The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Desmoplastic Small Round Cell Tumor Achieved Complete Remission after High-Dose Chemotherapy with Aggressive Local Treatment Modalities

완전 관해를 얻은 결합조직형성 소원형세포종양 1예

  • Kim, Sun-Mok (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park, Ji-Hyun (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Jung-Seok (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Min-Soo (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jang, Jung-Eun (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Hwang, Hee-Sang (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Suh, Cheol-Won (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
  • 김선목 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 박지현 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 김정석 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 김민수 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 장정은 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 황희상 (울산대학교 의과대학 서울아산병원 병리과) ;
  • 서철원 (울산대학교 의과대학 서울아산병원 종양내과)
  • Published : 2012.09.01
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Abstract

A desmoplastic small-round-cell tumor (DSRCT) is a rare, aggressive neoplasm that develops mostly in the abdominal cavity in children and young adults. We present a case of a 19-year-old male with right upper quadrant discomfort for 3 months. On abdominal computerized tomography, multiple huge and demarcated masses were found in the liver, retroperitoneal lymph nodes, and peritoneal and retroperitoneal cavities. Fine needle aspiration biopsy of the hepatic mass was performed and DSRCT was diagnosed by hematoxylin and eosin staining and immunohistochemical analysis. He was treated initially with high-dose systemic chemotherapy (alternating schedules of cyclophosphamide, vincristine, doxorubicin, ifosfamide, and etoposide), underwent two debulking surgeries and pelvic irradiation between systemic chemotherapy schedules, and achieved complete remission after the 15 months of treatment duration. We report this case to emphasize the importance of aggressive local treatment modalities as well as high-dose systemic chemotherapy for treatment of DSRCT even with initially unresectable or extensively metastatic presentation.

결합조직형성 소원형세포종양은 주로 소아나 젊은 연령의 남성에서 발생하는 드문 종양으로 비교적 최근에 독립된 질환으로 분류되었으며, 아직 표준화된 치료법이 없는 예후가 나쁜 암이다. 저자들은 고용량의 복합항암화학 요법 외에도 방사선치료 그리고 두 차례의 수술의 적극적인 국소 요법을 병행하여 만 23개월째 무질병 상태로 생존한 환자 1예를 경험하였으며, 처음부터 완전한 근치적 절제가 불가능한 경우라도 고용량의 항암화학 요법에 반응이 있는 경우 적극적인 국소 치료의 병행이 환자의 생존을 연장시키는 중요한 치료 전략임을 강조하고자 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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