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CTNNB1 somatic mutations drive Wnt pathway activation in a case of incidental intranodal palisaded myofibroblastoma
Germán Moreno de Juan1, Santiago Montes Moreno1
1Anatomic Pathology Service, Hospital Universitario Marqués de Valdecilla/IDIVAL, Universidad de Cantabria, Santander, Spain; Translational Hematopathology Lab, IDIVAL, Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Santander, Spain.
Abstract:
Intranodal palisaded myofibroblastoma (IPM) is a rare stroma-derived spindle-cell neoplasm of the lymph node with myofibroblastic differentiation and CTNNB1 (β-catenin gene) somatic mutations. We present a case of IPM found incidentally in the staging of lung adenocarcinoma. We describe the major histopathological and phenotypic features, including a palisaded bland spindle cell proliferation with myofibroblastic differentiation and Wnt pathway activation by immunohistochemistry, including β-catenin expression. Production of osteoid-like collagen directly from tumor cells was observed. We confirmed p.Gly34Arg CTNNB1 mutation by direct sequencing. We also reviewed the literature for similar cases.
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