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Inflammatory Myofibroblastic Tumor of Extremities

사지에 발생한 염증성 근섬유모세포성 종양

  • Kong, Chang-Bae (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Lee, Jeong-Dong (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Lee, Jung Uk (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Song, Won-Seok (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Cho, Wan-Hyeong (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Koh, Jae-Soo (Department of Pathology, Korea Cancer Center Hospital) ;
  • Jeon, Dae-Geun (Department of Orthopedic Surgery, Korea Cancer Center Hospital)
  • Received : 2013.03.22
  • Accepted : 2013.06.03
  • Published : 2013.06.30

Abstract

Purpose: We analyzed the oncologic characteristics and outcome of patients with inflammatory myofibroblastic tumor of extremities. Materials and Methods: Among the soft tissue tumor patients who were treated between 1999 and 2012, 5 patients who were pathologically confirmed as the inflammatory myofibroblastic tumor of extremities were analyzed retrospectively. Results: There were 1 man and 4 women with mean age of 44 years (37-55 years). The average follow up was 34.6 months (8-87 months). All patients underwent surgical treatment. Only 1 patient had wide resection margin and remaining 4 had marginal (3) or intralesional (1) resection margin. All of 4 patients without wide resection margin developed local recurrence at 10.3 months (8-19 months). Malignant transformation to fibrosarcoma was occurred in 2 patients who developed local recurrence, and 1 patient developed multiple metastases to lung, liver and lymph nodes and expired at 37 months. Three of 5 patients had tumor location abutted to or invasion to major arteries and 1 patient had tumor invading sciatic nerve. Conclusion: It is observed that inflammatory myofibroblastic tumor of extremities is usually located near the major neurovascular structure. Wide resection should be considered as the initial surgical treatment because this tumor showed a high local recurrence rate and possibility of malignant transformation.

목적: 사지에 발생한 염증성 근섬유모세포성 종양의 특성과 치료 결과에 대하여 알아보고자 하였다. 대상 및 방법: 본 연구는 1999년부터 2012년까지 본원 정형외과에서 치료받은 연부조직 종양 환자 중에서 사지에 발생한 염증성 근섬유모세포성 종양으로 병리학적으로 확진된 5명의 환자를 대상으로 하였다. 결과: 남자가 1예, 여자가 4예였으며, 평균 연령은 44세(37-55세)이고, 평균 추시 기간은 34.6개월(8-87개월)이었다. 5명의 환자 모두에서 외과적 절제술을 시행하였으며 이 중 1명의 환자에서만 광범위 절제연을 얻었고, 3명에서는 변연부 절제연을, 다른 1명에서는 병소내 절제연을 얻었다. 광범위 절제연을 얻지 못한 4명의 환자 모두에서 술 후 평균 10.3개월(8-19개월)에 재발이 발생하였으며, 재발한 4명의 환자 중 2명에서 섬유육종으로의 악성 변화가 관찰되었다. 악성 변화를 보인 2명의 환자 중 1명은 폐, 간 및 림프절로의 다발성 원격 전이가 발생하여 추시관찰 37개월에 사망하였다. 5예의 병변 중 3예에서 종양의 경계가 사지의 주요 동맥에 인접한 소견이 관찰되었으며 1예에서는 종양이 좌골 신경을 침범한 소견이 관찰되었다. 결론: 사지에 발생한 염증성 근섬유모세포성 종양은 주요 신경혈관계와 인접하여 발생하는 경향을 나타내었다. 이 종양은 재발을 잘하며 악성 변화의 가능성이 있으므로 광범위 절제술이 적절한 수술적 치료법이 될 것으로 생각된다.

Keywords

References

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