Treatment Results of Langerhans Cell Histiocytosis: A Long Term Follow-Up

Abstract

Purpose : To investigate the long-term treatment outcome of patients of Langerhans cell histiocytosis (LCH). Materials and Methods : Twenty patients with the diagnosis of LCH between January 1978 and December 2002 were included in our study. Median age was 12 years. Treatment included surgery, chemotherapy, radiotherapy, combination of multiple modalities and observation. The median follow-up is 87 months. Results : The 5-year overall and progression-free survival rates for all patients were 100% and 92%, respectively. The 5-year progression-free survival rates for patients treated with and without radiotherapy were 100% and 83%, respectively (p = 0.317). The overall local control rate was 87% at five years. Of these, the 5-year local control rates for stage 1, 2, 3, and 4 were 100%, 100%, 75%, and 50%, respectively (p = 0.695). The 5-year local control rates for patients treated with and without radiotherapy were 75% and 100%, respectively (p= 0.065, despite a good response to local radiotherapy). The 5-year local control rates for age ≤ 25 and ＞ 25 years were 100% and 50%, respectively (p = 0.0007). Conclusion : All of our LCH patients had a very high survival regardless of the treatment modalities. This suggests that less aggressive treatments may be the reasonable initial therapy to avoid side effects. The choice of treatment depends on the extent of disease. It seems that multiple bone or neurological involvement did not significantly influence survival. There is no difference in the 5-year local control and survival rates between stage 1 and 2.