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    BIOMARKER:

    RANBP2-ALK fusion

    i
    Other names: RANBP2, RAN Binding Protein 2, NUP358, Acute Necrotizing Encephalopathy 1 (Autosomal Dominant), Nuclear Pore Complex Protein Nup358, E3 SUMO-Protein Ligase RanBP2, Ran-Binding Protein 2, 358 KDa Nucleoporin, Nucleoporin Nup358, Nucleoporin 358, ADANE, P270, E3 SUMO-Protein Transferase RanBP2, Transformation-Related Protein 2, RanBP2, IIAE3, ANE1, TRP1, TRP2, NBLST3, CD246, Anaplastic Lymphoma Kinase, Anaplastic Lymphoma Kinase (Ki-1), CD246 Antigen, Mutant Anaplastic Lymphoma Kinase, ALK, ALK Re
    Entrez ID:
    238; 5903
    Related biomarkers:
    Expression
    Mutation
    CNA
    Fusion
    Others
    2years
    Correlation Analysis of Initial Methylation Stratification and Clinical Characteristics in Juvenile Myelomonocytic Leukemia (ASH 2022)
    The clinical characteristics of HM group are closely related to the currently recognized adverse risk factors of JMML. Due to the small number of cases, the prognosis of different methylation levels and the correlation with treatment and intervention still need to be further studied.
    Clinical
    |
    KRAS (KRAS proto-oncogene GTPase) • ALK (Anaplastic lymphoma kinase) • NRAS (Neuroblastoma RAS viral oncogene homolog) • ARID1A (AT-rich interaction domain 1A) • NF1 (Neurofibromin 1) • TET2 (Tet Methylcytosine Dioxygenase 2) • PTPN11 (Protein Tyrosine Phosphatase Non-Receptor Type 11) • FAT1 (FAT atypical cadherin 1) • RANBP2 (RAN Binding Protein 2) • JAK3 (Janus Kinase 3) • SETBP1 (SET Binding Protein 1)
    |
    KRAS mutation • NRAS mutation • HER-2 mutation • NF1 mutation • ALK fusion • PTPN11 mutation • NRAS G13 • JAK3 mutation • RANBP2-ALK fusion
    3years
    Primary Cutaneous Epithelioid Inflammatory Myofibroblastic Sarcoma Harboring RANBP2-ALK Fusion: Report of an Exceptional Case. (PubMed, Am J Dermatopathol)
    To date, EIMS has been reported almost exclusively in the abdominal and pelvic cavity, with the exception of some intrathoracic cases. Herein, we present the first case of primary cutaneous EIMS, confirmed by molecular analysis showing the diagnostic RANBP2-ALK fusion.
    Clinical • Journal
    |
    ALK (Anaplastic lymphoma kinase) • RANBP2 (RAN Binding Protein 2)
    |
    ALK fusion • RANBP2-ALK fusion
    3years
    Epithelioid inflammatory myofibroblastic sarcomas are not exclusive to ventral cavity sites. (PubMed, Histopathology)
    Exceptionally rare cases have been reported in the thoracic cavity (3 in lung/pleura) and pelvic cavity (1 in ovary) (4-7). To our knowledge, EIMS has not been reported outside the ventral cavity.
    Journal
    |
    ALK (Anaplastic lymphoma kinase) • EML4 (EMAP Like 4) • RANBP2 (RAN Binding Protein 2)
    |
    EML4-ALK fusion • ALK fusion • RANBP2-ALK fusion
    over3years
    [VIRTUAL] Acute Myeloid Leukemia With Myelodysplasia-Related Changes Harboring RAN-Binding Protein 2 (RANBP2)–Anaplastic Lymphoma Kinase (ALK) Fusion Gene: Case Report and Review of the Literature (CAP 2021)
    The patient died of refractory disease after 10 months of treatment with azacytidine and venetoclax. It is frequently associated with monocytic differentiation, monosomy 7, and dismal prognosis. Additional studies are warranted for better understanding of the independent impact of RANBP2-ALK fusion on the etiopathogenesis of myeloid neoplasia and utility of crizotinib treatment.
    Review • Clinical
    |
    ALK (Anaplastic lymphoma kinase) • TNFRSF8 (TNF Receptor Superfamily Member 8) • RANBP2 (RAN Binding Protein 2) • CD14 (CD14 Molecule) • ITGAM (Integrin, alpha M)
    |
    ALK rearrangement • ALK fusion • RANBP2-ALK fusion
    |
    FoundationOne® Heme CDx
    |
    Venclexta (venetoclax) • Xalkori (crizotinib) • azacitidine
    over3years
    Uterine Inflammatory Myofibroblastic Neoplasms With Aggressive Behavior, Including an Epithelioid Inflammatory Myofibroblastic Sarcoma: A Clinicopathologic Study of 9 Cases. (PubMed, Am J Surg Pathol)
    IMT referral cases were initially diagnosed as smooth muscle tumors in 87.5% of cases; while the EIMS was diagnosed as high-grade endometrial stromal sarcoma. Lack of consideration of IMT in the differential diagnosis of smooth muscle tumors with myxoid features can result in misdiagnosis and under-utilization of targeted therapy in these patients.
    Clinical • Journal
    |
    ALK (Anaplastic lymphoma kinase) • RANBP2 (RAN Binding Protein 2) • DCTN1 (Dynactin Subunit 1)
    |
    ALK positive • ALK rearrangement • ALK fusion • RANBP2-ALK fusion
    over3years
    [VIRTUAL] Loss of 5'ALK leads to better response to crizotinib in sarcomas with ALK rearrangement. (ASCO 2021)
    We report here 4 sarcomas with ALK rearrangement, including a novel fusion TMEM217-ALK . Two of these cases had a 5'ALK loss in addition to the 3'ALK rearrangement, and both had much longer PFS on Crizotinib therapy . The underlying mechanism deserved further investigation .
    ALK (Anaplastic lymphoma kinase) • EML4 (EMAP Like 4) • RANBP2 (RAN Binding Protein 2) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2) • TMEM217 (Transmembrane Protein 217)
    |
    ALK positive • ALK rearrangement • EML4-ALK fusion • ALK fusion • RANBP2-ALK fusion • PLEKHH2-ALK rearrangement • RANBP2-ALK + TMEM217-ALK
    |
    VENTANA ALK (D5F3) CDx Assay
    |
    Xalkori (crizotinib)
    almost4years
    Clinicopathological features of inflammatory myofibroblastic tumor (PubMed, Zhonghua Bing Li Xue Za Zhi)
    Twenty-two patients were alive with no evidence of tumor, four patients had tumor recurrences (three patients were treated with crizotinib and were alive with tumor), and three patients died of the disease (including two patients with EIMS). IMTs show a wide morphologic spectrum, and should be differentiated form a variety of benign or malignant tumors. Immunohistochemistry (ALKp80, ALKD5F3) and FISH (ALK break-apart probe) could assist the diagnosis of IMT, with NGS recommended for the atypical cases.
    Clinical • Journal
    |
    ALK (Anaplastic lymphoma kinase) • RANBP2 (RAN Binding Protein 2)
    |
    ALK rearrangement • ALK fusion • RANBP2-ALK fusion
    |
    VENTANA ALK (D5F3) CDx Assay
    |
    Xalkori (crizotinib)
    almost4years
    Characteristic nuclear membrane ALK reactivity in chronic myelomonocytic leukemia with RANBP2-ALK fusion. (PubMed, Am J Hematol)
    No abstract available
    Journal
    |
    RANBP2 (RAN Binding Protein 2)
    |
    ALK fusion • RANBP2-ALK fusion