Database : MEDLINE
Search on : Erdheim-Chester and Disease [Words]
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[PMID]: 29188284
[Au] Autor:Diamond EL; Subbiah V; Lockhart AC; Blay JY; Puzanov I; Chau I; Raje NS; Wolf J; Erinjeri JP; Torrisi J; Lacouture M; Elez E; Martínez-Valle F; Durham B; Arcila ME; Ulaner G; Abdel-Wahab O; Pitcher B; Makrutzki M; Riehl T; Baselga J; Hyman DM
[Ad] Address:Memorial Sloan Kettering Cancer Center, New York, New York.
[Ti] Title:Vemurafenib for BRAF V600-Mutant Erdheim-Chester Disease and Langerhans Cell Histiocytosis: Analysis of Data From the Histology-Independent, Phase 2, Open-label VE-BASKET Study.
[So] Source:JAMA Oncol;4(3):384-388, 2018 Mar 01.
[Is] ISSN:2374-2445
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Importance: The histiocytic neoplasms Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) are highly enriched for BRAF V600 mutations and have been previously shown to be responsive to treatment with vemurafenib, an inhibitor of the BRAF V600 kinase. However, the long-term efficacy and safety of prolonged vemurafenib use in these patients are not defined. Here we analyze the final efficacy and safety data for vemurafenib in patients with ECD and LCH enrolled in the VE-BASKET study. Objective: To determine the efficacy and safety of vemurafenib in adults with ECD or LCH enrolled in the VE-BASKET study. Design, Setting, and Participants: The VE-BASKET study was an open-label, nonrandomized, multicohort study for patients with nonmelanoma cancers harboring the BRAF V600 mutation. Patients with BRAF V600-mutant ECD or LCH were enrolled in an "other solid tumor" cohort of the VE-BASKET study, and they were enrolled in the present study. Interventions: Patients received vemurafenib, 960 mg, twice daily continuously until disease progression, study withdrawal, or occurrence of intolerable adverse effects. Main Outcomes and Measures: The primary end point was confirmed objective response rate (ORR) by Response Evaluation Criteria in Solid Tumors (RECIST, version 1.1). Secondary end points included progression-free survival (PFS), overall survival (OS), metabolic response by modified positron-emission tomography (PET) Response Criteria in Solid Tumors (PERCIST) using 18F-fluorodeoxyglucose (FDG)-PET/computed tomography (CT), and safety. Results: A total of 26 patients from the VE-BASKET trial (22 with ECD, 4 with LCH) were included in the present study (14 women and 12 men; median age, 61 years; age range, 51-74 years). The confirmed ORR was 61.5% (95% CI, 40.6%-79.8%) in the overall cohort and 54.5% (95% CI, 32.2%-75.6%) in patients with ECD. All evaluable patients achieved stable disease or better. The median PFS and OS had not been reached in the overall cohort at study closure despite a median follow-up of 28.8 months; 2-year PFS was 86% (95% CI, 72%-100%), and 2-year OS was 96% (95% CI, 87%-100%). All 15 patients evaluated by FDG-PET/CT achieved a metabolic response, including 12 patients (80%) with a complete metabolic response. The most common adverse events (AEs) in the overall cohort included arthralgia, maculopapular rash, fatigue, alopecia, prolonged QT interval, skin papilloma, and hyperkeratosis. Hypertension and dermatologic AEs occurred at higher rates than those reported in metastatic melanoma. Conclusions and Relevance: In this study, vemurafenib had prolonged efficacy in patients with BRAF V600-mutant ECD and LCH and warrants consideration as a new standard of care for these patients.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180311
[Lr] Last revision date:180311
[St] Status:In-Data-Review
[do] DOI:10.1001/jamaoncol.2017.5029

  2 / 769 MEDLINE  
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[PMID]: 29485431
[Au] Autor:Akin EA; Osman M; Ellenbogen AL
[Ti] Title:FDG PET/CT Findings of Erdheim-Chester Disease: Radiologic Response to a Novel Treatment Regimen.
[So] Source:Clin Nucl Med;, 2018 Feb 27.
[Is] ISSN:1536-0229
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis with deposition of lipid-laden macrophages in numerous organs. A 74-year-old man with a history of coronary artery disease, hypertension, and hyperlipidemia presented with nonspecific symptoms including back pain, nausea, vomiting, vertigo, and left leg pain. A neutrophil-predominant elevated WBC count and a bone biopsy revealing histiocytic proliferation positive for CD68 and CD163 and negative for S100 was noted. FDG PET/CT, MRA, and CTA images were obtained. We review the radiologic hallmarks of ECD and demonstrate the radiologic manifestations of response to combined BRAF and MEK inhibitor treatment.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[St] Status:Publisher
[do] DOI:10.1097/RLU.0000000000002033

  3 / 769 MEDLINE  
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[PMID]: 28455460
[Au] Autor:Bentel JM; Thomas MA; Rodgers JJ; Arooj M; Gray E; Allcock R; Fermoyle S; Mancera RL; Cannell P; Parry J
[Ad] Address:Anatomical Pathology, PathWest Laboratory Medicine, Fiona Stanley Hospital, Murdoch, Australia.
[Ti] Title:Erdheim-Chester disease associated with a novel, complex BRAF p.Thr599_Val600delinsArgGlu mutation.
[So] Source:BMJ Case Rep;2017, 2017 Apr 28.
[Is] ISSN:1757-790X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BRAF mutation testing to determine eligibility for treatment with vemurafenib was performed on archival skin lesions of a 54-year-old patient diagnosed with Erdheim-Chester disease (ECD) in 1999. Sanger sequencing of DNA extracted from a 2008 skin lesion identified two non-contiguous base substitutions in , which were shown by next-generation sequencing (NGS) to be located in the same allele. Due to its long-standing duration, molecular evolution of disease was possible; however, both Sanger and NGS of a 2000 skin lesion were unsuccessful due to the poor quality of DNA. Finally, droplet digital PCR using a probe specific for this novel mutation detected the complex BRAF mutation in both the 2000 and 2008 lesions, indicating this case to be ECD with a novel underlying BRAF p.Thr599_Val600delinsArgGlu mutation. Although well at present, molecular modelling of the mutant BRAF suggests suboptimal binding of vemurafenib and hence reduced therapeutic effectiveness.
[Mh] MeSH terms primary: Erdheim-Chester Disease/genetics
Histiocytosis, Langerhans-Cell/genetics
Mutation
Proto-Oncogene Proteins B-raf/genetics
[Mh] MeSH terms secundary: Enzyme Inhibitors/therapeutic use
Erdheim-Chester Disease/etiology
Erdheim-Chester Disease/pathology
Histiocytosis, Langerhans-Cell/pathology
Humans
Indoles/therapeutic use
Male
Middle Aged
Patient Outcome Assessment
Skin/pathology
Skin Neoplasms/genetics
Sulfonamides/therapeutic use
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Enzyme Inhibitors); 0 (Indoles); 0 (Sulfonamides); 207SMY3FQT (vemurafenib); EC 2.7.11.1 (Proto-Oncogene Proteins B-raf)
[Em] Entry month:1802
[Cu] Class update date: 180220
[Lr] Last revision date:180220
[Js] Journal subset:IM
[Da] Date of entry for processing:170430
[St] Status:MEDLINE

  4 / 769 MEDLINE  
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[PMID]: 29204962
[Au] Autor:Chiapparini L; Cavalli G; Langella T; Venerando A; De Luca G; Raspante S; Marotta G; Pollo B; Lauria G; Cangi MG; Gerevini S; Botturi A; Pareyson D; Dagna L; Salsano E
[Ad] Address:Unit of Neuroradiology, IRCCS Foundation "Carlo Besta" Neurological Institute, Via Celoria 11, 20133, Milan, Italy. luisa.chiapparini@istituto-besta.it.
[Ti] Title:Adult leukoencephalopathies with prominent infratentorial involvement can be caused by Erdheim-Chester disease.
[So] Source:J Neurol;265(2):273-284, 2018 Feb.
[Is] ISSN:1432-1459
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:BACKGROUND: Leukoencephalopathies with prominent involvement of cerebellum and brainstem, henceforward called prominent infratentorial leukoencephalopathies (PILs), encompass a variety of inherited and acquired white matter diseases. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis likely under-diagnosed as cause of adult PIL. METHODS: We reviewed the clinical and laboratory information of ten consecutive sporadic adult patients with PIL of unknown origin, who were investigated for ECD. RESULTS: There were seven males and three females; mean age at clinical onset was 49.6 years (range 38-59); cerebellar ataxia with or without other neurological symptoms was the only or the main clinical manifestation; diabetes insipidus was present in three individuals. Eight patients had white matter focal supratentorial abnormalities, in addition to the infratentorial white matter changes. Six out of eight patients had spinal cord lesions. Thoraco-abdominal CT showed periaortic sheathing in two patients, whole-body FDG-PET revealed increased glucose uptake in the long bones of the legs in five patients, brain FDG-PET showed overt infratentorial hypermetabolism in one patient. In eight patients, ECD was confirmed by bone scintigraphy, pathological data, or both. Two ECD patients treated with vemurafenib showed a marked improvement of neurological symptoms and brain MRI abnormalities at 1 year follow-up. CONCLUSIONS: Symptoms of PIL can be the only clinical manifestation of ECD. Adult patients with PIL of unknown origin should undergo investigations aimed at unveiling ECD, including bone scintigraphy and whole-body FDG-PET. The early diagnosis allows starting disease-modifying therapies of an otherwise life-threatening disease.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180219
[Lr] Last revision date:180219
[St] Status:In-Process
[do] DOI:10.1007/s00415-017-8692-8

  5 / 769 MEDLINE  
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[PMID]: 29191545
[Au] Autor:Wagner KM; Mandel JJ; Goodman JC; Gopinath S; Patel AJ
[Ad] Address:Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA.
[Ti] Title:Intracranial Erdheim-Chester Disease Mimicking Parafalcine Meningioma: Report of Two Cases and Review of the Literature.
[So] Source:World Neurosurg;110:365-370, 2018 Feb.
[Is] ISSN:1878-8769
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis that typically occurs in middle-aged patients. It is usually characterized by multifocal osteosclerotic lesions of the long-bones, however many cases have extraskeletal involvement. Central nervous system (CNS) involvement is common, but isolated CNS involvement at presentation has rarely been reported. CASE DESCRIPTION: Here we report two cases of dural-based ECD mimicking meningioma on imaging with no other identified sites of disease. CONCLUSION: ECD is a rare disease, with isolated CNS involvement reported only a few times in the literature. The significance of this presentation requires additional study and long-term follow up.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180213
[Lr] Last revision date:180213
[St] Status:In-Data-Review

  6 / 769 MEDLINE  
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[PMID]: 29396850
[Au] Autor:Cohen-Aubart F; Emile JF; Carrat F; Helias-Rodzewicz Z; Taly V; Charlotte F; Cluzel P; Donadieu J; Idbaih A; Barete S; Amoura Z; Haroche J
[Ad] Address:Internal Medicine Department 2, Pitié-Salpêtrière Hospital, French National Centre for Rare Systemic Diseases, AP-HP, Paris, 75013, France.
[Ti] Title:Phenotypes and survival in Erdheim-Chester disease: Results from a 165-patient cohort.
[So] Source:Am J Hematol;, 2018 Feb 02.
[Is] ISSN:1096-8652
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:LETTER
[Em] Entry month:1802
[Cu] Class update date: 180210
[Lr] Last revision date:180210
[St] Status:Publisher
[do] DOI:10.1002/ajh.25055

  7 / 769 MEDLINE  
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[PMID]: 29242623
[Au] Autor:Goyal G; Shah MV; Call TG; Hook CC; Hogan WJ; Go RS
[Ad] Address:Division of Hematology, Mayo Clinic, Rochester, MN, USA.
[Ti] Title:Clinical and radiological responses to oral methotrexate alone or in combination with other agents in Erdheim-Chester disease.
[So] Source:Blood Cancer J;7(12):647, 2017 Dec 15.
[Is] ISSN:2044-5385
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:LETTER
[Em] Entry month:1712
[Cu] Class update date: 180209
[Lr] Last revision date:180209
[St] Status:In-Data-Review
[do] DOI:10.1038/s41408-017-0034-7

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[PMID]: 29363511
[Au] Autor:Cohen-Aubart F; Maksud P; Emile JF; Benameur N; Charlotte F; Cluzel P; Amoura Z; Haroche J
[Ad] Address:Internal Medicine Department 2, AP-HP, French National Reference Centre for Rare Systemic Diseases, Pitié-Salpêtrière Hospital, Paris, France.
[Ti] Title:Efficacy of infliximab in the treatment of Erdheim-Chester disease.
[So] Source:Ann Rheum Dis;, 2018 Jan 23.
[Is] ISSN:1468-2060
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:LETTER
[Em] Entry month:1801
[Cu] Class update date: 180124
[Lr] Last revision date:180124
[St] Status:Publisher

  9 / 769 MEDLINE  
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[PMID]: 29357606
[Au] Autor:Chasset F; Haroche J
[Ad] Address:Assistance Publique Hôpitaux de Paris (AP-HP), Service de Dermatologie et d'Allergologie, Hôpital Tenon, 75020, Paris, France.
[Ti] Title:Cutaneous manifestations of paediatric Erdheim-Chester disease, a histiocytosis of the 'L' group.
[So] Source:Br J Dermatol;178(1):31-32, 2018 Jan.
[Is] ISSN:1365-2133
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180123
[Lr] Last revision date:180123
[St] Status:In-Data-Review
[do] DOI:10.1111/bjd.15860

  10 / 769 MEDLINE  
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[PMID]: 29285599
[Au] Autor:Gumeler E; Onur MR; Karaosmanoglu AD; Ozmen M; Akata D; Karcaaltincaba M
[Ad] Address:Department of Radiology, Tip Fakültesi Hastanesi, School of Medicine, Hacettepe University, Sihhiye, 06100, Ankara, Turkey.
[Ti] Title:Computed tomography and magnetic resonance imaging of peripelvic and periureteric pathologies.
[So] Source:Abdom Radiol (NY);, 2017 Dec 28.
[Is] ISSN:2366-0058
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Peripelvic and periureteric areas are frequently overlooked in the imaging evaluations of the urinary system on computed tomography and magnetic resonance imaging. Several neoplastic and non-neoplastic disorders and diseases including lipomatosis, angiomyolipoma and angiolipomatous proliferation, vascular lesions, lymphangiomatosis, Rosai-Dorfman disease, Erdheim-Chester disease, extramedullary hematopoiesis, IgG4-related disease, lymphoma, mesenchymal tumors, trauma, and Antopol-Goldman lesion may involve these areas. Differentiation of these benign or malignant pathologies among themselves and from primary renal pathologies is of utmost importance to expedite the triage of patients for correct treatment approach. In this article, we aim to increase the awareness of the imaging specialists to the typical and atypical imaging features of the entities affecting these areas.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1712
[Cu] Class update date: 171229
[Lr] Last revision date:171229
[St] Status:Publisher
[do] DOI:10.1007/s00261-017-1444-5


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