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

Pediatr Blood Cancer. 2018 Oct;65(10):e27291. doi: 10.1002/pbc.27291. Epub 2018 Jun 22.

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.

Keywords: EWSR1-CREB1; IL-6 receptor antibody; angiomatoid fibrous histiocytoma; interleukin-6; tocilizumab.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Antineoplastic Agents, Immunological / therapeutic use*
  • Child, Preschool
  • DiGeorge Syndrome / complications
  • Histiocytoma, Malignant Fibrous / complications
  • Histiocytoma, Malignant Fibrous / drug therapy*
  • Histiocytoma, Malignant Fibrous / genetics
  • Humans
  • Male
  • Oncogene Proteins, Fusion / genetics
  • Soft Tissue Neoplasms / complications
  • Soft Tissue Neoplasms / drug therapy*
  • Soft Tissue Neoplasms / genetics

Substances

  • Antibodies, Monoclonal, Humanized
  • Antineoplastic Agents, Immunological
  • EWSR1-CREB1 fusion protein, human
  • Oncogene Proteins, Fusion
  • tocilizumab

Supplementary concepts

  • Histiocytoma, Angiomatoid Fibrous