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

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...

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Published inPediatric 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
LanguageEnglish
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
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Summary: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.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
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ObjectType-Report-1
ObjectType-Article-3
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.27291