脊椎朗格汉斯组织细胞增生症13例报告

Langerhans cell histiocytosis of the spine: report of 13 cases

背景:脊椎朗格汉斯组织细胞增生症(LCH)是一种少见病,诊断困难,治疗方法仍有争议。目的:探讨脊椎LCH的临床和影像学特征及其治疗效果。方法:回顾性分析1985年10月至2003年10月收治的13例脊椎LCH患者的临床资料,男7例,女6例,年龄1.5～11岁,平均5.5岁。脊椎以外颅骨、腓骨、股骨颈、股骨大转子和坐骨等多发骨骼受累6例;勒-雪病发病1～2年内出现新的骨病变3例;患病处疼痛或放射痛、压痛或畸形;合并四肢轻瘫3例;骶神经受压1例;皮疹、间质性肺炎、肝大、尿崩症等多系统受累5例。对合并四肢轻瘫者行化疗或放疗加手术治疗2例;因骶神经受压行手术减压1例;仅行放疗1例;单用化疗3例;无全身症状,无进行性脊柱畸形者,密切观察不作特殊处理6例。X线、CT的特点有椎旁阴影扩大,椎体溶骨性改变(呈楔形变或致密薄片),病变严重者累及椎弓根或椎板6例。两相邻椎体受累者可见椎间变窄,与椎体炎症难鉴别。经活检或手术确诊7例,根据临床资料、影像学确诊6例。结果:经治疗4例脊髓及骶神经功能恢复。2例尿崩症随访11年1例无改善,另1例恶化。12例经2～12年随访(平均7.4年)椎体高度不同程度恢复,6例恢复正常,5例轻度后突畸形,1例轻度侧突畸形。失访1例。结论:根据临床及X线、CT或MRI资料,并结合活检及密切随访,有助于多系统或多发骨骼受累的脊椎LCH的诊断。化疗结合手术可控制脊椎LCH进展。全身无症状的脊椎LCH无进行性畸形者密切观察无需特殊处理。

Background： Langerhans cell histiocytosis （LCH） of the spine is a rare condition and is difficult to diagnose, and appropriate treatment is still controversial. Objective： To investigate the clinical and imaging features and therapeutic efficacy of spinal LCH Methods： Thirteen cases with spine LCH treated from October 1985 to October 2003 were reviewed retrospectively. There were 7 boys and 6 girls with an average age of 5.5 years （range, 1.5 to 11 years）. Six cases had extra-spinal skeletal lesions involved in skull, fibula, ischium, pubis, greater trochanter and neck of femur. Among those with Letterer-Siwe disease, new osseous lesions occurred in 3 cases within 1 to 2 years. The symptoms included pain, refered pain, tenderness or abnormalities in the focus. Of all cases, there were 3 cases with mild tetraplegia, 1 case with sacral nerve compression, and 5 cases with systemic lesions including rash, interstitial pneumonia, hepatomegaly and diabetes insipidus. Two cases with mild tetraplegia were treated with radiotherapy （or chemotherapy） plus surgery. One case with sacral nerve compression underwent decompression. One were treated with radiotherapy and 3 treated with chemotherapy only. For six cases without systemic symptoms or progressive spinal deformity, no special treatment was done under close observation. X-ray and CT showed wide paravertebral shadow, osteolysis of the vertebrae with wedge shape or vertebra plana. Serious lesions involved vertebral pedicles and even vertebral plates in 6 cases. When adjacent vertebral bodies were involved, the narrowed intervertebral space could be seen, which was difficult to be differentiated from vertebral inflammation. Seven cases were diagnosed by biopsy or surgery and 6 by clinical and imaging data. Results： The spinal cord and sacral nerve function recovered in 4 cases after treatment. Two cases with diabetes insipidus were followed for 11 years, in whom one was unchanged and one deteriorated. The vertebral height in 12 cases restored spontaneously to various extent over a follow-up of 2-12 years （mean,7.4 years）. Six cases restored completely, and mild kyphosis was found in 5 cases and mild scoliosis in 1. One case lost contact.Conclusions： Based on clinical findings, the characteristics of X-ray, CT and MR images combined with biopsy and close follow-up, the diagnosis of spinal LCH with multi-skeletal lesions or multi-systemic lesions can be made. Chemotherapy combined with surgery may control the progression of spinal LCH. For LCH of the spine in the absence of systemic disease, there is no need for special treatment except close follow-up, unless the progressive spinal deformity is found.