Department of Pediatrics, Texas Children's Cancer Center, Baylor College of Medicine, Houston, Texas.
Department of Pathology and Immunology, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas.
Pediatr Blood Cancer. 2018 Oct;65(10):e27291. doi: 10.1002/pbc.27291. Epub 2018 Jun 22.
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that has been associated with EWSR1-CREB1 gene fusion. Outcome in patients with unresectable distant metastases is generally fatal. Interleukin-6 (IL-6) secretion has been described in tumors with EWSR1-CREB1 fusion, and may promote tumor growth due to autocrine stimulation. Tocilizumab is an IL-6 receptor antibody that has potential benefit as a targeted therapy in refractory neoplasms with IL-6 secretion. We describe a child with metastatic AFH with EWSR1-CREB1 fusion and elevated IL-6 whose disease progressed during treatment with traditional chemotherapeutic agents, but improved after targeted therapy with tocilizumab.
血管肌纤维母细胞瘤（AFH）是一种罕见的软组织肿瘤，与 EWSR1-CREB1 基因融合有关。无法切除的远处转移患者的预后通常是致命的。具有 EWSR1-CREB1 融合的肿瘤中已描述了白细胞介素 6（IL-6）的分泌，并且由于自分泌刺激可能促进肿瘤生长。托珠单抗是一种白细胞介素 6 受体抗体，作为具有 IL-6 分泌的难治性肿瘤的靶向治疗具有潜在益处。我们描述了一名患有 EWSR1-CREB1 融合和升高的 IL-6 的转移性 AFH 儿童，其疾病在传统化疗药物治疗期间进展，但在用托珠单抗进行靶向治疗后改善。
