Oral oncology · 2025
Mesenchymal chondrosarcoma of the Jaw: A case report and 25-Year review of the literature.
Клинический случайСтатьяСистематический обзорНазвание и аннотация: Перевод пока не подготовлен
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Cueva-Cañolaa Luis E, Avellaneda Suárez Manuel M, Miura Machuca Jesus M, Cornejo Ochoa Ximena Rosario, Aliag Rolig, Castilla Leonardo Rangel, La Madrid Barreto Ana Karina, Gamez Mauricio E
Аннотация
BACKGROUND: Mesenchymal chondrosarcoma (MCS) of the jaws is a rare and aggressive malignancy characterized by biphasic histology, high recurrence rates, and poor long-term prognosis. Due to its low incidence, clinical features and optimal treatment strategies remain poorly defined.
METHODS: We conducted a systematic review of the literature from January 2000 to July 2025, in accordance with PRISMA 2020 guidelines, and included all case reports, case series, and studies providing individual-level data on MCS of the maxilla or mandible. Articles with sufficient diagnostic, therapeutic, and follow-up information were eligible. Additionally, we report a case diagnosed and managed at our institution.
RESULTS: A total of 55 patients were included (median age: 28 years; 47.3 % male). The maxilla was the most frequent site (63.6 %), with pain, swelling, and dental mobility being the most common initial manifestations. Immunohistochemistry frequently showed positivity for CD99 (64.7 %) and S100 (58.8 %). Surgery was the predominant therapeutic modality (46.2 %), followed by surgery combined with chemotherapy (23.1 %) and multimodal approaches (28.7 %). At a median follow-up of 31.2 months, 76.1 % of patients remained in clinical improvement, though recurrence (13.9 %) and distant metastasis (20.0 %) were common. Disease-specific mortality was 23.9 %.
CONCLUSION: MCS of the jaws predominantly affects young adults, with the maxilla being the most common site. Although multimodal treatment offers the best chance of disease control, recurrence and metastasis remain frequent. Early diagnosis, aggressive surgical management, and individualized therapy are essential to improve outcomes.
METHODS: We conducted a systematic review of the literature from January 2000 to July 2025, in accordance with PRISMA 2020 guidelines, and included all case reports, case series, and studies providing individual-level data on MCS of the maxilla or mandible. Articles with sufficient diagnostic, therapeutic, and follow-up information were eligible. Additionally, we report a case diagnosed and managed at our institution.
RESULTS: A total of 55 patients were included (median age: 28 years; 47.3 % male). The maxilla was the most frequent site (63.6 %), with pain, swelling, and dental mobility being the most common initial manifestations. Immunohistochemistry frequently showed positivity for CD99 (64.7 %) and S100 (58.8 %). Surgery was the predominant therapeutic modality (46.2 %), followed by surgery combined with chemotherapy (23.1 %) and multimodal approaches (28.7 %). At a median follow-up of 31.2 months, 76.1 % of patients remained in clinical improvement, though recurrence (13.9 %) and distant metastasis (20.0 %) were common. Disease-specific mortality was 23.9 %.
CONCLUSION: MCS of the jaws predominantly affects young adults, with the maxilla being the most common site. Although multimodal treatment offers the best chance of disease control, recurrence and metastasis remain frequent. Early diagnosis, aggressive surgical management, and individualized therapy are essential to improve outcomes.