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

Samara L. Potter, Norma M. Quintanilla, Danielle K. Johnston, Bindi Naik-Mathuria, Rajkumar Venkatramani

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

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 languageEnglish (US)
Article numbere27291
JournalPediatric Blood and Cancer
Volume65
Issue number10
DOIs
StatePublished - Oct 2018
Externally publishedYes

Keywords

  • angiomatoid fibrous histiocytoma
  • EWSR1-CREB1
  • IL-6 receptor antibody
  • interleukin-6
  • tocilizumab

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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