keyword
https://read.qxmd.com/read/38483831/epicardial-space-comprehensive-anatomy-and-spectrum-of-disease
#1
JOURNAL ARTICLE
Adria Roset-Altadill, Blanca Domenech-Ximenos, Noemi Cañete, Sergi Juanpere, Lucia Rodriguez-Eyras, Alberto Hidalgo, Daniel Vargas, Victor Pineda
The epicardial space (ES) is the anatomic region located between the myocardium and the pericardium. This space includes the visceral pericardium and the epicardial fat that contains the epicardial coronary arteries, cardiac veins, lymphatic channels, and nerves. The epicardial fat represents the main component of the ES. This fat deposit has been a focus of research in recent years owing to its properties and relationship with coronary gossypiboma plaque and atrial fibrillation. Although this region is sometimes forgotten, a broad spectrum of lesions can be found in the ES and can be divided into neoplastic and nonneoplastic categories...
April 2024: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://read.qxmd.com/read/38476885/erdheim%C3%A2-chester-disease-of-multisystem-involvement-with-delayed-diagnosis-a-case-report-and-literature-review
#2
Xiaotong Shi, Guangzhi Sun, Tongguan Li, Mengjiao Xu, Yixuan Liu, Zhankui Wang, Yanfeng Hou
Erdheim-Chester disease (ECD) is a rare tumor of histiocytic origin, characterized by foamy or lipid-laden histiocytes mixed or surrounded by fibrosis that infiltrate multiple organs. Misdiagnosis is common due to the diversity of clinical presentations. The present study reported a case of ECD with the involvements of bone, cardiac, aorta and retroperitoneum. The patient had no obvious clinical symptoms and no noteworthy foamy histiocytes or Touton giant cells were found on pathological examination, delaying the diagnosis...
April 2024: Experimental and Therapeutic Medicine
https://read.qxmd.com/read/38472383/-xanthogranulomatous-adrenalitis-a%C3%A2-rare-and-difficult-differential-diagnosis-of-adrenal-gland-tumors
#3
JOURNAL ARTICLE
Wolfgang Saeger, Andreas M Luebke, S T Mekoula, Jörg-Michael Pahnke
A radiologically diagnosed tumor in a 29-year-old woman with a fever of around 39 °C was operated on under the suspicion of cholecystitis or a liver abscess. A solid tumor was found in the adrenal gland and resected. The frozen section findings did not reveal a clear diagnosis of entity and assignment. Histologically, the tumor was found to consist of densely clustered large histiocyte-like cells with expression of vimentin, CD68, and CD163 as well as negativity for keratin, langerin, and SMA...
March 12, 2024: Pathologie (Heidelb)
https://read.qxmd.com/read/38469821/cardiovascular-manifestations-of-erdheim-chester-disease-a-narrative-review-with-two-cases
#4
REVIEW
Alan Wong, Aditya Sharma, Randy Ramcharitar
Erdheim-Chester disease (ECD) is a rare 'L' (Langerhans) group histiocytic neoplasm that affects a multitude of organ systems, causing osteosclerotic bone lesions, periaortic encasement ('coated' aorta), retroperitoneal fibrosis involving kidneys and ureters ('hairy kidney'), and infiltration of the central nervous system. Cardiovascular involvement can occur in up to 70% of patients and is usually found during computed tomography/magnetic resonance imaging evaluation. When present, cardiovascular symptoms can have wide variability in presentation from asymptomatic to pericarditis, fatal cardiac tamponade, myocardial infarction, conduction abnormalities, heart failure, renal artery stenosis, and claudication...
March 12, 2024: Vascular Medicine
https://read.qxmd.com/read/38427961/typical-bone-scintigraphy-presentation-of-erdheim-chester-disease-in-a-patient-diagnosed-with-igg4-related-disease
#5
JOURNAL ARTICLE
Chloé François, Clémentine Rousselin, Thomas Quemeneur, Franck Demailly, Guillaume Collet, Alexandre Fagart
A 50-year-old woman presented a dry syndrome, joint pain, inflammatory syndrome, polyclonal hypergammaglobulinemia, and tubulointerstitial nephritis. Imaging studies (including FDG PET/CT) revealed infrarenal retroperitoneal fibrosis with periaortitis and hypermetabolic osteosclerotic lesions. Bone scintigraphy demonstrated intense uptake in the femoral, tibial, and radial regions, suggestive of non-Langerhans histiocytosis, specifically Erdheim-Chester disease. A bone biopsy confirmed the presence of IgG4-positive plasma cells but no histiocytes...
April 1, 2024: Clinical Nuclear Medicine
https://read.qxmd.com/read/38427815/erdheim-chester-disease-with-eyelid-and-orbital-involvement-a-review-of-treatment-modalities-at-one-institution-from-2014-to-2022
#6
JOURNAL ARTICLE
Meghan Sharma, Shanlee M Stevens, Michelle M Maeng, Natalya Nagornaya, Rita G Bhatia, Sara T Wester
PURPOSE: To review all cases of Erdheim-Chester disease (ECD) with orbital involvement treated at Bascom Palmer Eye Institute in Miami, Florida from 2014 to 2022 and compare presentations, treatment modalities, and outcomes. METHODS: A retrospective chart review of all patients diagnosed with ECD who presented to Bascom Palmer Eye Institute from 2014 to 2022 was performed. Data collected included demographics, pretreatment history and ophthalmic examination, pathology report, treatment, subsequent examination, and relevant laboratory results...
February 27, 2024: Ophthalmic Plastic and Reconstructive Surgery
https://read.qxmd.com/read/38425778/erdheim-chester-disease-with-tendon-and-muscle-involvement-reports-of-a-rare-presentation
#7
Mahshid Golagha, Fatemeh Dehghani Firouzabadi, Corina Millo, Moozhan Nikpanah, Mark A Ahlman, Rahul H Dave, Juvianee I Estrada-Veras, Kevin O'Brien, Ashkan A Malayeri
Erdheim-Chester disease (ECD) is a rare histiocytic disease that affects multiple systems in the body. While it typically targets long bones, cardiovascular structures, the retroperitoneum, and the central nervous system, reports of tendon and skeletal muscle involvement are scarce. This review presents 2 cases: a case of ECD involving the left Achilles tendon and left abductor hallucis, as well as an unusual manifestation of ECD in the thigh musculature. In Case 1, studies involved a 39-year-old man who initially presented with bone and pituitary involvement...
May 2024: Radiology Case Reports
https://read.qxmd.com/read/38417471/multisystem-erdheim-chester-disease-presenting-with-pericardial-effusion-confirmed-by-the-effusion-cytology-specimen
#8
Shishuo Dai, Xueying Su, Wei-Ping Liu, Yu Wu
Erdheim-Chester disease (ECD) is a rare histiocytosis characterized by the foamy CD68+CD1a- histiocytes infiltrating multiple organs and tissues. ECD might be asymptomatic or present with variable manifestations. The diagnosis of ECD requires characteristic radiological findings and pathological features. Herein, we described a 52-year-old female patient who was admitted to our hospital for recurrent pericardial effusion for two months. She has a medical history of papillary thyroid carcinoma (PTC) and underwent a total thyroidectomy two years before admission...
February 26, 2024: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://read.qxmd.com/read/38408335/letter-to-the-editor-clinical-manifestations-of-erdheim-chester-disease-besides-epilepsy
#9
JOURNAL ARTICLE
Josef Finsterer
No abstract text is available yet for this article.
February 26, 2024: J Neurosurg Case Lessons
https://read.qxmd.com/read/38350070/multiple-nervous-system-involvement-of-erdheim-chester-disease-with-responsive-perinephritic-hypermetabolism
#10
JOURNAL ARTICLE
Zehra Pınar Koç, Pınar Pelin Özcan, Anıl Tombak, Kerim Sönmezoğlu
A 36-year-old man who was at follow-up for histiocytosis had sudden-onset symptoms of unilateral ophthalmic increased pressure. The patient was referred to the FDG PET/CT for determination of involvement with suspicion of Erdheim-Chester disease. The reduction of the FDG uptake in all of the lesions (medial temporal lobes, nasal septum, medulla spinalis in sacral region, as well as perinephritic infiltrations), which were determined by the first PET/CT, was achieved at second imaging.
February 7, 2024: Clinical Nuclear Medicine
https://read.qxmd.com/read/38321789/pericardiectomy-and-mechanical-mitro-aortic-valve-replacement-in-a-young-patient-with-erdheim-chester-disease
#11
JOURNAL ARTICLE
Nerea Lopez Perez, Mathieu van Steenberghe, Jörg D Seebach, Philippe Meyer, Johannes Alexander Lobrinus, Christoph Huber, Mustafa Cikirikcioglu
Erdheim-Chester disease is a rare histiocytosis that primarily affects the skeletal system, but cardiovascular manifestations occur in 75% of cases and are associated with a poor prognosis. Given the small number of cases, the evolution and management of the disease are uncertain. Therefore, it is important to report and share Erdheim-Chester cases. This report presents the case of a young patient with constrictive pericarditis and mitral valve regurgitation resulting from Erdheim-Chester disease.
February 7, 2024: Texas Heart Institute Journal
https://read.qxmd.com/read/38281751/-central-nervous-system-disorders-secondary-to-histiocytoses-neurodegeneration-with-potential-for-improvement
#12
JOURNAL ARTICLE
Akira Morimoto, Kenichi Sakamoto, Ko Kudo, Yoko Shioda
Histiocytoses, including Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), are inflammatory myeloid tumors in which monocyte lineage cells aggregate in various organs, causing tissue damage. Most of these tumors harbor oncogenic mutations in mitogen-activated protein kinase (MAPK) pathway genes, typified by BRAFV600E . Some patients with LCH develop bilateral symmetrical cerebellar lesions and brain atrophy several years after diagnosis when the initial symptoms disappear, leading to cerebellar ataxia and higher cerebral dysfunction...
January 27, 2024: Rinshō Shinkeigaku, Clinical Neurology
https://read.qxmd.com/read/38275171/dabrafenib-and-steroids-for-the-treatment-of-erdheim-chester-disease-with-extensive-cns-involvement-a-case-report
#13
JOURNAL ARTICLE
Carlen A Yuen, Silin Bao, Mya Sandi Aung, Rhea Shishodia, Xiao-Tang Kong
Erdheim-Chester disease (ECD) is an exceedingly rare non-Langerhans cell CD68+ CD1a- S100- histiocytic multi-organ disease. Diagnosis of ECD is often delayed due to non-specific radiographic findings and heterogeneous lesional tissue. Increasingly, the role of genomic alterations is being recognized for both diagnosis and treatment of ECD. More than half of ECD patients harbor the BRAFV600E mutation. Evaluation for this mutation be can falsely negative on immunohistochemical staining and confirmation with molecular analyses is recommended...
January 26, 2024: Personalized Medicine
https://read.qxmd.com/read/38262396/rare-onset-of-erdheim-chester-disease-in-children-and-young-adults-a-case-series-and-review-of-the-literature
#14
Carmine Romano, Francesco Pegoraro, Augusto Vaglio, Chiara Spezzani, Elena Sieni, Ilaria Fotzi, Matteo Lenge, Andrea Di Rita, Simone Peraio, Alice Noris, Carla Gaggiano, Salvatore Grosso, Flavio Giordano
INTRODUCTION: Erdheim-Chester disease (ECD) is a rare histiocytic neoplasm that affects patients, predominantly males aged 40-70 years, with very heterogeneous clinical presentation and prognosis. In 2020, Goyal et al. proposed consensus recommendations for the management of patients with ECD, remarking on the exceptional presentation of the disease in the pediatric population. CASE PRESENTATION: The first patient, a 20-year-old male, underwent cervical laminectomy and partial removal of a cervical spine lesion, initially apparently consistent with cervical schwannomas...
January 23, 2024: Pediatric Neurosurgery
https://read.qxmd.com/read/38249115/a-rare-case-report-of-erdheim-chester-disease-with-pericardial-effusion-conduction-abnormalities-and-atrial-infiltration
#15
Charaf Yassine, Giuseppe Colletti, Acasandrei Ciprian, Mairesse George
BACKGROUND: Erdheim-Chester disease (ECD) is a rare multisystem disorder that primarily affects adults. It is characterized by the excessive production and accumulation of histiocytes, a type of white blood cell, within multiple tissues and organs, including the cardiovascular system. The infiltration of histiocytes can cause a range of cardiovascular symptoms, including pericardial effusion, myocardial infiltration, and heart failure, among others. Despite the potential severity of these cardiovascular manifestations, ECD is often misdiagnosed or underdiagnosed, leading to delays in appropriate treatment and poor outcomes for patients...
January 2024: European Heart Journal. Case Reports
https://read.qxmd.com/read/38233339/a-rare-case-of-erdheim-chester-s-disease-presenting-as-atypical-breast-involvement
#16
Flavia Sprenger, Alana Gomes Pecorari, Valmir Vicente Filho, Sofia Tokars Kluppel, Ana Carolina Staats
Erdheim-Chester disease (ECD) is a rare multisystem disorder characterized by mitogen-activated protein kinase (MAPK) pathway mutations. Herein, we present a unique case of ECD in a 79-year-old female with predominant breast nodules. Comprehensive imaging and histopathological evaluations confirmed the diagnosis. Mammography and ultrasonography revealed multiple hyperdense circumscribed nodules with coalescing masses and blurred margins. Core biopsy revealed infiltrating foamy CD68+ and CD1a+ histiocytes. Because the tumor was negative for the BRAF V600E mutation, treatment with interferon-α was initiated...
January 18, 2024: Journal of Breast Cancer
https://read.qxmd.com/read/38231013/successful-treatment-of-cutaneous-onset-erdheim-chester-disease-with-cobimetinib-and-anakinra
#17
JOURNAL ARTICLE
Italo Francesco Aromolo, Chiara Moltrasio, Carlo Alberto Maronese, Corrado Campochiaro, Arturo Bonometti, Emilio Berti, Emanuela Passoni, Angelo Marzano
No abstract text is available yet for this article.
January 17, 2024: Clinical and Experimental Dermatology
https://read.qxmd.com/read/38222166/double-subclavian-steal-syndrome-as-initial-manifestation-of-erdheim-chester-disease-a-case-report
#18
Josef Finsterer
Erdheim-Chester disease (ECD) is a histiocytosis that infiltrates all organs, but especially large arteries such as the aorta and its branches, which become sheathed and lead to multiple stenoses or even occlusions. Subclavian steal syndrome (SSS) has not been reported in ECD. A 68-year-old female was diagnosed with ECD due to the typical sheathing of the aorta and its major branches. Five years previously, asymptomatic SSS due to stenosis of the left subclavian artery was incidentally diagnosed. In the following years, occlusion of the subclavian and left vertebral artery, and stenosis of basilar artery and right middle cerebral artery occurred...
December 2023: Curēus
https://read.qxmd.com/read/38180007/selected-case-from-the-arkadi-m-rywlin-international-pathology-slide-seminar-involvement-of-skin-and-soft-tissue-by-erdheim-chester-disease
#19
JOURNAL ARTICLE
David I Suster, Shira Ronen, Saul Suster
Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis that preferentially involves long bones but can affect a variety of other organs. Initial presentation with extraskeletal involvement is not unusual and is most commonly observed in the central nervous system, heart, retroperitoneum, lungs, and skin. Initial presentation of the disease as a subcutaneous soft tissue mass is exceedingly rare and may pose difficulties for diagnosis. We describe a case of Erdheim-Chester disease that initially presented as a cutaneous and subcutaneous soft tissue mass in the right posterior shoulder of a 52-year-old man...
January 5, 2024: Advances in Anatomic Pathology
https://read.qxmd.com/read/38172907/usefulness-of-18-f-fdg-pet-ct-for-assessing-large-vessel-involvement-in-patients-with-suspected-giant-cell-arteritis-and-negative-temporal-artery-biopsy
#20
JOURNAL ARTICLE
Javier Narváez, Paula Estrada, Paola Vidal-Montal, Iván Sánchez-Rodríguez, Aida Sabaté-Llobera, Joan Miquel Nolla, Montserrat Cortés-Romera
OBJECTIVE: To investigate the usefulness of 18 F-FDG PET-CT for assessing large-vessel (LV) involvement in patients with suspected giant cell arteritis (GCA) and a negative temporal artery biopsy (TAB). METHODS: A retrospective review of our hospital databases was conducted to identify patients with suspected GCA and negative TAB who underwent an 18 F-FDG PET-CT in an attempt to confirm the diagnosis. The gold standard for GCA diagnosis was clinical confirmation after a follow-up period of at least 12 months...
January 4, 2024: Arthritis Research & Therapy
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