Gaundong Mbéthé G-L, Dejean C, Henriques de Figueiredo B, Sargos P, Italiano A, Kantor G
Service de radiothérapie, centre régional de lutte contre le cancer, institut Bergonié, 229, cours de l'Argonne, 33076 Bordeaux cedex, France.
Cancer Radiother. 2010 Dec;14(8):759-62. doi: 10.1016/j.canrad.2010.03.018. Epub 2010 Jul 31.
Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100,000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology.
朗格汉斯细胞组织细胞增多症是一种病因不明的非恶性增殖性疾病，可累及一个或多个器官。这是一种罕见疾病，发病率为1至2/10万，主要影响儿童，男性居多。骨病变最为常见（60%至90%）。然而，对于这些部位的治疗尚无共识。我们报告了两名患者的病例，这两名患者在接受初始化疗后成功接受了放射治疗，一名患者分十次给予15 Gy，每次1.5 Gy，另一名患者分十次给予18 Gy，每次1.8 Gy。大多数情况下，无内脏受累的单灶或多灶性骨朗格汉斯细胞组织细胞增多症是一种良性的自限性疾病。一些骨病变可单独通过放射治疗。但目前文献中给出的剂量差异很大，无法确定限制儿童继发肿瘤风险或生长受损风险的最低有效剂量，对于儿童推荐的较低剂量为6至8 Gy。放射治疗的决定必须权衡疾病风险。在这种非恶性肿瘤病理学中应谨慎行事。
