[Surgical treatment of Langerhans cell histiocytosis with bone and soft tissue involvement in pediatric patients].

OBJECTIVES We evaluated pediatric patients who underwent surgery for Langerhans cell histiocytosis with mono- or polyostatic involvement and an extensive soft tissue component and/or a high risk for pathologic fractures. METHODS The study included 13 children (6 boys, 7 girls; mean age 6 years; range 2 to 11 years) who were treated surgically for bone and soft tissue involvement of Langerhans cell histiocytosis with a high risk for fractures of the long bones and the pelvis. Eleven patients had only skeletal involvement while three patients had multisystem involvement. There were 17 bone lesions in the following localizations: acetabulum (n=7), femur neck and subtrochanteric region-proximal femur (n=5), and proximal (n=2) and distal (n=2) humerus. One patient had multifocal bone involvement. The risk for pathologic fractures was assessed using the scoring system of Mirels. Treatment consisted of surgery followed by chemotherapy. The results of treatment were evaluated according to the criteria by the Study Group of Langerhans Cell Histiocytosis. The mean follow-up after orthopedic surgery was 67 months (range 34 to 172 months). RESULTS Only one patient developed recurrence, in which case bone marrow involvement responded well to chemotherapy. Radiographically, graft consolidation was observed in all the patients in the third postoperative month. No bone sequelae such as shortness and deformity were detected throughout the follow-up period. CONCLUSION Surgical treatment combined with chemotherapy is effective in the treatment of aggressive histiocytic bone lesions associated with extensive soft tissue involvement and high risk for fractures.