| Literature DB >> 29932283 |
Samara L Potter1, Norma M Quintanilla2, Danielle K Johnston1, Bindi Naik-Mathuria3, Rajkumar Venkatramani1.
Abstract
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.Entities:
Keywords: EWSR1-CREB1; IL-6 receptor antibody; angiomatoid fibrous histiocytoma; interleukin-6; tocilizumab
Mesh:
Substances:
Year: 2018 PMID: 29932283 DOI: 10.1002/pbc.27291
Source DB: PubMed Journal: Pediatr Blood Cancer ISSN: 1545-5009 Impact factor: 3.167