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Pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion: a case report.
Cua, Sean Kendrich N; Odoño, Eugene G; Chan, Kevin Ivan P; Casanova, Dondiego Eleazar G; Guerzon, Aileen; Khu, Kathleen Joy O.
Afiliación
  • Cua SKN; Division of Neurosurgery, Department of Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.
  • Odoño EG; Division of Pathology, Department of Laboratories, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.
  • Chan KIP; Division of Neurosurgery, Department of Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.
  • Casanova DEG; Division of Pathology, Department of Laboratories, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.
  • Guerzon A; Department of Pediatrics, Bicol Medical Center, Naga, Philippines.
  • Khu KJO; Division of Neurosurgery, Department of Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines. kathleen.khu@neurosurgery.ph.
Childs Nerv Syst ; 40(6): 1919-1924, 2024 Jun.
Article en En | MEDLINE | ID: mdl-38459146
ABSTRACT
Pediatric fibromyxoid soft tissue tumors may be associated with gene fusions such as YHWAZPLAG1, with only three reported cases in the literature. We present the fourth case, a 13-year-old male with a pediatric fibromyxoid brachial plexus tumor with YWHAZPLAG1 gene fusion. This is also the first case to be reported in an adolescent, in the brachial plexus, and in the Philippines. The patient presented with a 10-year history of a slowly growing left supraclavicular mass and a 1-year history of intermittent dysesthesia in the left upper extremity. Neurologic examination was unremarkable. Imaging revealed a large left supraclavicular lesion with intrathoracic extension. Surgical excision was performed, and histopathology revealed a fibromyxoid tumor with YWHAZPLAG1 gene fusion. Although previous examples of this gene fusion pointed toward lipoblastoma as their primary pathology, our tumor does not completely fulfill the current diagnostic criteria for a lipoblastoma and may represent an intermediate form of the disease. Our case is unique not only because it is the first reported adolescent patient harboring such a lesion but also because of the relatively lengthy natural history exhibited by the tumor prior to its resection. This provided us with valuable information about its behavior, which suggests a more indolent growth pattern. This case also highlights the clinical importance of molecular testing of tumors, where recognition of disease entities can assist clinicians in deciding and advocating for the proper management.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Plexo Braquial Límite: Adolescent / Humans / Male Idioma: En Revista: Childs Nerv Syst Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: Filipinas Pais de publicación: Alemania
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Plexo Braquial Límite: Adolescent / Humans / Male Idioma: En Revista: Childs Nerv Syst Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: Filipinas Pais de publicación: Alemania