Cardiac Synovial Sarcoma in Children: A Case Report and Literature Review

Résumé

Background: Synovial sarcomas are rare malignant mesenchymal tumors, primarily observed in adolescents and young adults. These tumors are predominantly located in the extremities. Cardiac involvement is exceptionally rare, infrequently reported in the literature, and presents diagnostic, therapeutic, and prognostic challenges.

Case Presentation: We report a case of cardiac synovial sarcoma in a 12-year-old child, located in the right ventricle and measuring 67 mm at its largest dimension. Histological and immunohistochemical analyses confirmed the diagnosis. The extension workup was negative. The tumor was resected by morcellation, and chemotherapy with an ifosfamide–doxorubicin regimen was initiated, combined with adjuvant radiotherapy. The patient is currently alive but presents with a recurrence at the initial site after 23 months.

Conclusion: Cardiac synovial sarcomas are rarely documented in the literature. Although rare, it is crucial to consider this diagnosis in the presence of a malignant cardiac tumor with biphasic or spindle cell morphology.