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.
| Original language | English (US) |
|---|---|
| Article number | e27291 |
| Journal | Pediatric Blood and Cancer |
| Volume | 65 |
| Issue number | 10 |
| DOIs | |
| State | Published - Oct 2018 |
| Externally published | Yes |
Keywords
- EWSR1-CREB1
- IL-6 receptor antibody
- angiomatoid fibrous histiocytoma
- interleukin-6
- tocilizumab
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Hematology
- Oncology
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