Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Esthesioneuroblastoma in a boy with 47, XYY karyotype

  • Jo, Hee Cheol (Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine) ;
  • Lee, Seong Wook (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Jung, Hyun Joo (Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine) ;
  • Park, Jun Eun (Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine)
  • 투고 : 2015.07.20
  • 심사 : 2015.10.12
  • 발행 : 2016.11.15
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초록

Neuroblastomas are sometimes associated with abnormal constitutional karyotypes, but the XYY karyotype has been rarely described in neuroblastomas. Here, we report a case of an esthesioneuroblastoma in a boy with a 47, XYY karyotype. A 6-year-old boy was admitted to our hospital because of nasal obstruction and palpable cervical lymph node, which he first noticed several days previously. A polypoid mass in the right nasal cavity was detected through sinuscopy. Biopsy of the right nasal polyp was performed. Based on the result, the patient was diagnosed with a high-grade esthesioneuroblastoma. Nuclear imaging revealed increased uptake in both the right posterior nasal cavity and the right cervical IB-II space, suggesting metastatic lymph nodes. Cytogenetic analysis revealed a 47, XYY karyotype. Twelve courses of concurrent chemotherapy were administered. Three years after the completion of chemotherapy, the patient had had no disease recurrence. He manifested behavioral violence and temper tantrums, so we started methylphenidate for correction of the behavior.

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참고문헌

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