The Journal of the Korean bone and joint tumor society (대한골관절종양학회지)

The Korean Musculoskeletal Tumor Society (대한근골격종양학회)
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Treatment Outcome of Langerhans Cell Histocytosis

랑게르한스 세포 조직구증의 치료 결과

  • Chung, So Hak (Department of Orthopedic Surgery, Kosin University Gospel Hospital) ;
  • Kim, Jae Do (Department of Orthopedic Surgery, Kosin University Gospel Hospital) ;
  • Jo, Hyun Ik (Department of Orthopedic Surgery, Kosin University Gospel Hospital)
  • 정소학 (고신대학교 의과대학 정형외과학교실) ;
  • 김재도 (고신대학교 의과대학 정형외과학교실) ;
  • 조현익 (고신대학교 의과대학 정형외과학교실)
  • Received : 2014.04.01
  • Accepted : 2014.05.29
  • Published : 2014.06.30
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Abstract

Purpose: To analyze the clinical features and treatment outcome of Langerhans' cell histocytosis. Materials and Methods: From August 1996 to June 2013, 28 patients who histologically proven with LCH were analyzed of medical records, radiography, pathologic character retrospectively. Results: A total of 28 cases of LCH including 22 child has been reported. Onset age was 0.6 to 51 years old, occurred in the average age was 14.8 years. Follow-up period was 6 months to 134 months average was 44.6 months. The M:F ratio was 2.5:1. The initial symptoms was pain in 18 cases, 5 cases of pathologic fracture, 3 case of palpable mass, 1 case of discovered by accident in radiography, 1 case of torticollis. In radiological examination osteolysis was seen all cases, 7 cases showed a periosteal reaction, 1 case showed soft tissue extension. Clinical type of all cases were eosinophilic granuloma. 25 cases were classified as unifocal disease and 3 cases were multifocal single systemic diseases. In all cases, incisional biopsy was performed. After histologic confirmed, 14 cases was treated with curettage or surgical excision of the lesion and the other 14 cases were followed up without treatment. There is no death during follow up period. 11 cases has no radiological improvement after 3-6 months observation, intralesional steroid injection was performed. Conclusion: Patients with LCH who has rapid systemic onset is very rare, so if you meet the young children who suspected LCH, you shoulder avoid the examination which cause excessive radiation exposure to the young patient. In order to confirm the diagnosis of disease, biopsy is needed. Close observation after confirmed by histological method will bring the satisfactory results. But the patients who had pathologic fracture or wide bone destruction already may need curettage and bone grafting to lesion or internal fixation. The lesion which has no radiological improvement after 3-6 months observation or appear with pain interferes daily life may need local steroid injection as a good treatment.

목적: 본 교실에서 경험한 랑게르한스 세포 조직구증의 임상적 특징을 알아보고 치료방법 및 예후를 알아보고자 한다. 대상 및 방법: 1996년 8월부터 2013년 6월까지 본 교실에서 수술 후 랑게르한스 세포 조직구증으로 확진 된 총 28례를 대상으로 발병 당시부터 최근까지의 진찰소견, 의무기록, 방사선 사진, 병리소견을 토대로 임상소견과 진단 및 치료결과에 대해 후향적으로 분석하였다. 결과: 총 28례로 소아 22례 성인 6례였다. 발병나이는 0.6세에서 51세 사이에서 발생하였으며 평균연령은 14.8세였다. 추시 기간은 6개월에서 134개월까지 평균 44.6개월 이었다. 환자의 구성은 남자 20명 여자 8명으로 남녀성비는 2.5:1이었다. 초기증상은 동통 18례, 병적 골절 5례, 종괴 3례, 방사선 검사상 우연히 발견된 경우 1례, 사경 1례였다. 방사선학적 소견에서는 모든 증례에서 골 용해 소견을 보였으며 이중 13례에서는 골막 반응이 동반되었으며 1례에서는 연부조직 침범이 관찰되었다. 임상적 분류로 28례 모두 호산구성 육아종 이었으며 조직구 협회가 제시한 분류법으로는 단일계통의 다발 병소 질환 3례, 단일 병소 질환 25례였다. 전례에서 조직 생검술이 시행되었으며 6례에서는 생검술 이후 특별한 치료 없이 골 유합을 얻었으며 11례에서는 스테로이드 국소 주입술을 시행하였으며 11례는 조직생검과 동시에 소파술 및 골이식술을 시행하거나 추가로 금속 내고정술을 시행하였다. 추시기간 중 환자가 사망한 경우는 없었다. 병변이 국소 재발한 경우는 없었으나 신체 다른 부위에 골병변이 생긴 경우가 3례 있었다. 합병증은 총 2례가 있었으며 소파술 및 골 이식술을 시행한 이후 감염소견을 보여 변연절제술을 시행한 경우였다. 결론: 랑게르한스 세포 조직구증 환자에서 급격한 전신적 발병을 가져오는 경우는 매우 드물기 때문에 초기 진단 시에 어린 환아에게 과도한 방사선 노출을 가져오는 검사는 지양해야 할 것으로 생각된다. 질환의 확진을 위해서는 조직 생검이 필요하며 조직학적 확진 이후에는 경과 관찰만으로도 대부분에서 만족스러운 결과를 얻을 수 있으나 술 전 병적 골절이 있거나 골 파괴 병변의 범위가 넓은 경우 소파술 및 골 이식술과 내고정술이 필요할 수도 있다. 3-6개월간의 경과관찰에도 병변의 호전이 느리거나 일상생활에 지장을 주는 통증을 호소하는 경우 스테로이드 국소 주입법이 좋은 치료가 될 수 있다.

Keywords

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