American journal of clinical oncology · 2014
Clinical outcomes of radiation therapy in the management of Langerhans cell histiocytosis.
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Kotecha Rupesh, Venkatramani Rajkumar, Jubran Rima F, Arkader Alexandre, Olch Arthur J, Wong Kenneth
Аннотация
OBJECTIVES: Langerhans cell histiocytosis (LCH) is a rare disease with variable clinical presentation. In the present study, we report on the effectiveness and clinical complications of radiation therapy in children with LCH.
MATERIALS AND METHODS: We retrospectively reviewed all patients with LCH treated with radiation therapy over a 6-decade period at a single institution. Radiotherapy data, clinical features, radiographic data, and vital status were analyzed.
RESULTS: The mean age at diagnosis for 69 patients was 5.3 years (3 mo to 37 y) and the median duration of follow-up was 6 years (7 d to 32 y). Radiation therapy was performed for 169 sites, primarily bone lesions. The median radiotherapy dose was 10 Gy (2.5 to 45 Gy). Radiographic follow-up data were available for 139 of the sites treated and clinical follow-up was available for 156 of sites treated. The radiographic local control was 91.4%, and 13% of lesions showed complete sclerosis or reconstitution of bone. A total of 90.4% of patients reported stabilization or improvement in lesion-related symptoms, most often pain. Twelve patients had diabetes insipidus at diagnosis or during follow-up. Eight of these patients received radiation treatment to the pituitary and none experienced a reduction in desmopressin dosage posttreatment. Radiation complications were few, including femoral neck fracture in 1 patient and facial asymmetry in 3 patients. No secondary malignancies were observed.
CONCLUSIONS: Radiotherapy for LCH has high rates of local control and symptomatic improvement. Importantly, however, there is evidence of short-term and long-term morbidity when children are treated with low-dose irradiation.
MATERIALS AND METHODS: We retrospectively reviewed all patients with LCH treated with radiation therapy over a 6-decade period at a single institution. Radiotherapy data, clinical features, radiographic data, and vital status were analyzed.
RESULTS: The mean age at diagnosis for 69 patients was 5.3 years (3 mo to 37 y) and the median duration of follow-up was 6 years (7 d to 32 y). Radiation therapy was performed for 169 sites, primarily bone lesions. The median radiotherapy dose was 10 Gy (2.5 to 45 Gy). Radiographic follow-up data were available for 139 of the sites treated and clinical follow-up was available for 156 of sites treated. The radiographic local control was 91.4%, and 13% of lesions showed complete sclerosis or reconstitution of bone. A total of 90.4% of patients reported stabilization or improvement in lesion-related symptoms, most often pain. Twelve patients had diabetes insipidus at diagnosis or during follow-up. Eight of these patients received radiation treatment to the pituitary and none experienced a reduction in desmopressin dosage posttreatment. Radiation complications were few, including femoral neck fracture in 1 patient and facial asymmetry in 3 patients. No secondary malignancies were observed.
CONCLUSIONS: Radiotherapy for LCH has high rates of local control and symptomatic improvement. Importantly, however, there is evidence of short-term and long-term morbidity when children are treated with low-dose irradiation.