Advances and Challenges in the Multidisciplinary Treatment of Retroperitoneal Infantile Fibrosarcoma in Early Life.
Gaia Brunetti, Angelo Zarfati, Giorgio Persano et al.
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Case in brief
This retrospective case series describes three infants with histologically confirmed retroperitoneal infantile fibrosarcoma, diagnosed at a median age of 2 months (range: 0-8 months). Diagnosis was established by open surgical biopsy. Because the tumors were locally aggressive and situated near critical structures, management was individualized and multimodal, incorporating neoadjuvant chemotherapy, surgical resection, and interventional radiology procedures for symptom control. In the most recent case, genetic profiling guided targeted molecular therapy. The report describes evolving multidisciplinary approaches but does not provide individual outcomes or details of how each case resolved.
What made this case unusual
The retroperitoneal location creates management challenges because of the tumor’s proximity to, and potential invasion of, critical structures such as the inferior vena cava; the series also includes targeted therapy guided by genetic profiling in its most recent case.
Diagnostic / clinical pearl
In an infant with a retroperitoneal mass, histopathologic confirmation is central to diagnosing infantile fibrosarcoma; treatment planning may require coordinated input across specialties because of the tumor’s location and local aggressiveness.
Why it matters
The cases illustrate how individualized combinations of surgery, chemotherapy, interventional radiology, and molecularly guided therapy may help balance oncological control with surgical morbidity.