[Neonatal Pepper's syndrome. Apropos of a series of 22 cases].
From 1960 to 1987, 22 neonates were treated at the Institut Gustave-Roussy for a Pepper' syndrome. During this period of time, 83 children were treated for the same syndrome, the neonatal type representing 26% of cases. Twelve of the 22 neonates presented with the rapidly evolving type, requiring an immediate aggressive treatment. In 4 of them death occurred early despite treatment. The 10 other neonates presented with a slowly progressive disease. In 5 of them treatment of the metastatic disease was not necessary to obtain remission. The survival rate of disease without relapses in 77%, with a median follow-up of 8 years. So, despite the high incidence of rapidly progressive forms, the prognosis of this disease remains favorable in neonates, provided an accurate treatment initiated early.
Labreveux de Cervens
C
C
Département de Pédiatrie, Institut Gustave-Roussy, Villejuif.
Hartmann
O
O
Valteau
D
D
Brugières
L
L
de Cervens
T
T
Kalifa
C
C
Patte
C
C
Flamant
F
F
Lemerle
J
J
D000310:Adrenal Gland Neoplasms / Q000473:pathology* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000556:secondary* / Q000628:therapy; D008297:Male; D009382:Neoplasms, Unknown Primary / Q000473:pathology*; D009447:Neuroblastoma / Q000556:secondary* / Q000628:therapy; D011379:Prognosis; D013577:Syndrome; D013899:Thoracic Neoplasms
Syndrome de Pepper néonatal. A propos d'une série de 22 cas.