颅骨和脊椎朗格汉斯组织细胞增生症

Langerhans cell histocytosis (LCH) in skull and spine

目的 探讨颅骨和脊椎朗格汉斯组织细胞增生症 (LCH)的诊断和治疗。方法 总结 5年中经手术后病理证实的颅骨和脊椎LCH15例 ,男 10例 ,女 5例 ,年龄 1.5岁～ 10岁。颅骨损害 11例 (单发 9例 ,多发 2例 ) ,颅骨合并脊椎损害 2例 ,脊椎损害伴椎旁软组织肿块 2例。影像学上均表现为骨质破坏。颅骨单发和多发损害 ,术后化疗 ,不作放疗。颅骨伴脊椎损害和脊椎损害伴椎管内外软组织肿块 ,脊椎放疗后化疗。结果 全部病例随访 0 .5～ 4 .5年 ,无一例死亡。 2例颅骨多发损害 ,术后化疗期间 ,颅骨有新病灶出现 ,改用环磷酰胺 ,病灶消失 ,无复发。结论 病变组织活检是明确诊断的依据。术后化疗、放疗应视疾病程度而定。

Objective To explore the diagnosis and treatment of Langerhans cell histocytosis (LCH) in skull and spine.Methods Fifteen cases ( male 10,female 5) between 1.5 and 12 years of age with LCH of skull and spine,were diagnosed on the basis of surgery and pathology within last 5 years. Eleven patients had skull involvement (9 with isolated and 2 multifocal lesions).They were treated by postoperative chemotherapy.Two children with both skull and spine LCH and 2 cases of spinal LCH associated with paraspinal soft tissue mass underwent spinal radiotherapy followed by chemotherapy.Results The patients were followed up for 1.5 ～ 4.5 years.All were alive and well.New LCHs appeared in 2 cases of multifocal skull lesions during chemotherapy and disappeared after changing to cyclophosphamide.Conclusions For LCH,only histopathologic diagnosis is definitive.Postoperative chemotherapy and radiotherapy should be administrated according to the extent of involvement. The prognosis of skull and spine LCH is good.