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https://www.readbyqxmd.com/read/28295484/who-2016-classification-changes-and-advancements-in-the-diagnosis-of-miscellaneous-primary-cns-tumours
#1
Felix Sahm, David E Reuss, Caterina Giannini
This short review highlights significant changes and recent findings incorporated to varying extent in the WHO 2016 definition of a variety of tumours, including peripheral nerve sheath tumours, meningiomas, mesenchymal non-meningothelial tumours, melanocytic tumours, lymphomas and histiocytic tumours, germ cell tumours and non-neuroendocrine pituitary tumours. Most notable classification changes include: adding "hybrid nerve sheath tumours" to the spectrum of benign nerve sheath tumours; an updated definition of atypical meningioma (WHO grade II), including cases with brain invasion; recognizing dural solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) as a single tumour entity characterized by NAB2 and STAT6 gene fusions for which the term solitary SFT/HPC was chosen; recognizing that pituitary granular cell tumour, spindle cell oncocytoma, and pituicytoma all share nuclear expression of TTF-1, possibly representing a spectrum of a single nosological entity derived from posterior pituitary glial cells...
March 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28268124/-usefulness-of-nuclear-medicine-in-erdheim-chester-disease-a-lille-experience
#2
A Adens, P Landy, L Terriou, C Baillet, A Beron, M Lambert, D Launay, D Huglo
INTRODUCTION: Erdheim-Chester disease is a rare form of non-langerhans histiocytosis and its etiology is still not well established. The aims of the study were to assess the value of the bone scintigraphy and the (18)F-FDG PET/CT for the diagnostic and for the latter in the therapeutic evaluation. METHODS: We retrospectively reviewed 49 patients suspected of Erdheim-Chester disease between 2004 and 2016. Bone scintigraphy was compared with histopathology and PET-CT to conventional morphological examinations and bone scintigraphy...
March 3, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28262109/adult-xanthogranulomatous-disease-of-the-orbit-clinical-presentations-evaluation-and-management
#3
J M Ortiz Salvador, D Subiabre Ferrer, A Pérez Ferriols
Adult xanthogranulomatous disease of the orbit refers to a heterogeneous group of clinical syndromes with differing degrees of systemic involvement and distinct prognoses. The different syndromes all present clinically with progressively enlarging, yellowish lesions of the orbit. Histologically, the lesions are characterized by an inflammatory infiltrate of foam cells and Touton-type multinucleated giant cells. The xanthomatized histiocytes are CD68(+), S100(-), and CD1a(-). There are 4 clinical forms of xanthogranulomatous disease of the orbit: adult xanthogranulomatous disease of the orbit, adult onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, and Erdheim-Chester disease...
March 2, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28253394/clinical-and-radiologic-responses-to-cladribine-for-the-treatment-of-erdheim-chester-disease
#4
Gaurav Goyal, Mithun V Shah, Timothy G Call, Mark R Litzow, William J Hogan, Ronald S Go
Importance: While cladribine is best known for the treatment of hairy cell leukemia and other lymphoid cancers, it also has activity against myeloid neoplasms, such as Erdheim-Chester disease (ECD). Objective: To assess the efficacy of cladribine (2-chloro-2'-deoxyadenosine) in the treatment of ECD. Design, Setting, and Participants: This study was a single-institution retrospective medical record review from January 1, 1998, to April 6, 2016, at a tertiary academic medical center...
March 2, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28222655/a-cytologic-diagnosis-of-braf-v600e-erdheim-chester-disease-on-pericardial-fluid
#5
P Chasseur, M Kyriakopoulou, B Vokaer, I Beukinga, R Casado Arroyo, E Cogan, B Couturier
We report the case of a 74-year-old woman admitted to the emergency unit for resting dyspnea. Clinical presentation, cardiac MRI and echocardiography were consistent with cardiac tamponade requiring emergency pericardiocentesis. Cytologic examination of the pericardial fluid revealed the presence of CD68(pos) CD1a(neg) S100(neg) foamy histiocytes (Fig. 1). Additional investigations complied with the diagnosis of Erdheim-Chester histiocytosis. Treatment with αIFN was initiated but the patient developed severe neutropenia that contraindicated further administration...
February 22, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28217025/the-imaging-findings-of-erdheim-chester-disease-a-multimodality-approach-to-diagnosis-and-staging
#6
Patrick Martineau, Matthieu Pelletier-Galarneau, Wanzhen Zeng
Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytic disorder. The most common manifestations consist of polyostotic sclerotic lesions with the majority of cases also demonstrating soft tissue involvement of the sinuses, retroperitoneum, large vessels, heart, lungs, and central nervous system. Nuclear medicine can play an important role in assessing the extent of the disease with bone scintigraphy and fluorodeoxyglucose (FDG)-positron emission tomography (PET). We present the case of a middle-aged female who initially presented with tooth pain...
January 2017: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/28214412/histiocytoses-emerging-neoplasia-behind-inflammation
#7
REVIEW
Julien Haroche, Fleur Cohen-Aubart, Barret J Rollins, Jean Donadieu, Frédéric Charlotte, Ahmed Idbaih, Augusto Vaglio, Omar Abdel-Wahab, Jean-François Emile, Zahir Amoura
Histiocytoses are disorders characterised by inflammation and the accumulation of cells derived from the monocyte and macrophage lineages, which results in tissue damage. Although they are often considered rare disorders with protean clinical manifestations, considerable advances in the understanding of their genetics have led to increased clinical recognition of these conditions, and fuelled further insights into their pathogenesis. In this Review, we describe insights into the cells of origin, molecular pathology, clinical features, and treatment strategies for some of the most common histiocytic disorders, including Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease...
February 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28194436/real-time-genomic-profiling-of-histiocytoses-identifies-early-kinase-domain-braf-alterations-while-improving-treatment-outcomes
#8
Lynn H Lee, Anjelika Gasilina, Jayeeta Roychoudhury, Jason Clark, Francis X McCormack, Joseph Pressey, Michael S Grimley, Robert Lorsbach, Siraj Ali, Mark Bailey, Philip Stephens, Jeffrey S Ross, Vincent A Miller, Nicolas N Nassar, Ashish R Kumar
Many patients with histiocytic disorders such as Langerhans cell histiocytosis (LCH) or Erdheim-Chester disease (ECD) have treatment-refractory disease or suffer recurrences. Recent findings of gene mutations in histiocytoses have generated options for targeted therapies. We sought to determine the utility of prospective sequencing of select genes to further characterize mutations and identify targeted therapies for patients with histiocytoses. Biopsies of 72 patients with a variety of histiocytoses underwent comprehensive genomic profiling with targeted DNA and RNA sequencing...
February 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28182116/effective-braf-inhibitor-vemurafenib-therapy-in-a-2-year-old-patient-with-sequentially-diagnosed-langerhans-cell-histiocytosis-and-erdheim-chester-disease
#9
Zsófia Váradi, Rita Bánusz, Judit Csomor, Krisztián Kállay, Edit Varga, Gabriella Kertész, Monika Csóka
Erdheim-Chester disease (ECD) is a rare histiocytic disorder, characterized by the xanthomatous infiltration of tissues by CD68-positive and CD1a-/CD100-negative foamy histiocytes. In childhood, ECD is exceptionally rare, and only a dozen cases have been published so far. The cooccurence of Langerhans cell histiocytosis (LCH) and ECD is even rarer. Here, we report a 2-year-old boy, the youngest patient in the literature so far, who was diagnosed with concomitant BRAF mutation-positive LCH and ECD. In his case, conventional LCH treatment proved to be ineffective, but he is the youngest patient who was successfully treated with the BRAF inhibitor vemurafenib...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28153505/three-cases-of-erdheim-chester-disease-with-intraocular-manifestations-imaging-and-histopathology-findings-of-a-rare-entity
#10
Anna C S Tan, Suzanne Yzer, Neal Atebara, Brian P Marr, Robert M Verdijk, Virgil A S H Dalm, K Bailey Freund, Lawrence Yannuzzi, Tom Missotten
PURPOSE: To report intraocular manifestations of ECD with multimodal imaging. DESIGN: A retrospective observational case series. METHODS: This was a multi-center case series of three patients with confirmed tissue diagnosis of ECD that showed intraocular manifestations and were imaged at baseline and follow-up visits. RESULTS: Intraocular manifestations are rarely observed in association with Erdheim-Chester disease (ECD)...
January 30, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28151461/a-rare-case-of-erdheim-chester-disease-in-the-breast
#11
Razan F Binyousef, Aboelkhair M Al-Gahmi, Zahid Rahman Khan, Elham Rawah
Erdheim-Chester disease (ECD) is rare non-Langerhans histiocytosis with distinctive radiologic and pathologic entities. We report a rare case of which ECD was involving the breast with only eight similar cases reported in the English literature. Our patient was a 52-year-old female patient with ECD involving the breast, mesentery, left kidney, retroperitoneum and the skeleton. The diagnosis was based on distinctive imaging and histopathological findings. The patient received a new novel treatment as part of a clinical trial in the United States and showed clinical and radiological improvement...
January 2017: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/28149747/an-unusual-thoracic-localizations-of-erdheim-chester-disease-a-case-report
#12
C Massaccesi, S Colella, F Fioretti, V D'Emilio, G Panella, G Primomo, F Barbisan, R Pela, V Poletti
Erdheim- Chester disease is a rare non- Langerhans cell histiocytosis that usually involves the bones, heart, central nervous system, retroperitoneum, eyes, kidneys, skin and adrenals. Lungs are affected in up to one-half cases; at CT scan various patterns are described: interstitial disease, consolidations, micronodules and microcysts, with or without pleural involvement. We presented a case of a 59 year-old man with unusual intrathoracic manifestation of Erdheim- Chester disease. Singularities of our report are the lonely thoracic involvement at the onset of the disease and a histiocytic lesion in the posterior mediastinum...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28099231/vemurafenib-braf-inhibitor-therapy-for-orbital-erdheim-chester-disease
#13
Adit Gupta, Amir Yeganeh, Daniel Rootman, Robert Goldberg
Erdheim-Chester disease is a rare xanthogranulomatous systemic disease, which involves the orbit in some cases. Through this case report, the authors review the treatment modalities used in orbital Erdheim-Chester disease and explore a newer modality of treatment. Cases of orbital Erdheim-Chester disease were identified in the literature utilizing a PubMed search and all the treatment modalities were reviewed. The response to treatment of orbital Erdheim-Chester disease has been poor with the various medical and surgical treatment modalities used in the past...
January 17, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28095017/spectrum-of-xanthogranulomatous-processes-in-the-abdomen-and-pelvis-a-pictorial-review-of-infectious-inflammatory-and-proliferative-responses
#14
Kelsey S Bourm, Christine O Menias, Kamran Ali, Kinan Alhalabi, Khaled M Elsayes
OBJECTIVE: Xanthogranulomatous (XG) processes are rare inflammatory conditions with the characteristic pathologic feature of lipid-laden macrophages or histiocyte cells. Imaging findings are nonspecific and can simulate aggressive neoplastic processes. XG processes can be caused by infection, inflammation, histolytic process, or an inherited lysosomal disorder. XG infectious processes are mainly seen in cholecystitis and pyelonephritis, but several other organs can also be involved. Histiocytic processes can be divided into Langerhans and non-Langerhans cell histiocytosis...
March 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28080175/combination-of-adalimumab-with-lower-dose-of-methylprednisolone-in-erdheim-chester-disease-with-systemic-involvement
#15
Xin Jia, Xiaoxuan Ning, Qingxian Bai, Nan Leng, Shengjun Wang, Xiaoming Wang, Shoujing Yang
No abstract text is available yet for this article.
January 12, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/28078666/pediatric-erdheim-chester-disease-with-aggressive-skin-manifestations
#16
H-H Su, W Wu, Y Guo, H-D Chen, S-J Shan
Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, is uncommon and characterized by the accumulation of CD68+/CD1a- foamy histiocytes. It is extremely rare in children. The skin lesions of pediatric ECD was not systemically described before. We report a case of ECD in a 3.5 year old Chinese boy. The patient present with generalized skin and bone involvement for 3 years duration. Marked generalized annular maculopapular lesions with central atrophy which differed from previously reported adult xanthoma-like papules or periorbital xanthelasma-like lesions...
January 12, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28051224/erdheim-chester-disease-and-radiobiphosphonates-bone-scan-from-a-simple-peripheral-form-to-a-rare-axial-location-a-case-report
#17
Salah Oueriagli Nabih
The Erdheim-Chester disease is a rare form of non Langherans histiocytosis. It can be expressed by a multi systemic disease interesting: bone, lung, heart, brain, retro peritoneum, skin and retro-orbital tissue. The diagnosis is established under the radiological aspect, bone scan uptake and immunohistological profile. The bone lesions are almost constant and rather characteristic of bone scintigraphy. We related the case of a young female patient of 26 years old with Erdheim-Chester bone disease. Diagnosis was revealed by diabetes insipidus (nerve damage) and diffuses bones pain...
June 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27995058/erdheim-chester-disease-moving-away-from-the-orphan-diseases-a-case-report
#18
Jessica M Stempel, Jean G Bustamante Alvarez, Andres Mora Carpio, Varun Mittal, Claudia Dourado
With approximately 750 cases reported, Erdheim-Chester disease is an exceedingly rare histiocyte cell disorder. Affected sites typically include long bones, large vessels and central nervous system. However, cutaneous and pulmonary involvement can also occur. The diagnosis is ascertained by identification of foamy histiocytes positive for CD68, CD163, and factor XIIIa on immunoperoxidase staining. Recently published literature have described an association between Erdheim-Chester disease and BRAF V600E mutation...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27940476/trametinib-after-disease-reactivation-under-dabrafenib-in-erdheim-chester-disease-with-both-braf-and-kras-mutations
#19
Thierry M Nordmann, Freimut D Juengling, Mike Recher, Christoph T Berger, Daniel Kalbermatten, Andreas Wicki, Aino Paasinen-Sohns, Gieri Cathomas, Alexandar Tzankov, Thomas Daikeler
Major advances have been made in understanding the pathogenesis of Erdheim-Chester disease (ECD) leading to novel treatment strategies. Targeted therapies such as BRAF inhibition have shown a significant impact on disease management, emphasizing the importance of the activated mitogen-associated protein kinase pathway in this disease. However, incomplete responsiveness, potentially limiting adverse effects, and the occurrence of treatment resistance to BRAF inhibition observed in other BRAF-mutant malignancies imply the importance of therapeutic strategies beyond BRAF inhibition...
February 16, 2017: Blood
https://www.readbyqxmd.com/read/27900869/-evaluation-of-five-years-of-treatment-of-erdheim-chester-disease-with-anakinra-case-report-and-overview-of-literature
#20
Zdeněk Adam, Hana Petrášová, Zdeněk Řehák, Renata Koukalová, Marta Krejčí, Luděk Pour, Eva Vetešníková, Aleš Čermák, Sabina Ševčíková, Petr Szturz, Zdeněk Král, Jiří Mayer
Erdheim-Chester disease is a histiocytic neoplasm of diseases from the group of non-Langerhans-cell histiocytoses, formed by infiltrates of foamy histiocytes. These pathological histiocytes produce pro-inflammatory cytokines. Therefore Erdheim-Chester disease is called inflammatory histiocytary neoplasm. The disease is accompanied by clinical symptoms of systemic inflammatory response, i.e. B symptoms. Imaging examinations detect typical osteosclerotic changes affecting diaphyses and metaphyses of the lower long bones and fibrotic changes which affect the aorta wall and the vessels leading from it...
2016: Vnitr̆ní Lékar̆ství
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