Advances and Challenges in the Multidisciplinary Treatment of Retroperitoneal Infantile Fibrosarcoma in Early Life.

BACKGROUND AND AIMS: Retroperitoneal Infantile Fibrosarcoma (RIFS) is a rare, locally aggressive pediatric soft tissue tumor. Its retroperitoneal location poses challenges due to proximity and potential invasion of critical structures such as the inferior vena cava (IVC). This study aims to illustrate the range of multidisciplinary management strategies and evolving therapeutic approaches.

METHODS

We conducted a retrospective case series of infants diagnosed with RIFS and treated at our tertiary center. Clinical presentation, imaging findings, histopathology, treatment modalities (including chemotherapy, surgery, interventional radiology, targeted therapies), and follow-up data were analyzed.

RESULTS

Three infants with histologically confirmed RIFS were identified, with a median age at diagnosis of 2 months (range: 0-8 months). Diagnosis was established through open surgical biopsy. Management was individualized and multimodal, including neoadjuvant chemotherapy, surgical resection, interventional radiology procedures for symptom control, and, in the most recent case, targeted molecular therapy guided by genetic profiling.

CONCLUSIONS

Management of RIFS has significantly evolved over the last decade. Advances in surgical techniques combined with the integration of interventional radiology and novel targeted therapies allow a nuanced balance between maximizing oncological control and minimizing surgical morbidity. Multidisciplinary individualized treatment is essential to optimize outcomes in this rare and challenging pediatric tumor.