Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era
Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK (RAS-RAF-MEK-ERK) pathway mutations. Typical findings of ECD include central diabetes insipidus, restrictive pericarditis, perinephric fibro...
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| Veröffentlicht in: | Blood Jg. 135; H. 22; S. 1929 |
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| Hauptverfasser: | , , , , , , , , , , , , , , , , , , , , , , , , |
| Format: | Journal Article |
| Sprache: | Englisch |
| Veröffentlicht: |
United States
28.05.2020
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| ISSN: | 1528-0020, 1528-0020 |
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| Abstract | Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK (RAS-RAF-MEK-ERK) pathway mutations. Typical findings of ECD include central diabetes insipidus, restrictive pericarditis, perinephric fibrosis, and sclerotic bone lesions. The histopathologic diagnosis of ECD is often challenging due to nonspecific inflammatory and fibrotic findings on histopathologic review of tissue specimens. Additionally, the association of ECD with unusual tissue tropism and an insidious onset often results in diagnostic errors and delays. Most patients with ECD require treatment, except for a minority of patients with minimally symptomatic single-organ disease. The first ECD consensus guidelines were published in 2014 on behalf of the physicians and researchers within the Erdheim-Chester Disease Global Alliance. With the recent molecular discoveries and the approval of the first targeted therapy (vemurafenib) for BRAF-V600-mutant ECD, there is a need for updated clinical practice guidelines to optimize the diagnosis and treatment of this disease. This document presents consensus recommendations that resulted from the International Medical Symposia on ECD in 2017 and 2019. Herein, we include the guidelines for the clinical, laboratory, histologic, and radiographic evaluation of ECD patients along with treatment recommendations based on our clinical experience and review of literature in the molecular era. |
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| AbstractList | Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK (RAS-RAF-MEK-ERK) pathway mutations. Typical findings of ECD include central diabetes insipidus, restrictive pericarditis, perinephric fibrosis, and sclerotic bone lesions. The histopathologic diagnosis of ECD is often challenging due to nonspecific inflammatory and fibrotic findings on histopathologic review of tissue specimens. Additionally, the association of ECD with unusual tissue tropism and an insidious onset often results in diagnostic errors and delays. Most patients with ECD require treatment, except for a minority of patients with minimally symptomatic single-organ disease. The first ECD consensus guidelines were published in 2014 on behalf of the physicians and researchers within the Erdheim-Chester Disease Global Alliance. With the recent molecular discoveries and the approval of the first targeted therapy (vemurafenib) for BRAF-V600-mutant ECD, there is a need for updated clinical practice guidelines to optimize the diagnosis and treatment of this disease. This document presents consensus recommendations that resulted from the International Medical Symposia on ECD in 2017 and 2019. Herein, we include the guidelines for the clinical, laboratory, histologic, and radiographic evaluation of ECD patients along with treatment recommendations based on our clinical experience and review of literature in the molecular era.Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK (RAS-RAF-MEK-ERK) pathway mutations. Typical findings of ECD include central diabetes insipidus, restrictive pericarditis, perinephric fibrosis, and sclerotic bone lesions. The histopathologic diagnosis of ECD is often challenging due to nonspecific inflammatory and fibrotic findings on histopathologic review of tissue specimens. Additionally, the association of ECD with unusual tissue tropism and an insidious onset often results in diagnostic errors and delays. Most patients with ECD require treatment, except for a minority of patients with minimally symptomatic single-organ disease. The first ECD consensus guidelines were published in 2014 on behalf of the physicians and researchers within the Erdheim-Chester Disease Global Alliance. With the recent molecular discoveries and the approval of the first targeted therapy (vemurafenib) for BRAF-V600-mutant ECD, there is a need for updated clinical practice guidelines to optimize the diagnosis and treatment of this disease. This document presents consensus recommendations that resulted from the International Medical Symposia on ECD in 2017 and 2019. Herein, we include the guidelines for the clinical, laboratory, histologic, and radiographic evaluation of ECD patients along with treatment recommendations based on our clinical experience and review of literature in the molecular era. Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK (RAS-RAF-MEK-ERK) pathway mutations. Typical findings of ECD include central diabetes insipidus, restrictive pericarditis, perinephric fibrosis, and sclerotic bone lesions. The histopathologic diagnosis of ECD is often challenging due to nonspecific inflammatory and fibrotic findings on histopathologic review of tissue specimens. Additionally, the association of ECD with unusual tissue tropism and an insidious onset often results in diagnostic errors and delays. Most patients with ECD require treatment, except for a minority of patients with minimally symptomatic single-organ disease. The first ECD consensus guidelines were published in 2014 on behalf of the physicians and researchers within the Erdheim-Chester Disease Global Alliance. With the recent molecular discoveries and the approval of the first targeted therapy (vemurafenib) for BRAF-V600-mutant ECD, there is a need for updated clinical practice guidelines to optimize the diagnosis and treatment of this disease. This document presents consensus recommendations that resulted from the International Medical Symposia on ECD in 2017 and 2019. Herein, we include the guidelines for the clinical, laboratory, histologic, and radiographic evaluation of ECD patients along with treatment recommendations based on our clinical experience and review of literature in the molecular era. |
| Author | Goyal, Gaurav Haroche, Julien Kurokawa, Mineo Jacobsen, Eric D Collin, Matthew Cohen-Aubart, Fleur Vaglio, Augusto Janku, Filip Dagna, Lorenzo Picarsic, Jennifer Girschikofsky, Michael Shpilberg, Ofer McClain, Kenneth L Goodman, Aaron M Toyama, Kazuhiro Heaney, Mark L Abdo, André Estrada-Veras, Juvianee I Cao, Xin-Xin Durham, Benjamin H Mazor, Roei D Go, Ronald S Hendrie, Paul Hershkovitz-Rokah, Oshrat Diamond, Eli L |
| Author_xml | – sequence: 1 givenname: Gaurav surname: Goyal fullname: Goyal, Gaurav organization: Division of Hematology-Oncology, University of Alabama at Birmingham, Birmingham, AL – sequence: 2 givenname: Mark L surname: Heaney fullname: Heaney, Mark L organization: Department of Hematology, Columbia University Medical Center, New York, NY – sequence: 3 givenname: Matthew surname: Collin fullname: Collin, Matthew organization: Newcastle upon Tyne Hospitals, Newcastle upon Tyne, United Kingdom – sequence: 4 givenname: Fleur surname: Cohen-Aubart fullname: Cohen-Aubart, Fleur organization: Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France – sequence: 5 givenname: Augusto surname: Vaglio fullname: Vaglio, Augusto organization: Biomedical, Experimental and Clinical Sciences, University of Florence, Florence, Italy – sequence: 6 givenname: Benjamin H surname: Durham fullname: Durham, Benjamin H organization: Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY – sequence: 7 givenname: Oshrat surname: Hershkovitz-Rokah fullname: Hershkovitz-Rokah, Oshrat organization: Department of Molecular Biology, Faculty of Natural Sciences, Ariel University, Ariel, Israel – sequence: 8 givenname: Michael surname: Girschikofsky fullname: Girschikofsky, Michael organization: Internal Medicine I (Hemostasis, Hematology and Stem Cell Transplantation and Medical Oncology), Ordensklinikum Linz Elisabethinen, Linz, Austria – sequence: 9 givenname: Eric D surname: Jacobsen fullname: Jacobsen, Eric D organization: Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA – sequence: 10 givenname: Kazuhiro surname: Toyama fullname: Toyama, Kazuhiro organization: Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan – sequence: 11 givenname: Aaron M surname: Goodman fullname: Goodman, Aaron M organization: Division of Blood and Marrow Transplantation, Department of Medicine, University of California San Diego, La Jolla, CA – sequence: 12 givenname: Paul surname: Hendrie fullname: Hendrie, Paul organization: Division of Hematology, University of Washington, Seattle, WA – sequence: 13 givenname: Xin-Xin surname: Cao fullname: Cao, Xin-Xin organization: Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences/Peking Union Medical College, Beijing, China – sequence: 14 givenname: Juvianee I surname: Estrada-Veras fullname: Estrada-Veras, Juvianee I organization: Walter Reed National Military Medical Center, Bethesda, MD – sequence: 15 givenname: Ofer surname: Shpilberg fullname: Shpilberg, Ofer organization: Clinic of Histiocytic Neoplasms, Institute of Hematology, Assuta Medical Center, Tel-Aviv, Israel – sequence: 16 givenname: André surname: Abdo fullname: Abdo, André organization: Oncology Center, Hospital Alemão Oswaldo Cruz, São Paulo, Brazil – sequence: 17 givenname: Mineo surname: Kurokawa fullname: Kurokawa, Mineo organization: Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan – sequence: 18 givenname: Lorenzo surname: Dagna fullname: Dagna, Lorenzo organization: Department of Medicine, Vita-Salute San Raffaele University, Milan, Italy – sequence: 19 givenname: Kenneth L surname: McClain fullname: McClain, Kenneth L organization: Department of Pediatrics, Baylor College of Medicine, Houston, TX – sequence: 20 givenname: Roei D surname: Mazor fullname: Mazor, Roei D organization: Clinic of Histiocytic Neoplasms, Institute of Hematology, Assuta Medical Center, Tel Aviv, Israel – sequence: 21 givenname: Jennifer surname: Picarsic fullname: Picarsic, Jennifer organization: Division of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH – sequence: 22 givenname: Filip surname: Janku fullname: Janku, Filip organization: Department of Investigational Cancer Therapeutics, The University of Texas MD Anderson Cancer Center, Houston, TX – sequence: 23 givenname: Ronald S surname: Go fullname: Go, Ronald S organization: Division of Hematology, Mayo Clinic, Rochester, MN – sequence: 24 givenname: Julien surname: Haroche fullname: Haroche, Julien organization: Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2 Maladies Auto-Immunes et Systémiques, Centre National de Références des Histiocytoses, Hôpital Pitié-Salpêtrière, Paris, France; and – sequence: 25 givenname: Eli L surname: Diamond fullname: Diamond, Eli L organization: Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, NY |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/32187362$$D View this record in MEDLINE/PubMed |
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| Snippet | Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK... |
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| SubjectTerms | Clinical Trials as Topic Erdheim-Chester Disease - diagnosis Erdheim-Chester Disease - genetics Erdheim-Chester Disease - therapy Female Histiocytosis, Langerhans-Cell - diagnosis Histiocytosis, Langerhans-Cell - genetics Histiocytosis, Langerhans-Cell - therapy Humans Male MAP Kinase Signaling System - drug effects MAP Kinase Signaling System - genetics Molecular Targeted Therapy Mutation Prognosis Proto-Oncogene Proteins B-raf - antagonists & inhibitors Proto-Oncogene Proteins B-raf - genetics |
| Title | Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era |
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