Approaches to treatment of unifocal langerhans cell histiocytosis: is biopsy alone enough?

BACKGROUND: Langerhans cell histiocytosis (LCH) is a variable disorder involving either single bone or multiorgan systems. The most effective treatment of unifocal osseous lesions is debated in the literature. This study describes the treatment approaches for LCH and demonstrates the effectiveness of biopsy in providing symptom resolution. METHODS: Records of 61 patients diagnosed with LCH at a single institution over an 11-year period were reviewed. Thirty-nine patients with biopsy-confirmed diagnoses of unifocal osseous LCH were included in the analysis. At this institution, lesions are surgically treated by incisional biopsy, trocar biopsy, or curettage and grafting. Patients receive chemotherapy on a case-by-case basis, depending on the lesion location and size. A Kaplan-Meier analysis was used to compare time with symptom resolution across treatment groups. RESULTS: In the 39 patients with unifocal osseous LCH, treatment approaches included incisional biopsy (n = 18, 46.15%), trocar biopsy (n = 8, 20.51%), incisional biopsy and chemotherapy (n = 8, 20.51%), and biopsy with bone grafting (n = 5, 12.82%). The median time from biopsy to symptom resolution was 5.43 weeks, with an average length of follow-up of 1.59 years. The median time to symptom resolution was 3.86 weeks with incisional biopsy, 5.43 weeks with biopsy and grafting, 5.64 weeks with trocar biopsy, and 16.57 weeks with biopsy and chemotherapy. Overall, there was a significant difference (P = 0.0262) in the time to symptom resolution across the different treatment approaches. Time to symptom resolution was significantly different between incisional biopsy and chemotherapy treatment compared with the incisional biopsy treatment (P = 0.0027), as well as biopsy with grafting treatment (P = 0.0264). CONCLUSIONS: Symptom resolution occurred rapidly after biopsy and did not significantly differ among patients who received incisional biopsy, trocar biopsy, or biopsy with grafting. Unifocal osseous LCH likely does not require aggressive surgical or medical management. Biopsy alone both confirms the diagnosis and precedes a predictable resolution of symptoms. LEVEL OF EVIDENCE: Level III, retrospective comparative therapeutic study.

Bone tumors: osteosarcoma and Ewing's sarcoma.

PURPOSE OF REVIEW: Osteosarcoma and Ewing's sarcoma are the two most common primary malignant bone tumors in children and account for approximately 6% of all childhood malignancies. Treatment methods have seen significant advancements, particularly in regard to chemotherapy and limb-sparing surgery. These advancements have led to increased survival rate. With many long-term survivors, it is important to evaluate long-term patient outcomes following treatment, including function and health-related quality of life. We will review the current trends in treatment of these diseases, different reconstructive options available, and the methods and results for evaluating the long-term results. RECENT FINDINGS: There have been many improvements in the medical treatment of these tumors leading to increasing long-term survival. There have also been improvements in reconstructive techniques for the maintenance of functional extremities in these patients. Newer evaluation methods for both functional outcome and health-related quality of life measures that are more specific to children and adolescents are being developed and in use. SUMMARY: This report will provide an overview of the current treatment options and long-term complications in primary malignant bone tumors for the pediatrician caring for a child with these problems.