Locoregional MYCN-amplified neuroblastoma.
MYCN-amplification is strongly associated with other high-risk prognostic factors and poor outcome in neuroblastoma. Infrequently, amplification of MYCN has been identified in localized tumors with favorable biologic features. Outcome for these children is difficult to predict and optimal treatment strategies remain unclear. We report a 5-month-old who presented with an MYCN-amplified INSS stage 3, pelvic neuroblastoma. The tumor had favorable histology, hyperdiploidy, and lacked 1p36 and 11q23 aberrations. Although the patient met the criteria for high-risk neuroblastoma, because of the discordant prognostic markers we elected to treat her according to an intermediate-risk protocol. She remains event-free more than 18 months.
Department of Pediatrics, University of Chicago, Chicago, Illinois 60637, USA.
Andres
A
Morales La Madrid
Samuel
S
Volchenboum
Julie M
JM
Gastier-Foster
Robert
R
Pyatt
Don
D
Liu
Peter
P
Pytel
Cinzia
C
Lavarino
Eva
E
Rodriguez
Susan L
SL
Cohn
D005260:Female; D005784:Gene Amplification; D006801:Humans; D007223:Infant; D000071447:N-Myc Proto-Oncogene Protein; D009447:Neuroblastoma / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D009687:Nuclear Proteins / Q000235:genetics*; D015513:Oncogene Proteins / Q000235:genetics*; D010386:Pelvic Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D011379:Prognosis
C459801:MYCN protein, human; D000071447:N-Myc Proto-Oncogene Protein; D009687:Nuclear Proteins; D015513:Oncogene Proteins