Paratesticular liposarcomas: A rare but crucial diagnosis. Case series and review of literature

睾丸旁脂肪肉瘤:一种罕见但至关重要的诊断。病例系列及文献综述

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Abstract

INTRODUCTION: Soft tissue sarcomas arise from mesenchymal tissue, with liposarcomas being the largest subgroup. These malignancies are classified into five subtypes by the WHO: atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLPS), dedifferentiated liposarcoma (DDLPS), myxoid liposarcoma, pleomorphic liposarcoma, and myxoid pleomorphic liposarcoma. WDLPS and DDLPS are the most prevalent, the latter being associated with higher metastatic rates (15-30%) and a 5-year mortality of 28-30% if metastases are present. This report discusses three cases of rare paratesticular liposarcomas, a subgroup originating from the tunica vaginalis and spermatic cord. MATERIAL AND METHODS: Case 1 involved a 62-year-old man with a scrotal mass diagnosed as WDLPS after extensive examinations. Case 2 was a 72-year-old man with testicular swelling. Pathology revealed ALT/WDLPS with dedifferentiated zones, necessitating further wide resection due to positive margins. Case 3 described a 63-year-old man with a ductus deferens nodule diagnosed as WDLPS. Negative margins were achieved, and follow-up showed no recurrence. RESULTS: Diagnosis of liposarcoma remains challenging due to overlapping imaging characteristics with benign conditions. Advanced modalities like positron emission tomography - computed tomography and magnetic resonance imaging can aid in differentiating liposarcomas based on metabolic activity and tissue characteristics. Surgical resection with negative margins remains the gold standard for treatment. Recurrence risks increase with positive margins and dedifferentiated histology. Adjuvant radiotherapy and chemotherapy show limited efficacy, emphasizing the importance of precision in surgical and pathological evaluation. Emerging therapies targeting the MDM2 and CDK4 pathways show promise for advanced or recurrent cases. CONCLUSIONS: This report highlights the complexity of diagnosing and managing paratesticular liposarcomas, underlining the importance of multimodal approaches for improved outcomes.

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