Trezza Andrea, Rui Chiara B, Chiaravalli Stefano, Biassoni Veronica, Schiavello Elisabetta, Vennarini Sabina, Orlandi Ester, Carrabba Giorgio G, Massimino Maura, Giussani Carlo G
Neurosurgery, Fondazione IRCCS San Gerardo dei Tintori, Via Pergolesi 33, 20900 Monza, Italy.
Pediatrics, Fondazione IRCCS Istituto Nazionale dei Tumori, 20133 Milan, Italy.
Children (Basel). 2025 Sep 10;12(9):1214. doi: 10.3390/children12091214.
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. 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. 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. 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.
脊柱孤立性纤维瘤（SFTs）是一种罕见的肿瘤实体，在儿科人群中几乎鲜有报道。它们具有较高的复发率，是当前肿瘤治疗面临的一项挑战。在本文中，我们从一份病例报告开始进行系统综述，以突出这类肿瘤治疗的当前技术水平。脊柱孤立性纤维瘤（SFTs）是罕见的、生长缓慢的肿瘤，可位于髓内或髓外。仅有有限数量的研究聚焦于原发性小儿脊髓定位。文献中已记录了5例小儿脊柱SFT病例。在磁共振成像（MRI）上，它们通常表现为高度血管化、有对比增强的肿块。组织学上，它们由胶原基质内的梭形细胞组成，具有鹿角状血管。更具侵袭性的亚型，如去分化SFTs，类似于高级别肉瘤。NAB2 - STAT6融合是一个关键标志物，驱动表皮生长因子受体（EGFR）信号传导、胶原蛋白生成和纤维化。其他诊断标志物包括CD34、CD99和Bcl - 2。手术切除仍然是主要治疗方法。在转移性病例中，主要使用化疗药物——蒽环类药物、达卡巴嗪或替莫唑胺，尽管尚无标准化的儿科治疗方案。包括酪氨酸激酶抑制剂在内的抗血管生成药物已显示出前景。放疗用于术后局部疾病控制，但其对生存率的影响仍在研究中。手术仍然是治疗的基石，对疾病的自然病程和症状控制有重大影响。虽然针对成人的放疗和化疗临床试验正在进行，但尚未为儿科患者制定特定的治疗方案。
