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[Neonatal Pepper's syndrome. Apropos of a series of 22 cases]
C Labreveux de Cervens1, O Hartmann, D Valteau
1Département de Pédiatrie, Institut Gustave-Roussy, Villejuif.
Insights
Neonatal Pepper syndrome, a rare cancer in newborns, has a favorable prognosis. Early and accurate treatment leads to a high survival rate, even in aggressive forms.
Area of Science:
- Pediatric Oncology
- Neonatal Medicine
- Rare Cancers
Context:
- Pepper syndrome is a rare cancer affecting neonates.
- Neonatal cases constituted 26% of all Pepper syndrome cases treated between 1960-1987.
- The study reviewed 22 neonatal cases from Institut Gustave-Roussy.
Purpose:
- To evaluate the treatment outcomes and prognosis of Pepper syndrome in neonates.
- To analyze the impact of disease progression and treatment strategies on survival rates.
Summary:
- Of 22 neonates with Pepper syndrome, 12 had rapidly evolving disease requiring aggressive treatment, with 4 early deaths.
- Ten neonates had slowly progressive disease; 5 achieved remission without metastatic treatment.
- The overall relapse-free survival rate was 77% with an 8-year median follow-up.
Impact:
- Despite aggressive forms, neonatal Pepper syndrome has a favorable prognosis with timely, accurate treatment.
- Early intervention is crucial for improving survival outcomes in affected neonates.
- This study highlights the importance of prompt diagnosis and tailored treatment approaches.
Abstract:
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.