KCA
Articles
- Page Path
- HOME > J Korean Cancer Assoc > Accepted articles > Article
-
Original Article
- Clinico-Pathologic Features of Adult Erdheim–Chester Disease in South Korea: A Multicenter Retrospective Study (KCSG LY22-15)
-
Ji Yun Lee1
, Heounjeong Go2, Myung-won Lee3, Ji Hyun Lee4, Sang Eun Yoon5, Jee Hyun Kong6, Sojung Lim7, Yoon Kyung Jeon8
, Tae Min Kim9
-
DOI: https://doi.org/10.4143/crt.2026.0404 [Accepted]
Published online: June 29, 2026
This article has been accepted for publication following full peer review and is provided as an unedited Accepted Article to allow early access to its findings. It has not yet undergone copyediting, typesetting, pagination, or proofreading, and the final Version of Record may differ from this version.
1Division of Hematology-Oncology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
3Division of Hematology-Oncology, Department of Internal Medicine, Chungnam National University Hospital, Daejeon, Korea
4Division of Hematology-Oncology, Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea
5Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
6Department of Hematology-Oncology, Division of Internal Medicine, Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine, Wonju, Korea
7Department of Forensic Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
8Department of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
9Division of Hematology-Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
3Division of Hematology-Oncology, Department of Internal Medicine, Chungnam National University Hospital, Daejeon, Korea
4Division of Hematology-Oncology, Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea
5Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
6Department of Hematology-Oncology, Division of Internal Medicine, Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine, Wonju, Korea
7Department of Forensic Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
8Department of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
9Division of Hematology-Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
-
Corresponding author:
Yoon Kyung Jeon
Tel: 82-2-2072-1347
Email: ykjeon@snu.ac.kr
Tae Min Kim
Tel: 82-2-2072-3559
Email: gabriel9@snu.ac.kr
- Ji Yun Lee and Heounjeong Go contributed equally to this work.
Received: 14 April 2026 • Accepted: 22 June 2026
- 535 Views
- 32 Download
- 0 Crossref
- 0 Scopus
Abstract
Purpose
Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by heterogeneous multisystem manifestations and frequent MAPK–ERK pathway alterations. Data on the clinico-pathologic features and real-world outcomes of adult ECD in South Korea are limited.
Materials and Methods
We conducted a retrospective multicenter cohort study of adult patients (≥18 years) with ECD at seven Korean Cancer Study Group (KCSG)-affiliated institutions between October 2008 and June 2024. Clinical and pathologic features, BRAF V600E mutation status, treatment patterns, and overall survival (OS) were analyzed using the Kaplan–Meier method.
Results
Twenty-two patients were included (median age 57 years [IQR 45–64]; 50% male). Organ involvement was heterogeneous: bone (54.5%), retroperitoneal/renal (31.8%), central nervous system (27.3%), cardiovascular (27.3%), hypothalamic-pituitary axis (27.3%), pulmonary (18.2%), and skin (13.6%); multisystem disease (≥2 organs) was present in 40.9%. BRAF V600E was positive in 11 of 19 tested patients (57.9%). Systemic treatment was initiated in 18 patients (81.8%); interferon-α-based regimens were the most common first-line approach (50.0%). Among 16 evaluable patients, the disease control rate (CR/PR/SD) was 87.5%. With a median follow-up of 4.6 years (IQR 1.3–6.5), 2- and 5-year OS rates were 90.0% and 74.3%, respectively.
Conclusion
Korean adult patients with ECD demonstrate heterogeneous organ involvement and frequent BRAF V600E positivity. Real-world treatment remains dominated by interferon-α-based approaches, underscoring the need for standardized molecular testing and improved access to targeted therapy.
Cite
