S. Sessa et al., TREATMENT OF LANGERHANS-CELL HISTIOCYTOSIS IN CHILDREN - EXPERIENCE AT THE CHILDRENS-HOSPITAL OF NANCY, Journal of bone and joint surgery. American volume, 76A(10), 1994, pp. 1513-1525
Forty children, who had Langerhans-cell histiocytosis were followed fo
r an average of six: Sears (range, excluding patients who died of the
disease, two to fifteen gears). The patients were divided into two dia
gnostic groups: those who had localized disease (involving one bone or
more only) and those who had multifocal disease (an osseous lesion an
d a soft-tissue mass, a skin rash, diabetes insipidus, or generalized
disease). Methods of treatment included curettage, bone grafting, chem
otherapy, local or systemic corticosteroids, and radiotherapy. Ninetee
n of the thirty patients who had localized disease had a complete resp
onse to the therapy four had a partial response, and seven had no resp
onse. Twenty-one of these thirty patients had not had a recurrence by
the time of the latest follow-up examination; nine had a local recurre
nce within four years after the initial therapy but had no additional
recurrences after treatment of the local recurrence. No recurrence occ
urred more than four years after the time that the initial diagnosis h
ad been made. Five of the ten patients who had multifocal disease had
a complete response to the therapy, two had a partial response, and th
ree 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.