Rupesh Kotecha1, Rajkumar Venkatramani, Rima F Jubran, Alexandre Arkader, Arthur J Olch, Kenneth Wong. 1. *Radiation Oncology Program †Division of Hematology/Oncology ‡Children's Orthopaedic Center, Children's Hospital Los Angeles §Keck School of Medicine, University of Southern California ∥Department of Radiation Oncology, University of California Los Angeles, Los Angeles, CA.
Abstract
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.
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.
Authors: Seong Wook Lee; Hyery Kim; Jin Kyung Suh; Kyung-Nam Koh; Ho Joon Im; Hee Mang Yoon; Jong Jin Seo Journal: Int J Hematol Date: 2017-05-17 Impact factor: 2.490
Authors: James Laird; Jennifer Ma; Karen Chau; Monica Chelius; Weiji Shi; Zhigang Zhang; Benjamin H Lok; Joachim Yahalom Journal: Int J Radiat Oncol Biol Phys Date: 2017-11-06 Impact factor: 7.038