Abstract
Infantile fibrosarcoma is a malignant tumor originating from fibroblasts. Histologically it resembles adult fibrosarcoma but shows distinctive epidemiological, molecular, and prognostic characteristics.
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Notes
- 1.
ETV6-NTRK3 fusion gene: ETV6 encodes an ETS family transcription factor associated with leukemia and congenital fibrosarcoma. NTRK3 encodes neurotrophic receptor tyrosine kinase 3 and belongs to the tropomyosin receptor kinases (TRK) family which also includes NTRK1 (encoding neurotrophic receptor tyrosine kinase 1) and NTRK2 (neurotrophic receptor tyrosine kinase 2). The encoded proteins elicit activities that regulate the natural growth, differentiation, and survival of neurons when they interact with endogenous neurotrophin ligands. Chromosomal rearrangements involving in-frame fusions of these genes with various partners, translocations in the TRK kinase domains, mutations in the TRK ligand-binding site, amplifications of NTRK, or the expression of TRK splice variants can result in constitutively activated chimeric TRK fusion proteins that can act as oncogenic drivers that promote cell proliferation and survival in tumor cell lines.
- 2.
Larotrectinib: tyrosine kinase inhibitor targeting the following: NTRK1, NTRK2, NTRK3.
- 3.
Entrectinib: tyrosine kinase inhibitor targeting the following: NTRK1, NTRK2, NTRK3, ROS1, ALK.
Suggested Readings
Fletcher (2020) WHO classification of tumours of soft tissue and bone, 5th ed.
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Kheder (2018) emerging targeted therapy for tumors with NTRK Fusion Proteins. Clin Cancer Res 24(23):5807–5814
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Orbach (2016) Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience. Eur J Cancer 57:1–9
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Mocellin, S. (2021). Infantile Fibrosarcoma. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_130
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DOI: https://doi.org/10.1007/978-3-030-58710-9_130
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