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Vol. 56. Issue 4.
Pages 279-283 (October - December 2023)
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Vol. 56. Issue 4.
Pages 279-283 (October - December 2023)
Brief report
CTNNB1 somatic mutations drive Wnt pathway activation in a case of incidental intranodal palisaded myofibroblastoma
Mutaciones somáticas de CTNNB1 inducen la activación de la vía Wnt en un caso incidental de miofibroblastoma en empalizada de localización intraganglionar
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Germán Moreno de Juana,b, Santiago Montes Morenoa,b,
Corresponding author
santiago.montes@scsalud.es

Corresponding author.
a Anatomic Pathology Service, Hospital Universitario Marqués de Valdecilla/IDIVAL, Universidad de Cantabria, Santander, Spain
b Translational Hematopathology Lab, IDIVAL, Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Santander, Spain
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Table 1. Summary of epidemiologic data, localization, size, immunohistochemical and molecular study and outcome, from articles published to date.
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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.

Keywords:
Intranodal palisaded myofibroblastoma
Spindle-cell
CTNNB1
Beta-catenin
Wnt pathway
Resumen

El miofibroblastoma en empalizada intraganglionar linfático (MEIG) es una neoplasia infrecuente de células fusiformes del estroma del ganglio linfático con diferenciación miofibroblástica y mutaciones en CTNNB1 (gen de la β-catenina). Aquí mostramos el caso de un paciente con MEIG encontrado incidentalmente en la estadificación por un adenocarcinoma de pulmón. Se describen las características histopatológicas principales de la entidad, incluyendo una proliferación de células fusiformes con escasa atipia, empalizadas celulares y diferenciación miofibroblástica con activación de la vía Wnt, incluyendo expresión inmunohistoquímica de β-catenina. Se observó producción de colágeno de tipo osteoide por parte de las células tumorales. Se confirmó la presencia de la mutación p.Gly34Arg de CTNNB1 mediante secuenciación directa. Se recogen adicionalmente publicaciones de casos similares al nuestro.

Palabras clave:
Miofibroblastoma en empalizada intraganglionar linfático
Celulas fusiformes
CTNNB1
β-catenina
Vía Wnt

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