Intimal sarcoma, review and future perspectives

Intimal sarcoma is an exceptionally rare and aggressive vascular malignancy that remains frequently misdiagnosed. This review synthesizes recent advances in epidemiology, diagnosis, molecular characterization and treatment, highlighting why updated guidance is urgently needed.

Current literature emphasizes persistent diagnostic delays due to nonspecific clinical and radiological features. Molecular studies consistently show recurrent amplification of MDM2, PDGFRA and CDK4 as key oncogenic drivers. Surgical resection remains the only potentially curative option, although complete removal is often challenging. Retrospective data suggest that multimodal therapy, particularly anthracycline-based chemotherapy, may improve outcomes in selected patients. Targeted therapies and immunotherapy have shown limited but emerging activity in moleculary defined subgroups.

Despite incremental progress, prognosis remains poor, underscoring the need for earlier recognition, molecularly guided treatment strategies and clinical trial participation. Management in specialized multidisciplinary centers is essential to optimized care and advance research in this ultra-rare sarcoma.