Cancer Research and Treatment

Case Reports

Case Report of Erdheim-Chester Disease Successfully Treated with Pegylated Interferon: A Single-Center Experience: Yujin Lim, Sang Eun Yoon, Junhun Cho, Darae Kim, Chul Won Jung; Cancer Res Treat. 2023;55(3):1053-1057. Published online January 19, 2023; DOI: https://doi.org/10.4143/crt.2022.1535

Erdheim-Chester disease (ECD), also known as non-Langerhans cell histiocytosis, is a multi-systemic disease with unclear pathogenesis. Based on a small number of case studies, pegylated interferon-α (PEG-IFN-α) has been used as the front-line treatment option. However, there are limited data regarding administration of ropegylated-interferon α-2b (ROPEG-IFN-α 2b) for ECD patients. Herein, we report two cases of severe ECD treated with two types of PEG-IFN-α. One patient with heart and skeleton involvement and BRAF V600E mutation was treated with weekly PEG-IFN-α 2a. Another patient with bone involvement and no BRAF V600E mutation was administered monthly ROPEG-IFN-α 2b. The two types of PEG-IFN-α showed excellent disease control, excellent survival outcomes, and manageable toxicities in ECD patients. These results suggest that ROPEG-IFN-α 2b could be used equivalently to PEG-IFN-α 2a for management of advanced ECD.

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An Autopsied Case of Erdheim-Chester Disease with Severe Cardiovascular Involvement
Atsushi Matsunashi, Wang Zhipeng, Akihiko Sugimoto, Masakazu Fujimoto, Akihiko Yoshizawa, Ryo Sakamoto, Michihiro Uyama, Kohei Ikezoe, Kiminobu Tanizawa, Tomohiro Handa, Toyohiro Hirai
Internal Medicine.2025;[Epub] CrossRef
Recent advances in therapeutic strategies of Erdheim-Chester disease
Rohit Doke, Rahul Lokhande, Kalyani Chande, Kuldeep Vinchurkar, Bhupendra G. Prajapati
Naunyn-Schmiedeberg's Archives of Pharmacology.2025;[Epub] CrossRef
Erdheim–Chester disease: Comprehensive insights from genetic mutations to clinical manifestations and therapeutic advances
Rishabh Chaudhary, Anand Kumar, Alpana Singh, Vipul Agarwal, Mujeeba Rehman, Arjun Singh Kaushik, Siddhi Srivastava, Sukriti Srivastava, Vikas Mishra
Disease-a-Month.2025; 71(2): 101845. CrossRef
Advances in Understanding and Management of Erdheim-Chester Disease
Aniruddha Murahar Kulkarni, Prasanna Kumar Reddy Gayam, Jesil Mathew Aranjani
Life Sciences.2024; 348: 122692. CrossRef

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Erdheim-Chester Disease with Emperipolesis: A Unique Case Involving the Heart: Pengcheng Zhu, Naping Li, Lu Yu, Mariajose Navia Miranda, Guoping Wang, Yaqi Duan; Cancer Res Treat. 2017;49(2):553-558. Published online July 28, 2016; DOI: https://doi.org/10.4143/crt.2016.078

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Histiocytosis is an uncommon disease characterized by excessive accumulation of histiocytes. Here, we report a rare case of non-Langerhans-cell histiocytosis in a 51- year-old woman who presented with severe symptoms of pericardial effusion. Radiologic investigation also detected multiple bone (lower limbs, vertebrae, ribs, and ilium) lesions. Resected pericardium showed abundant mono- or multi-nucleated non-foamy histiocytes (CD68⁺/CD163⁺/S-100⁺/CD1α⁻/langerin⁻) in a fibroinflammatory background. The histiocytes demonstrated emperipolesis of lymphocytes, a hallmark feature of Rosai-Dorfman disease (RDD). However, molecular analysis revealed a BRAF V600E mutation of the proliferating histiocytes, highlighting the neoplastic features frequently observed in another non-Langerhans-cell histiocytosis known as Erdheim-Chester Disease (ECD). We consider this case to be a unique presentation of ECD harboring some RDD-like cells with emperipolesis, but not a case of RDD with a BRAF mutation concerning its clinical manifestation (involvement of the heart and bones) and neoplastic features.

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An Autopsied Case of Erdheim-Chester Disease with Severe Cardiovascular Involvement
Atsushi Matsunashi, Wang Zhipeng, Akihiko Sugimoto, Masakazu Fujimoto, Akihiko Yoshizawa, Ryo Sakamoto, Michihiro Uyama, Kohei Ikezoe, Kiminobu Tanizawa, Tomohiro Handa, Toyohiro Hirai
Internal Medicine.2025;[Epub] CrossRef
Infantile Rosai-Dorfman Disease With Isolated Brain Lesions Disseminated to the Parenchyma and Intraventricular Ependyma, Alteration of Leukocytes as a Promotion Factor in Immune Defense, and New Proposals: A Case Report and Literature Review
Nasser Kamalian, Shahmir Kamalian, Mohammad Vasei
Cureus.2024;[Epub] CrossRef
Emperipolesis in pleural fluid mesothelial cells . A phenomenon not associated with Rosai-Dorfman disease, report of a case
Diana Sofía Vargas-Lías, Mónica Lizette Serrano-Arévalo, Lara-Torres César, Corredor-Alonso Guillermo Ernesto, Hugo R. Dominguez-malagon
Ultrastructural Pathology.2024; 48(5): 438. CrossRef
BRAF V600E mutation and high expression of PD-L1 in Rosai-Dorfman disease: case report and review of the literature
Farnoush M. Moen, Mariam M. Youssef, Mihir Shukla, Mary Lynn Nierodzik, Marius E. Mayerhoefer, Christopher Park
Journal of Hematopathology.2024; 17(4): 183. CrossRef
Characterizing Rosai-Dorfman disease with [18F]FDG PET/CT: a retrospective analysis of a single-center study
Jianan Zhang, Ruixue Cui, Jian Li, Xinxin Cao, Yaping Luo
European Radiology.2023; 33(9): 6492. CrossRef
Atypical skeletal involvement in patients with Erdheim–Chester disease: CT imaging findings
Zaizhu Zhang, Wei Yu, Wenmin Guan, Qiang Lin, Ali Guermazi
Orphanet Journal of Rare Diseases.2022;[Epub] CrossRef
BRAF mutation in overlapping form of Erdheim-Chester and Rosai Dorfman diseases: a unique case restricted to the central nervous system
J. Dufour, B. Mathon, M. Touat, K. Hoang-Xuan, K. Mokhtari, A. Idbaih
Revue Neurologique.2021; 177(6): 708. CrossRef
Reticulohistiocytoses: a revision of the full spectrum
A. Bonometti, E. Berti
Journal of the European Academy of Dermatology and Venereology.2020; 34(8): 1684. CrossRef
Orbital histiocytosis with systemic involvement
Joaquín J.R. Civit, Davinia Godoy, Alicia Conde, Jorge Arencibia, Ramón Medel, Miguel A. Limeres, Iñigo S. Miguel, José D. Marín, Y. Aguilar, Francesc Tresserra, Francisco Medina
Saudi Journal of Ophthalmology.2020; 34(4): 319. CrossRef
Coccidioidomycosis with emperipolesis in fine needle aspiration
Alexandra Grosse, Claudia Grosse
Cytopathology.2019; 30(4): 451. CrossRef
Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease
Julien Sarkis, Fady Haddad, Sarah Nasr, Elie Hanna, Ahmad Mroueh, Elie Nemr
Case Reports in Urology.2019; 2019: 1. CrossRef
Erdheim–Chester disease: description of two illustrative cases involving the lung
Magda Zanelli, Maxwell Smith, Maria C Mengoli, Lucia Spaggiari, Loredana De Marco, Filippo Lococo, Francesco Puma, Stefano Ascani
Histopathology.2018; 73(1): 167. CrossRef

11,957 View
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