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  • Open Access

    VIEWPOINT

    Non-canonical BRAF variants and rearrangements in hairy cell leukemia

    STEPHEN E. LANGABEER*

    Oncology Research, Vol.32, No.9, pp. 1423-1427, 2024, DOI:10.32604/or.2024.051218 - 23 August 2024

    Abstract Hairy cell leukemia (HCL) is an uncommon mature B-cell malignancy characterized by a typical morphology, immunophenotype, and clinical profile. The vast majority of HCL patients harbor the canonical BRAF V600E mutation which has become a rationalized target of the subsequently deregulated RAS-RAF-MEK-MAPK signaling pathway in HCL patients who have relapsed or who are refractory to front-line therapy. However, several HCL patients with a classical phenotype display non-canonical BRAF mutations or rearrangements. These include sequence variants within alternative exons and an oncogenic fusion with the IGH gene. Care must be taken in the molecular diagnostic work-up of patients More >

  • Open Access

    REVIEW

    Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment

    PAULINA CHMIEL1,2, ALEKSANDRA SłOWIKOWSKA1,2, ŁUKASZ BANASZEK1,2, ANNA SZUMERA-CIEćKIEWICZ3, BARTłOMIEJ SZOSTAKOWSKI1, MATEUSZ J. SPAłEK1,4,*, TOMASZ ŚWITAJ1, PIOTR RUTKOWSKI1, ANNA M. CZARNECKA1

    Oncology Research, Vol.32, No.7, pp. 1141-1162, 2024, DOI:10.32604/or.2024.050350 - 20 June 2024

    Abstract Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate. Diagnostic challenges arise from the diverse pathological presentation, variable symptomatology, and lack of different imaging features. However, IMT is identified by the fusion of the anaplastic lymphoma kinase (ALK) gene, which is present in approximately 70% of cases, with various fusion partners, including ran-binding protein 2 (RANBP2), which allows confirmation of the diagnosis. While surgery is the preferred approach for localized tumors, the optimal long-term treatment for advanced or metastatic disease is difficult… More >

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