The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Single-Center Real-World Experience with Primary Central Nervous System Lymphoma in the 21st Century

원발 중추신경계림프종의 단일 기관 현실 세계 21세기 경험

  • Hyungwoo Cho (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jung Yong Hong (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Dae Ho Lee (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Shin Kim (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kyoungmin Lee (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Eun Hee Kang (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Sunjong Lee (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jung Sun Park (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jeong Hoon Kim (Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jin Sook Ryu (Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jooryung Huh (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Cheolwon Suh (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
  • 조형우 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 홍정용 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 이대호 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 김신 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 이경민 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 강은희 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 이순종 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 박정선 (울산대학교 의과대학 서울아산병원 종양내과) ;
  • 김정훈 (울산대학교 의과대학 서울아산병원 신경외과) ;
  • 류진숙 (울산대학교 의과대학 서울아산병원 핵의학과) ;
  • 허주령 (울산대학교 의과대학 서울아산병원 병리과) ;
  • 서철원 (울산대학교 의과대학 서울아산병원 종양내과)
  • Received : 2022.01.07
  • Accepted : 2023.09.07
  • Published : 2024.02.01
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Abstract

Background/Aims: In Korea, the incidence of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is increasing and autologous stem cell transplantation (ASCT) has improved the survival of younger patients. We explored our real-world experience with PCNSL at Asan Medical Center (AMC). Methods: We used the AMC lymphoma registry to collect patient data prospectively. We analyzed 279 patients diagnosed from 2002 until August 2019. Results: The PCNSL incidence at AMC increased progressively and comprised 7.4-8.9% of new non-Hodgkin lymphoma patients annually during the most recent 4 years. The median age was 60 years (range, 17-85) and males comprised 55%. Patients under 65 years of age (n = 183) had no significant differences in characteristics compared to those aged 65 years or over, with the exception of less occipital lobe involvement and lower beta-2 microglobulin levels. Rituximab, methotrexate, procarbazine, and vincristine (R-MPV) combination induction had the best overall response, of 95%. The median overall survival was 3.8 years with 5- and 10-year survival rates of 41.5% and 30.2%, respectively. Survival was better in younger patients and those treated with ASCT. Thiotepa, busulfan, and cytoxan (TBC) conditioning chemotherapy had better survival than other combinations. The International Extranodal Lymphoma Study Group and Memorial Sloan Kettering Cancer Center prognostic score systems were valid in this cohort. Age and performance status were independent prognostic factors. Exclusive extra-central nervous system failure occurred in six patients (5.6%) among 107 failures. Conclusions: The incidence of PCNSL is rising. R-MPV induction therapy followed by ASCT with TBC has improved the survival of young, fit PCNSL patients.

목적: 대한민국에서 원발 중추신경계림프종(primary central nervous system lymphoma, PCNSL)의 발생이 증가하고 있고, 자가 조혈모세포 이식술(autologous stem cell transplantation, ASCT)이 젊은 환자들의 생존 성적을 호전시켜 왔다. 이에 서울아산병원(Asan Medical Center, AMC)의 PCNSL 환자 현실 세계 경험을 분석하고자 하였다. 방법: 환자 자료를 전향적으로 수집, 기록 중인 AMC 림프종 등록을 이용하였다. 2002년부터 2019년 8월까지 279명의 환자를 분석하였다. 결과: AMC의 PCNSL 발생은 점진적으로 증가하여 최근 4년 동안은 매해 전체 비호지킨림프종 신규 환자의 7.4-8.9%를 차지하였다. 중앙 연령은 60세(범위, 17-85)였고, 남자가 55%였다. 65세 미만(n = 183)은 후두엽 침범이 보다 적고, 베타-2 저분자글로불린 수치가 낮다는 것 외에는 65세 이상과 비교해 의미 있는 차이를 보이지 않았다. 리툭시맵, 메토트렉세이트, 프로카르바진, 빈크리스틴(rituximab, methotrexate, procarbazine, and vincristine, R-MPV) 복합 유도 요법의 전체 반응률이 95%로 가장 우수하였다. 전체 환자의 중앙 생존 기간은 3.8년, 5년 생존율은 41.5%, 10년 생존율은 30.2%였다. 비교 시 젊은 연령군과 ASCT 시행군의 생존이 더 우수하였다. 싸이오테파, 부설판, 사이클로포스퍼마이드(thiotepa + busulfan + cyclophosphamide, TBC) 고용량 요법이 여타 조합보다 성적이 우수하였다. 기존의 PCNSL 예후 점수 체계가 본 연구에서도 유효하였다. 연령과 수행도가 독립 예후 인자였다. 중추신경계 외에서만 실패하는 경우는 전체 107명의 실패 중 6명(5.6%)이었다. 결론: PCNSL의 발생은 증가 추세를 보이고 있다. R-MPV 유도 요법 후 TBC 고용량 요법을 동반한 ASCT 시행이 젊고 적응증에 해당하는 PCNSL 환자의 생존 성적을 향상시켜 왔다.

Keywords

Acknowledgement

이러한 연구가 가능하도록 원발 중추신경계림프종 진단과 치료의 현실 세계 경험을 함께 해 주신 환자분들과 보호자분들께 고맙습니다. 환자분들의 안녕을 빕니다. 환자 진료에 함께 애써 주신 간호사 분들, 전공의, 임상 강사, 동료 교수진들이 계셔서 이 일이 가능했습니다. 특히, 제가 이 일을 하도록 이끌어 주신 김상희, 홍창기 두 분 교수님께 이 논문을 바칩니다.

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