Korean J Pediatr.  2016 Nov;59(Suppl 1):S92-S95. 10.3345/kjp.2016.59.11.S92.

Esthesioneuroblastoma in a boy with 47, XYY karyotype

• Jo HC
• Lee SW
• Jung HJ
• Park JE

• KMID: 2362265
• DOI: http://doi.org/10.3345/kjp.2016.59.11.S92

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.

Keyword

Esthesioneuroblastoma; XYY karyotype; Chemotherapy; Chromosome abnormality

MeSH Terms

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*
Methylphenidate