Investigation On Natural History And Clinical, Imaging And Pathological Correlation Study Of Spinal Langerhans Cell Histiocytosis

Objectives:To investigate the epidemiological features of spinal Langerhans cell histiocytosis(LCH) in order to analyze its natural history and to provide clinical evidences of its diagnosis,treatment and prognosis.Methods:From December 1985 to January 2008,fifty-four patients diagnosed of spinal LCH in our department were investigated retrospectively.The differences of case history, clinical manifestation,imageological finding and follow-up result were studied between affected ages and locations using software of SPSS 13.In order to compare between affected ages conveniently,the patients were divided into teenagers group(age from 5 yrs to 17 yrs,28 patients) and adult group(age older than 18 yrs,26 patients) according to the international conventions.Results:Fifty-four patients with spinal LCH were followed-up successfully.Forty-three male patients and eleven female patients were involved,whose mean age was 22 yrs(5 yrs to 71 yrs).Of the 54 patients,the mono-segmental invasion was found in 45 patients,and the poly-segmental invasion was found in 9 patients.Of the total of 59 affected vertebrae, cervical vertebrae were found in 32 cases(54%),thoracic vertebrae in 17 cases(29%), lumbar vertebrae in 8 cases and sacral vertebrae in 2 cases(17%).Furthermore,the incidence rate in the cervical vertebrae was significantly higher than that in other vertebrae(p<0.01).The frequently-occurring of mono-segmental invasion was C2(10 cases).And no viscera were affected in all cases.The interval between occurring of symptom and visiting in clinic was 2.7 months(0.5 months to 12 months).There was no significant difference between ages,genders and locations.Pain was the most frequently occurring symptom,of which 47 cases(87%) was affected. Other symptom such as limitation of activity was found in 26 cases(44%),spinal deformity in 7 cases(13%) and nervous lesion in 29 cases(54%).According to Frankel grading system,grade A was found in 1 cases,grade B in 1 case,grade C in 2 cases and grade D in 25 cases.The nervous lesion rate of adult group(17 cases,65%) was significantly high compared with that of teenagers group(12 cases,36%)(0.010.05 ). The destruction of bone was found in teenagers group entirely according to imageological findings.Whereas only 20 cases(77%) of destruction in adult group was detected with X-ray examination.Among 6 cases(23%) with the negative X-ray findings,5 cases were found destruction with CT or MRI,and 1 case without positive findings.The incidence rate of platyspondyl in teenagers group(13 cases) was significantly higher than that of in adult group(1 case)(P<0.01).Furthermore,the affected segments of platyspondyl was no great significant difference(P>0.05 ),with 8 case in cervical,1 case in lumbar 5 and 1 case in sacral 1.Lumps surrounding of the soft tissue was found in 28 cases(47%),which consisted of cervical vertebrae in 16 cases(50%),thoracic in 8 cases(47%),and lumbar in 4 cases (40%).There was no great significant difference between teenagers group(16 cases,48 %) and adult group(12 cases,46%)(P>0.05).And there was also no great significant difference between affected segments(P>0.05 ).After follow-up,47 patients was good,7 patients poor.Among 5 recurrent patients,1 case and 4 cases were in teenagers group and adult group respectively.And statistic study indicated there was no great significant difference between teenagers group and adult group(P>0.05 ).The recurrent case in teenagers group was carried out long-term hormone replacement therapy due to the pituitary disorder with polyuria.And one case recovered from grade A to E according to Frankel grading system.The other patients recovered well.Conclusions:1 Spinal LCH might occur in male or female in any ages,which was a benign disease with a generally good prognosis.2 The incidence rate of spinal LCH in the cervical vertebrae was higher than that in other vertebrae,mostly in C2 singly.3 The incidence rate of nerve lesion in spinal LCH related to age of the patients,and not to location of vertebra.And the incidence rate of nerve lesion in adult was higher than that in teenagers.The causes induced nerve lesion included unstable spine due to destruction of tumor and compression to spinal cord due to intraspinal invasion of tumor4 Follow-up was essential to all patients with spinal LCH.And the time of follow-up determined with individual conditions,at least 3-4 years away from the last onste. Objectives:To find the natural history,imaging and pathological correlation of spinal Langerhans cell histiocytosis(LCH) in order to investigate the feasibility of assessment the stage and the degree of LCH using imaging findings.Methods:From December 1985 to January 2008,forty-five patients with intact case history record,imaging and pathological findings of sixty-three patients diagnosed of spinal LCH in our department were investigated retrospectively.Thirty-eight male patients and seven female patients were involved,whose mean age was 24 yrs.Cervical vertebrae were found in 27 patients,thoracic vertebrae in 13 patients and lumbar vertebrae in 5 patients.The imaging staging of early stage,intermediate stage and advanced stage were performed according to the criteria established by Mirra and Gold.And the pathological staging of early stage,intermediate stage and advanced stage were performed according to the criteria established by pathology of bone and joint.The double-blind evaluations of the imaging and pathological findings were performed by the doctors from imaging and pathological department respectively.Then the statistic analysis was carried out to determine the correlation between them.After the calculations of the emergence time of the symptoms with the two staging,combined with the clinical manifestations,the R×C chi square test and Kappa test were performed to determine the correlation between the imaging findings and the pathological findings using software of SPSS 13.P<0.05 was considered significantly different.Results:Using imaging staging,23 patients was in the early stage,which consisted of 15 patients in cervical vertebrae,5 patients in thoracic and 3 patients in lumbar.Using imaging staging,23 patients were in the early stage,which consisted of 15 patients in cervical vertebrae,5 patients in thoracic and 3 patients in lumbar.Using pathological staging,19 patients were in the early stage,which consisted of 12 patients in cervical vertebrae,5 patients in thoracic and 3 patients in lumbar.The statistic analysis indicated the obvious correlation between the imaging staging and the pathological staging using the RxC chi square test and Kappa test(P<0.001 ).Furthermore,the imaging staging was delayed slightly compared with that of the pathological staging.The mean courses of early stage,intermediate stage and advanced stage were 1.2 months,3.8 months and 8 months respectively according to the imaging staging.And the mean courses of early stage, intermediate stage and advanced stage were 1 month,2.4 months and 7.5 months respectively according to the pathological staging.Conclusions:The obvious correlation between the imaging staging and the pathological staging was found in the spinal LCH.And the imaging findings might represent the changes of the disease.According to the imaging findings,it might be practicable to determine the stage of the disease.In clinic,the therapeutic regimen might be made according to the imaging staging to determine the course of disease.