Pediatric Spinal Solitary Fibrous Tumor: A Systematic Review of a Rare Condition.

Abstract

Background: Spinal solitary fibrous tumors (SFTs) are a rare oncological entity, almost anecdotal in the pediatric population. They have a high relapse rate and represent an ongoing oncological challenge. Methods: In this article, we conducted a systematic review starting from a case report to highlight the current state of the art in managing these tumors. Results: Spinal solitary fibrous tumors (SFTs) are rare, slow-growing neoplasms that can be either intra- or extramedullary. Only a limited number of studies focus on primary pediatric spinal cord localization. Five pediatric cases of spinal SFT have been documented in the literature. On MRI, they typically present as highly vascularized, contrast-enhancing masses. Histologically, they are composed of spindle-shaped cells within a collagenous stroma featuring staghorn-shaped blood vessels. More aggressive subtypes, such as dedifferentiated SFTs, resemble high-grade sarcomas. The NAB2-STAT6 fusion is a key marker, driving EGFR signaling, collagen production, and fibrosis. Additional diagnostic markers include CD34, CD99, and Bcl-2. Surgical resection remains the primary treatment. In metastatic cases, chemotherapy-mainly with anthracyclines, dacarbazine, or temozolomide-is employed, although no standardized pediatric protocols exist. Anti-angiogenic agents, including tyrosine kinase inhibitors, have shown promise. Radiotherapy is used postoperatively for local disease control, but its impact on survival is still under investigation. Conclusions: Surgery remains the cornerstone of treatment, significantly impacting the natural history of the disease and symptom control. While clinical trials exploring radiotherapy and chemotherapy are ongoing in adults, no specific treatment protocol has been established for pediatric patients.