Abstract:
BACKGROUND: Langerhans cell histiocytosis (LCH) in children is
relatively rare, and the long-term analysis of therapy results
has not been done yet in Hungary. PURPOSE: In this review we
summarise the incidence, clinical features, prognostic risk
factors and treatment results of children's LCH in Hungary,
using data from the National Childhood Cancer Registry in
Hungary in a 20-year period between 1981 and 2000. RESULTS: From
January 1981 to December 2000, 111 children under 18 years of
age were newly diagnosed with LCH in Hungary. The male-female
ratio was 1.36:1, the mean age: 4 years 11 months. The minimal
and median follow-up time was 3.48 years and 10.98 years
respectively. 38 children had single-system disease, while in 73
cases we found systemic dissemination already at the time of
diagnosis. Twenty-two patients were treated only by local
surgery, 7 by surgery with local irradiation and 5 children
received only local irradiation. In two cases remission was
obtained with local steroid administration. 75 patient received
chemotherapy. During the twenty years 14 children died, 9 due to
the progression of the disease. Sixteen of the 111 patients had
relapse with a mean of 2.16+/-1.29 years after the first
diagnosis. Three patients with relapse got chemotherapy
generally used in lymphoma and remission was achieved. The
overall survival of all patients (n=111) was 88.3+/-3.1% at 5
years and 87.3+/-3.2% at 10 and 20 years. CONCLUSION: Childhood
LCH is a well treatable disease and the survival rate is high.
Even disseminated diseases have a quite good prognosis in
childhood.
