Therapeutic response of metastatic angiomatoid fibrous histiocytoma carrying EWSR1‐CREB1 fusion to the interleukin‐6 receptor antibody tocilizumab

• Published in: Pediatric blood & cancer Vol. 65; no. 10; pp. e27291 - n/a
• Main Authors: Potter, Samara L., Quintanilla, Norma M., Johnston, Danielle K., Naik‐Mathuria, Bindi, Venkatramani, Rajkumar
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.10.2018
• Subjects: angiomatoid fibrous histiocytoma; Antibodies, Monoclonal, Humanized - therapeutic use; Antineoplastic Agents, Immunological - therapeutic use; Autocrine signalling; Chemotherapy; Child, Preschool; Cytokines; DiGeorge Syndrome - complications; EWSR1‐CREB1; FLI-1 protein; Gene fusion; Hematology; Histiocytoma; Histiocytoma, Malignant Fibrous - complications; Histiocytoma, Malignant Fibrous - drug therapy; Histiocytoma, Malignant Fibrous - genetics; Humans; IL‐6 receptor antibody; Immunosuppressive agents; interleukin‐6; Male; Medical treatment; Metastases; Metastasis; Monoclonal antibodies; Neoplasia; Oncogene Proteins, Fusion - genetics; Oncology; Pediatrics; Soft Tissue Neoplasms - complications; Soft Tissue Neoplasms - drug therapy; Soft Tissue Neoplasms - genetics; tocilizumab; Tumors; angiomatoid fibrous histiocytoma; tocilizumab; EWSR1-CREB1; interleukin-6; IL-6 receptor antibody

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...