引言朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的,以骨髓树突状细胞增殖和组织浸润为特征的克隆性疾病,最常见于儿科人群。通常表现为骨骼病变,可能累及盆腔。很少有研究对孤立的骨盆LCH病变治疗后的结果进行表征和回顾。方法对18岁以下诊断为累及骨盆的单灶性或多灶性骨骼LCH病变的患者进行回顾性单机构审查。临床表现,病变部位,病灶分类,射线照相结果,治疗,并发症,并对复发率进行了回顾。结果20例单灶性或多灶性LCH盆腔病变(男性11例,九位女性)。诊断时的中位年龄为3.5岁(0.8-21.6)。8例(40%)累及单灶性病变,和12(60%)涉及多灶性病变,最常见的相关骨骼疾病发生在髂骨。100%的病例有溶解性骨病变,无病理性骨折。所有病例非手术治疗化疗药物,皮质类固醇,或单独观察。75%的病例以100%的决议率化疗医治。中位随访时间为4.5年(0.4-16.7)。结论我们的研究发现,单独化疗或糖皮质激素补充化疗是治疗单部位盆腔LCH病变的合适选择。相比之下,作为多灶性表现的一部分的盆腔病变可以通过多种化疗方案得到充分治疗.皮质类固醇治疗和单独观察对于单个器官系统也可能是合理的,多焦点,在解剖学上可以进行活检并且数量或大小较小的骨骼病变。
Introduction Langerhans cell histiocytosis (
LCH) is a rare, clonal disorder characterized by proliferation and tissue infiltration by myeloid dendritic cells, most commonly occurring in pediatric populations. It often manifests as skeletal lesions with possible pelvic involvement. Few studies have characterized and reviewed outcomes after treatment of isolated pelvic
LCH lesions. Methods A retrospective single-institution review was conducted on diagnoses of patients younger than 18 with a diagnosis of unifocal or multifocal skeletal
LCH lesions involving the pelvis. Clinical presentations, lesion sites, focal classification, radiographic findings, treatments, complications, and recurrence rates were reviewed. Results Twenty patients had unifocal or multifocal
LCH pelvic lesions (11 males, nine females). The median age at diagnosis was 3.5 years (0.8-21.6). Eight cases (40%) involved unifocal lesions, and twelve (60%) involved multifocal lesions, with the most common associated skeletal disease occurring at the ilium. 100% of cases had a lytic bone lesion with no pathologic fractures. All cases were treated nonoperatively with chemotherapy medications, corticosteroids, or observation alone. 75% of cases were treated with chemotherapy with a 100% resolution rate. The median length of follow-up was 4.5 years (0.4-16.7). Conclusion Our study found that chemotherapy alone or chemotherapy with corticosteroid supplementation are appropriate options for unifocal pelvic
LCH lesions. In contrast, pelvic lesions that are part of a multifocal presentation may be managed adequately with varied chemotherapy regimens. Corticosteroid therapy and observation alone may also be reasonable for a single organ system, multifocal, skeletal lesions that are anatomically accessible for biopsy and small in number or size.