N-myc oncogene and urinary catecholamines in children with neuroblastoma.

This paper reports the analysis of N-myc amplification, urinary vanillylmandelic acid (VMA), and norepinephrine (NE) excretion and survival in 22 children with neuroblastoma and 7 with ganglio-neuroblastoma. Five patients had N-myc amplification (from 30 to more than 200 copies), all of whom had advanced-stage disease. The urinary excretion of VMA was normal in all of them, only one showed increased NE excretion. All patients with stage C-D disease and one copy of N-myc had increased VMA urinary excretion and increased (9/14) or normal (5/14) NE urinary excretion. All patients with stage A or B disease had one copy of N-myc. Half of them showed increased VMA urinary levels, while only 3/10 showed increased NE urinary values. Comparison of cumulative survival curves in relation to N-myc amplification and to VMA and NE urinary excretion showed a clear parallelism. Amplification of N-myc corresponded to normal VMA and NE urinary excretion, and was associated with the worst prognosis (P < 0.01), while increased VMA and/or NE excretion was found in patients with only one copy of N-myc.

[Dysplastic gangliocytoma of the cerebellum or Lhermittee-Duclos disease]. / Gangliocitoma displásico de cerebelo o enfermedad de Lhermittee-Duclos.

Lhermitte-Duclós disease is an infrequent condition of which there are only 37 cases described in the literature. Although presently considered as a displasic process, its clinical presentation is of a typical posterior fossa tumour and should be treated as such. Even though it is a benign growth there are only 7 cases described with postoperative survival. The uniform pathological anatomy in all the cases presented makes the definitive diagnosis. The authors present a new case with postoperative survival and discuss its clinical presentation, treatment and origin of the disease.