Clinical analysis of 29 children with skull cranial langerhans cell histiocytosis / 中国医师进修杂志

ABSTRACT

Objective To investigate the clinical characteristics of skull cranial langerhans cell histiocytosis in children and improve clinical understanding and therapeutic effect Methods A retrospective clinical study was conducted in 29 cases of skull cranial langerhans cell histiocytosis treated in Shengjing Hospital Affiliated to China Medical University from January 2012 to January 2018. Results The main lesion of skull bone was dissolved bone. Among them, there were 5 cases of frontal bone lesions, 10 cases of temporal bone lesions, 2 cases of occipital lesions, and 12 cases of multiple lesions in the skull;There were 10 cases of vertebral lesions and 5 cases of multiple bone lesions involved around the body. Other clinical manifestations included insipidus in 4 cases, erythra in 3 cases, hepatosplenomegaly in 2 cases and lymphadenectasis in 1 case. All cases were followed up for 0.5 to 5.0 years. In the group of combined surgery and chemotherapy treatment, the prognosis of single system involvement cases was that 9 patients were cured, 2 patients were stable and 1 patients were progressing, while the prognosis of multiple systems involvement cases was that 1 patient was cured, 2 patients were stable and 3 patients were progressing. The prognosis of patient with multiple systems lesions was relatively poor compared to that of the cases of single system lesion and there was statistical difference. In the pure operation group , the prognosis of single system involvement cases was that 5 patients were cured, 1 patient was stable and 1 patient was progressing, while the prognosis of multiple systems involvement cases was that 1 patient was stable and 3 patients were progressing. Conclusions Children with skull cranial langerhans cell histiocytosis present single or multiple cranial osteolytic lesions. It is often accompanied by multiple systems lesions around the body, and prognosis of children with multiple systems lesions was is relatively poor.