The Journal of Bone and Joint Surgery, Vol 76, Issue 10 1513-1525, Copyright © 1994 by Journal of Bone and Joint Surgery, Inc
Treatment of Langerhans-cell histiocytosis in children. Experience at the Children's Hospital of Nancy
S Sessa, D Sommelet, P Lascombes and J Prevot
Department of Pediatric Surgery, Children's Hospital, Nancy, France.
Forty children who had Langerhans-cell histiocytosis were followed for an
average of six years (range, excluding patients who died of the disease,
two to fifteen years). The patients were divided into two diagnostic
groups: those who had localized disease (involving one bone or more only)
and those who had multifocal disease (an osseous lesion and a soft-tissue
mass, a skin rash, diabetes insipidus, or generalized disease). Methods of
treatment included curettage, bone-grafting, chemotherapy, local or
systemic corticosteroids, and radiotherapy. Nineteen of the thirty patients
who had localized disease had a complete response to the therapy, four had
a partial response, and seven had no response. Twenty-one of these thirty
patients had not had a recurrence by the time of the latest follow-up
examination; nine had a local recurrence within four years after the
initial therapy but had no additional recurrences after treatment of the
local recurrence. No recurrence occurred more than four years after the
time that the initial diagnosis had been made. Five of the ten patients who
had multifocal disease had a complete response to the therapy, two had a
partial response, and three had no response. Six patients had a recurrence;
four did not. Two patients died of the disease. As a result of this study,
we recommend the avoidance of intensive measures of treatment, if possible,
and we advise long-term follow-up of these patients.
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