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• W2896472722 abstract "Langerhans cell histiocytosis (LCH) is a rare disease characterized by histiocytic proliferation with varied clinical presentations in both children and adults. Although treatment guidelines exist for systemic therapy, there is limited evidence to guide selection of treatment modality (radiation, systemic therapy, excision, or observation) among the many presentations of LCH, particularly in limited, single-system LCH involvement. Here we report treatment modality selection and outcome in a large single-institution database of this rare disease, and the role of radiation therapy (RT) in the treatment of single-system LCH. We hypothesize that RT provides good local control in single-system LCH disease. We identified 184 patients with histologically confirmed LCH who presented to our center from 2000-2015. Clinical and treatment characteristics were examined, and disease was classified by single or multisystem organ LCH involvement. The pediatric group was defined by age ≤18 years at time of LCH diagnosis. Response assessment per lesion was assessed using the RECIST 1.1 and PERCIST 1.0 criteria. For patients with multiple lesions, treatment and response for each lesion was assessed individually. Adverse events were evaluated using CTCAE v4.0. Of 184 patients, 108 were adults and 76 children with median age 31 (0.1-89). The most common sites of LCH were as follows: adults: bone, lung, skin, and brain, and children: bone, brain, and skin. Physician choice of RT versus other modalities, primarily excision, was guided by proximity to critical structures, extent of disease, or patient preference. Of 42 adult bone lesions, 28 (67%) underwent excision, 10 (24%) RT, 3 (7%) observed, and 1 (2%) received systemic therapy. Of the 10 RT patients, none required additional therapy for LCH disease. Of 73 bone lesions in children, 32 (44%) underwent excision, 19 (26%) had systemic treatment, 15 (21%) received RT, and 7 (10%) were observed. No patients required additional therapy post-excision. Of the 15 RT patients, only 1 (7%) required a second-line treatment. Median age of RT patients was 35 years (1.5 – 67). The median RT dose was 11.4 Gy (7.5-50.4). There were no acute Grade 3 events and only 1 late Grade 3 event of chronic headache noted. Bone lesions are the most common site of LCH involvement in single-system disease in both pediatric and adult populations. Radiotherapy provides good local control with low likelihood of local failure and is well tolerated in the treatment of select LCH bone lesions for which resection is sub-optimal. Radiotherapy is a safe and effective modality that should be considered for local control of single-system LCH bone lesions among both adult and pediatric patients. Further characterization of the role of RT in site-specific single-system LCH may guide future management decisions." @default.
• W2896472722 created "2018-10-26" @default.
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• W2896472722 date "2018-11-01" @default.
• W2896472722 modified "2023-09-25" @default.
• W2896472722 title "Treatment Modality Choices and Outcome in Langerhans Cell Histiocytosis: Radiation Therapy Versus Other Modalities" @default.
• W2896472722 doi "https://doi.org/10.1016/j.ijrobp.2018.07.910" @default.
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