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https://www.readbyqxmd.com/read/29225878/erdheim-chester-disease-with-atrial-mass-and-perinephric-calcification
#1
Michel Villatoro-Villar, Matthew J Koster
Erdheim-Chester Disease is a multi-systemic condition characterized by non-Langerhans histiocytic infiltration. Cardiovascular involvement with pseudotumoral infiltration of the right atrium is present in approximately one-third of patients and may be asymptomatic. Although retroperitoneal fibrosis is common, perinephric dystrophic calcification has not been previously described.
December 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29214086/erdheim-chester-disease-with-no-skeletal-bone-involvement-and-massive-weight-loss
#2
Hind Salama, Suleiman Kojan, Shaima Abdulrahman, Fahad Azzumeea, Ayman Alhejazi
Erdheim-Chester disease (ECD) is a rare type of non-Langerhans cell histiocytosis, with only 550 cases reported worldwide. ECD is characterized by diffuse histiocytic infiltration of multiorgans. The age of presentation of this disease is typically between 40 and 70 years. Bone disease is the most common symptom, as unique radiological findings of long bone sclerosis occur in 96% of cases. Furthermore, BRAF V600E mutation is detected in 60% of ECD cases. In this manuscript, we are describing a unique case of ECD; the patient is younger than most reported cases and has no bone pain or any skeletal involvement...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29204962/adult-leukoencephalopathies-with-prominent-infratentorial-involvement-can-be-caused-by-erdheim-chester-disease
#3
Luisa Chiapparini, Giulio Cavalli, Tiziana Langella, Anna Venerando, Giacomo De Luca, Sergio Raspante, Giorgio Marotta, Bianca Pollo, Giuseppe Lauria, Maria Giulia Cangi, Simonetta Gerevini, Andrea Botturi, Davide Pareyson, Lorenzo Dagna, Ettore Salsano
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...
December 4, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29195598/orphan-drug-approval-for-erdheim-chester-disease
#4
EDITORIAL
The Lancet Haematology
No abstract text is available yet for this article.
December 2017: Lancet Haematology
https://www.readbyqxmd.com/read/29192649/the-histopathology-of-erdheim-chester-disease-a-comprehensive-review-of-a-molecularly-characterized-cohort
#5
Neval Ozkaya, Marc K Rosenblum, Benjamin H Durham, Janine D Pichardo, Omar Abdel-Wahab, Meera R Hameed, Klaus J Busam, William D Travis, Eli L Diamond, Ahmet Dogan
Erdheim-Chester disease is a rare, non-Langerhans cell histiocytosis histologically characterized by multi-systemic proliferation of mature histiocytes in a background of inflammatory stroma. The disease can involve virtually any organ system; most commonly the bones, skin, retroperitoneum, heart, orbit, lung, and brain are affected. Although a histiocytic proliferation is the histological hallmark of the disease, a wide range of morphological appearances have been described as part of case studies or small series...
December 1, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29191545/intracranial-erdheim-chester-disease-mimicking-parafalcine-meningioma-report-of-two-cases-and-review-of-the-literature
#6
Kathryn M Wagner, Jacob J Mandel, J Clay Goodman, Shankar Gopinath, Akash J Patel
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. Here we report two cases of dural-based ECD mimicking meningioma on imaging with no other identified sites of disease.
November 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29188284/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
#7
Eli L Diamond, Vivek Subbiah, A Craig Lockhart, Jean-Yves Blay, Igor Puzanov, Ian Chau, Noopur S Raje, Jurgen Wolf, Joseph P Erinjeri, Jean Torrisi, Mario Lacouture, Elena Elez, Ferran Martínez-Valle, Benjamin Durham, Maria E Arcila, Gary Ulaner, Omar Abdel-Wahab, Bethany Pitcher, Martina Makrutzki, Todd Riehl, José Baselga, David M Hyman
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...
November 29, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/29145922/erdheim-chester-disease-and-palatal-tremor
#8
Natalie E Parks, R Allan Purdy
No abstract text is available yet for this article.
November 17, 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/29133287/basket-study-yields-approval-for-rare-blood-cancer
#9
(no author information available yet)
The FDA, in a regulatory first, approved a targeted therapy based on a basket study. The move, which expanded the indications for the BRAF inhibitor vemurafenib to include Erdheim-Chester disease, points to a new approval pathway for drugs that treat rare cancers.
November 13, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/29132763/spontaneous-bilateral-pneumothoraces-in-erdheim-chester-disease
#10
Ruben Eduardo Pacios Blanco, Luis Gorospe Sarasua, María Eugenia Reguero
No abstract text is available yet for this article.
November 10, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/29099004/isolated-pulmonary-involvement-in-erdheim-chester-disease
#11
Enambir Singh Josan, Jason W Green, Syed Imran M Zaidi, Jayantilal B Mehta
Erdheim-Chester disease is a rare non-Langerhans cell histiocytic disorder. It is primarily a disease of the long bones. Pulmonary involvement in systemic disease is detected in about half the reported cases. Isolated lung involvement is extremely rare with no clear recommendations for treatment. A 52-year-old caucasian male was evaluated for 1.9 cm × 1.6 cm spiculated nodule in the right upper lobe. Pulmonary function testing and bronchoscopy with endobronchial ultrasound, transbronchial biopsy, and microbiology were inconclusive...
November 2017: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/29097410/f-18-fdg-pet-ct-in-erdheim-chester-disease-imaging-findings-and-potential-braf-mutation-biomarker
#12
Jason Young, Geoffrey Bates Johnson, Robert Charles Murphy, Ronald S Go, Stephen M Broski
This study's purpose was to evaluate F-18 fluorodeoxyglucose positron emission tomography - computed tomography (FDG PET-CT) in the diagnosis, management and treatment of Erdheim-Chester disease (ECD). Methods: Our institutional database (2007-2017) was retrospectively reviewed for patients with pathologically-proven ECD. A chart review recorded demographics, clinical information and five categories of clinical impact. Two radiologists in consensus interpreted the images. Imaging findings were correlated with clinical data...
November 2, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/29096034/cns-erdheim-chester-disease-a-challenge-to-diagnose
#13
Zenggang Pan, Bette K Kleinschmidt-DeMasters
Erdheim-Chester disease (ECD) is a rare nonLangerhans cell histiocytosis. Although approximately 50% of cases eventually involve the central nervous system (CNS), the CNS has seldom been reported as the initial biopsy site. The diagnosis of CNS ECD can be challenging due to morphologic overlap with reactive histiocytic proliferation, Langerhans cell histiocytosis (LCH), and extranodal Rosai-Dorfman disease (RDD). We present 3 cases from our files that illustrate the protean manifestations of ECD. Case 1 was a 47-year-old man with ataxia, dysarthria, and intermittent ophthalmoplegia whose cerebellar biopsy had shown only profuse, nonspecific Rosenthal fiber-rich piloid gliosis; ECD was diagnosed only at autopsy...
December 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29058834/-difficult-diagnosis-of-erdheim-chester-disease-revealed-by-central-diabetes-insipidus
#14
W Alaya, B Zantour, W Ben Salem, W Chebbi, H-M Sfar
The Erdheim Chester disease is a rare form of non Langerhans cell histiocytosis. Its rarity and its unspecific clinical presentation, make that its diagnosis is often delayed. We report the case of a 50 years old female who has an Erdheim Chester disease, revealed by a central diabetes insipidus with thickening of the pituitary stalk, with associated gonadotropin deficiency. The Erdheim Chester disease was suspected because of the association with other evocative systemic lesions: eyelid xanthelasmas and bone lesions in metaphyseal-diaphyseal region of the upper and lower ends of both femurs and tibias on bone scan...
October 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/29038235/rare-tumor-clinic-the-university-of-california-san-diego-moores-cancer-center-experience-with-a-precision-therapy-approach
#15
Shumei Kato, Kellie Kurasaki, Sadakatsu Ikeda, Razelle Kurzrock
BACKGROUND: Patients with rare tumors may lack approved treatments and clinical trial access. Although each rare tumor is uncommon, cumulatively they account for approximately 25% of cancers. We recently initiated a Rare Tumor Clinic that emphasized a precision medicine strategy. MATERIALS AND METHODS: We investigated the first 40 patients presenting at the Rare Tumor Clinic. Next-generation sequencing (NGS) of tissue and plasma-derived, circulating-tumor DNA (ctDNA), and protein markers were assessed...
October 16, 2017: Oncologist
https://www.readbyqxmd.com/read/29025773/erdheim-chester-disease-atypical-presentation-of-a-rare-disease
#16
Cristian Ricardo Calandra, Ariel Bustos, Florencia Falcon, Naomi Arakaki
We report the clinical case of an adult patient referred to our hospital because of trismus due to a tumour in the right infratemporal and pterygomaxillary fossa. He referred hyporexia, weight loss and right trigeminal neuralgia. On physical examination, he had trismus and diplopia. On neuroimaging, the tumour invaded the central nervous system affecting the right temporal lobe and orbit, and the sellar region. Tumour biopsy revealed foamy histiocytes and isolated giant multinuclear cells immunoreactive to CD68 and negative to CD1a and S100...
October 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28912295/erdheim-chester-disease-the-targeted-revolution
#17
Augusto Vaglio, Eli L Diamond
No abstract text is available yet for this article.
September 14, 2017: Blood
https://www.readbyqxmd.com/read/28868020/erdheim-chester-disease-the-importance-of-information-integration
#18
Anna Nikonova, Khashayar Esfahani, Guillaume Chausse, Stephan Probst, Tina Petrogiannis-Haliotis, Hans Knecht, Genevieve Gyger
BACKGROUND: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. CASE PRESENTATION: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28834636/intraosseous-rosai-dorfman-disease-diagnosed-by-touch-imprint-cytology-evaluation-a-case-series
#19
Avani A Pendse, Sara E Wobker, Kevin G Greene, Scott V Smith, Robert J Esther, Leslie G Dodd
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is a rare benign disorder that primarily affects the lymph nodes. Localized lymphadenopathy is the most common clinical manifestation of this disorder. However, RDD has been described in several extra-nodal sites including the head and neck region, soft tissue, skin, upper respiratory tract, gastro-intestinal tract and central nervous system (CNS). Involvement of the bone is considered very rare, occurring in less than 10% patients...
August 23, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28825890/case-245-erdheim-chester-disease
#20
Mark D Mamlouk, Mariam S Aboian, Christine M Glastonbury
History A 53-year-old man experienced headache and double vision that progressed over 1 year. After a traumatic fall, he was hospitalized, and proptosis was identified at physical examination. Laboratory tests were remarkable for leukocytosis. Hematocrit level, thyroid stimulating hormone level, autoimmune antibody level, erythrocyte sedimentation rate, and C-reactive protein level were normal. Computed tomography (CT) of the head revealed bilateral intraconal masses, for which magnetic resonance (MR) imaging of the orbits was subsequently performed ( Fig 1 )...
September 2017: Radiology
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