Esthesioneuroblastoma in a boy with 47, XYY karyotype.
10.3345/kjp.2016.59.11.S92
- Author:
Hee Cheol JO
1
;
Seong Wook LEE
;
Hyun Joo JUNG
;
Jun Eun PARK
Author Information
1. Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Suwon, Korea. pedpje@ajou.ac.kr
- Publication Type:Case Report
- Keywords:
Esthesioneuroblastoma;
XYY karyotype;
Chemotherapy;
Chromosome abnormality
- MeSH:
Biopsy;
Child;
Chromosome Aberrations;
Cytogenetic Analysis;
Drug Therapy;
Esthesioneuroblastoma, Olfactory*;
Humans;
Karyotype;
Lymph Nodes;
Male*;
Methylphenidate;
Nasal Cavity;
Nasal Obstruction;
Nasal Polyps;
Neuroblastoma;
Recurrence;
Violence;
XYY Karyotype*
- From:Korean Journal of Pediatrics
2016;59(Suppl 1):S92-S95
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
