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    GENE:

    PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2)

    i
    Other names: PLEKHH2, Pleckstrin Homology, MyTH4 And FERM Domain Containing H2, Pleckstrin Homology Domain Containing, Family H (With MyTH4 Domain) Member 2, Pleckstrin Homology Domain-Containing Family H Member 2 ,PLEKHH1L,
    12d
    A Giant Thoracic ALK-Rearranged Mesenchymal Neoplasm in a Child. (PubMed, Cancer Rep (Hoboken))
    Our findings indicate that ALK-rearranged mesenchymal neoplasms with fibrosarcoma-like features, particularly those associated with elevated mitotic activity or tumor necrosis, should be classified as high grade in pathology reports. In addition, this case also demonstrated that neoadjuvant therapy may be a better treatment strategy compared to upfront surgery for ALK-rearranged mesenchymal neoplasms with a relatively high tumor burden.
    Journal
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    ALK (Anaplastic lymphoma kinase) • CD34 (CD34 molecule) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2)
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    ALK rearrangement • ALK fusion
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    Alecensa (alectinib)
    9ms
    Case Report: ALK-rearranged mesenchymal neoplasms with S100 and CD34 co-expression: additional cases with distinct characteristics. (PubMed, Front Oncol)
    In case 1, no indications of disease progression or metastasis was observed within the limited follow-up (36 months). However, the patient in case 2 experienced a rapid recurrence and metastasis.
    Journal
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    ALK (Anaplastic lymphoma kinase) • STRN (Striatin) • CD34 (CD34 molecule) • NTRK (Neurotrophic receptor tyrosine kinase) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2)
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    ALK rearrangement • ALK fusion
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    VENTANA ALK (D5F3) CDx Assay
    over1year
    Epithelioid Fibrous Histiocytoma Is on a Continuum With Superficial ALK-rearranged Myxoid Spindle Cell Neoplasm: A Clinicopathologic Series of 35 Cases Including Alternate RET and NTRK3 Fusions. (PubMed, Am J Surg Pathol)
    SAMS is on a morphologic and molecular genetic spectrum with EFH, with a similar body site distribution, frequent clinical presentation as an exophytic skin tumor, and invariably benign outcomes; we conclude that SAMS should be considered a histologic variant of EFH. Some morphologically typical examples harbor alternate RET and NTRK3 fusions, such that SAMS is not an appropriate designation for this morphologic class; instead, to highlight the clinicopathologic similarities to EFH, we propose the diagnostic term "myxoid spindle cell variant of epithelioid fibrous histiocytoma."
    Journal
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    ALK (Anaplastic lymphoma kinase) • RET (Ret Proto-Oncogene) • NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • EML4 (EMAP Like 4) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • CDKN2B (Cyclin Dependent Kinase Inhibitor 2B) • CD34 (CD34 molecule) • NCOA4 (Nuclear Receptor Coactivator 4) • DCTN1 (Dynactin Subunit 1) • SQSTM1 (Sequestosome 1) • MPRIP (Myosin Phosphatase Rho Interacting Protein) • SPECC1L (Sperm Antigen With Calponin Homology And Coiled-Coil Domains 1 Like) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2) • PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
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    NTRK3 fusion • RET fusion • ALK rearrangement • ALK fusion • CDKN2A deletion • NCOA4-RET fusion
    over1year
    ALK-rearranged, CD34-positive spindle cell neoplasms resembling dermatofibrosarcoma protuberans: a study of seven cases. (PubMed, Histopathology)
    We describe a cohort of novel ALK-rearranged tumours with morphologic features similar to DFSP.
    Journal
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    ALK (Anaplastic lymphoma kinase) • CD34 (CD34 molecule) • DCTN1 (Dynactin Subunit 1) • PDGFB (Platelet Derived Growth Factor Subunit B) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2)
    over1year
    ALK-rearranged mesenchymal neoplasms: a clinicopathological and molecular study of eight additional cases of an emerging group of tyrosine kinase fusion mesenchymal tumours. (PubMed, J Clin Pathol)
    Our study expands the clinicopathological and molecular spectrum of ALK-rearranged mesenchymal neoplasms.
    Journal
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    ALK (Anaplastic lymphoma kinase) • EML4 (EMAP Like 4) • CD34 (CD34 molecule) • MMP2 (Matrix metallopeptidase 2) • CLIP1 (CAP-Gly Domain Containing Linker Protein 1) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2)
    2years
    CD30 expression in an emerging group of mesenchymal spindle cell neoplasms with ALK fusion detected by flow cytometry and immunohistochemistry. (PubMed, Genes Chromosomes Cancer)
    The patient showed significant tumor response to single-agent treatment with alectinib, an ALK-tyrosine kinase inhibitor...The benefit of the early detection of CD30 expression by FCM for a prompt diagnosis and treatment is highlighted in the context of an aggressive clinical course. This case represents a learning experience regarding the need to the check the status of CD30 expression in these tumors and suggests the potential clinical benefits of CD30-targeted therapy.
    Journal
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    BRAF (B-raf proto-oncogene) • ALK (Anaplastic lymphoma kinase) • TNFRSF8 (TNF Receptor Superfamily Member 8) • CD34 (CD34 molecule) • NTRK (Neurotrophic receptor tyrosine kinase) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2)
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    ALK rearrangement • ALK fusion • TNFRSF8 expression • CD34 positive • NTRK fusion
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    Alecensa (alectinib)
    over2years
    A Rare Case of Uterine High-Grade Spindle Cell Sarcoma With PLEKHH2::ALK Fusion in 31-Year-Old Woman (CAP 2023)
    This fusion has been reported to be sensitive to the ALK inhibitor Alectinib...The incidence of mesenchymal tumors harboring ALK rearrangements is increasing. To our knowledge only 3 cases of mesenchymal tumors with PLEKHH2::ALK fusion have been reported in the literature so far.
    Clinical
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    CCND1 (Cyclin D1) • NCAM1 (Neural cell adhesion molecule 1) • MME (Membrane Metalloendopeptidase) • CD99 (CD99 Molecule) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2) • SYP (Synaptophysin)
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    ALK rearrangement • ALK fusion
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    Alecensa (alectinib)
    over2years
    Comprehensive analysis to identify a novel diagnostic marker of lung adenocarcinoma and its immune infiltration landscape. (PubMed, Front Oncol)
    The mechanism of LUAD pathogenesis and progression is closely linked to T cells, B cells, and monocytes. 12 HUB genes(ADAMTS8, CD36, DPYSL2, FABP4, FGFR4, HBA2, OCIAD2, PARP1, PLEKHH2, STX11, TCF21, TNNC1) may participate in the progression of LUAD via immune-related signaling pathways.
    Journal • PARP Biomarker
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    FGFR4 (Fibroblast growth factor receptor 4) • PARP1 (Poly(ADP-Ribose) Polymerase 1) • CD36 (thrombospondin receptor) • CD4 (CD4 Molecule) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2) • DPYSL2 (Dihydropyrimidinase Like 2) • FABP4 (Fatty Acid Binding Protein 4)
    over3years
    PLEKHH2 binds β-arrestin1 through its FERM domain, activates FAK/PI3K/AKT phosphorylation, and promotes the malignant phenotype of non-small cell lung cancer. (PubMed, Cell Death Dis)
    Furthermore, the dissociation of FAK promotes its autophosphorylation, activates the PI3K/AKT signaling pathway, and subsequently promotes lung cancer cell proliferation, migration, and invasion. These results provide evidence for the potential use of PLEKHH2 inhibition as an anticancer therapy.
    Journal
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    PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2)
    over3years
    ALK-rearranged Mesenchymal Neoplasms: A Report of 9 Cases Further Expanding the Clinicopathologic Spectrum of Emerging Kinase Fusion Positive Group of Tumors. (PubMed, Genes Chromosomes Cancer)
    Of the 4 patients with follow-up (median 5.5 months), 1 remained alive with stable disease and 3 were alive without disease. We expand the clinicopathologic spectrum of ALK-fused mesenchymal neoplasms, including a low-grade malignant peripheral nerve sheath tumor-like subset, and another subset characterized by epithelioid and high-grade morphology.
    Journal
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    ALK (Anaplastic lymphoma kinase) • EML4 (EMAP Like 4) • CD34 (CD34 molecule) • DCTN1 (Dynactin Subunit 1) • SOX10 (SRY-Box 10) • KLC1 (Kinesin light chain 1) • PLEKHH2 (Pleckstrin Homology, MyTH4 And FERM Domain Containing H2) • TIMP3 (TIMP Metallopeptidase Inhibitor 3)
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    ALK positive • ALK rearrangement • ALK fusion