Opportunities: Scholarships in Brazil - Master
• FAPESP's process: 16/11419-0 - Clinicopathological correlation of the degree of differentiation, tetraspanin expression, viral agents and cell proliferation in a large sample of squamous cell carcinoma of the head and neck region.
• Grantee: Jorge Esquiche León
• Support Opportunities: Regular Research Grants
• FAPESP's process: 22/12760-9 - Clinicopathological correlation of the immune system, cell differentiation, angiogenesis, lipid metabolism and transcriptionally active HPV in a large series of head and neck squamous cell carcinomas
• Grantee: Jorge Esquiche León
• Support Opportunities: Regular Research Grants