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https://www.readbyqxmd.com/read/28936088/symptomatic-bilateral-xanthogranuloma-of-the-choroid-plexus
#1
Selin Tural Emon, Erek Ozturk, Kaan Meric, Fugen Aker, Metin Orakdogen
Xanthogranulomas (XGRs) of the choroid plexus are rare, asymptomatic, and benign lesions usually found incidentally. Here, we present a case of a 47-year-old male with bilateral XGR of the choroid plexus with periventricular edema and discuss our case in relation to a review of existing literature pertaining to the radiology of XGRs. To the best of our knowledge, this is the first reported case of bilateral trigonal XGR causing brain edema without ventricular dilatation. Despite the fact that they can cause hydrocephalus, XGRs are silent and benign lesions...
August 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28920477/if-it-looks-like-a-xanthogranuloma-and-walks-like-a-xanthogranuloma
#2
Paul B Medline
No abstract text is available yet for this article.
September 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28920472/it-may-look-similar-to-a-xanthogranuloma-under-the-microscope-but-it-does-not-really-walk-like-one-clinically
#3
Sate Hamza, Alexandra Kuzyk, Shane Silver, Sharon Wallace, John Toole
No abstract text is available yet for this article.
September 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28875110/monomorphic-xanthomatous-type-of-xanthogranuloma-a-rare-entity
#4
Pratheeba Devi Nivean, M Nivean
Xanthogranuloma is an uncommon tumor in the ocular adnexa. It usually presents as diffuse lid swelling with or without orbital involvement. Xanthogranulomas can be associated with systemic comorbidities. They are diagnosed by their characteristic pathological finding. They are further subclassified based on immunohistochemistry. We present two cases with lid swelling which were diagnosed to be xanthogranuloma. Our cases were special because of the unique presentation as localized monomorphic lesions.
2017: GMS Ophthalmology Cases
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#5
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28863117/rituximab-monotherapy-is-effective-in-treating-orbital-necrobiotic-xanthogranuloma
#6
Oded Sagiv, Sudip D Thakar, Gail Morrell, Michael T Tetzlaff, Bita Esmaeli
The authors report a case of a patient with bilateral orbital necrobiotic xanthogranuloma and no associated systemic paraproteinemia. Orbital biopsy showed strong expression of CD20-positive cells. The patient was treated with systemic rituximab monotherapy, with excellent clinical response and marked regression of the orbital lesions on imaging. At the time of writing, the patient has been treated with bimonthly rituximab maintenance therapy for 22 months and has stable clinical and imaging findings with sustained response to treatment and no reported side effects...
August 23, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28836893/systemic-juvenile-xanthogranuloma-involving-the-bone-marrow-multiple-bones-and-the-skin-that-developed-during-treatment-of-acute-lymphoblastic-leukemia-in-remission-state
#7
Eunjae Cheon, Saemi Yang, Jae Ho Han, Kwang Chul Lee, Jun Eun Park
Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement. We encountered a systemic JXG case involving the bone marrow, multiple bones, and the skin during acute lymphoblastic leukemia treatment...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28816552/the-role-of-biopsy-in-lacrimal-gland-inflammation-a-clinicopathologic-study
#8
Panitee Luemsamran, Jack Rootman, Valerie A White, Nariman Nassiri, Manraj K S Heran
PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone...
August 17, 2017: Orbit
https://www.readbyqxmd.com/read/28802503/fibrohistiocytic-tumors
#9
REVIEW
Ryan C Romano, Karen J Fritchie
Fibrohistiocytic tumors are a diverse group of reactive and neoplastic lesions including xanthoma, fibrous histiocytoma and its variants, solitary xanthogranuloma, dermatofibrosarcoma protuberans, and atypical fibroxanthoma. This article reviews some of the more commonly encountered fibrohistiocytic tumors with an emphasis on clinical presentation, macroscopic and histologic characteristics, molecular/cytogenetic findings where applicable, and differential diagnoses.
September 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/28752840/genetic-evaluation-of-juvenile-xanthogranuloma-genomic-abnormalities-are-uncommon-in-solitary-lesions-advanced-cases-may-show-more-complexity
#10
Christian N Paxton, Dennis P O'Malley, Andrew M Bellizzi, Deema Alkapalan, Yuri Fedoriw, Jason L Hornick, Sherrie L Perkins, Sarah T South, Erica F Andersen
Juvenile xanthogranuloma is a rare histiocytic proliferation primarily affecting infants and young children, characterized by aberrant infiltration of histiocyte-derived cells in the skin, soft tissues and more rarely, visceral organs. Juvenile xanthogranuloma is generally considered to be a benign disorder; most lesions are solitary cutaneous nodules that resolve spontaneously without treatment. However, cases with extracutaneous involvement, multiple lesions, and/or systemic disease often require aggressive therapy...
September 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28739622/an-unusual-case-of-interstitial-lung-disease-in-a-patient-with-cardiopulmonary-syndrome-as-the-initial-presentation-of-erdheim-chester-disease
#11
Domingo Franco-Palacios, April McDonald, R Neal Aguillard, Allen Berry
Erdheim-Chester disease (ECD) is a very rare disorder with only approximately 600 cases reported in the literature. ECD has been recently reclassified as a histiocytic dendritic cell neoplasm. The clinical spectrum ranges from asymptomatic tissue accumulation of histiocytes to invasive tissue infiltration, which can cause fulminant multisystem failure. It typically presents with bone pain and constitutional symptoms. Extraosseous manifestations are not uncommon. ECD-associated interstitial lung disease has been described in 20%-35% of patients...
July 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28671710/adult-xanthogranuloma-diagnosed-on-reflectance-confocal-microscopy
#12
LETTER
Attiya Haroon, Shahram Shafi, Babar Rao
No abstract text is available yet for this article.
September 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28626159/limbal-xanthogranuloma-in-a-dog
#13
Juri Ota-Kuroki, Keiichi Kuroki
A 10-year-old female spayed American Pitt Bull Terrier was presented with a slow growing mass on the temporal limbus area of the right eye. Canine nodular granulomatous episclerokeratitis was suspected, and the affected eye was treated with 1% prednisolone acetate ophthalmic suspension and 0.03% Tacrolimus ophthalmic solution. As the lesion did not respond to the medical treatments and continued to grow, the mass was excised by lamellar sclerokeratectomy. Microscopically, the mass was composed of granulomatous inflammation with intrahistiocytic lipids and lakes of acicular cholesterol clefts, histopathology findings consistent with xanthogranuloma...
July 19, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28583250/oral-juvenile-xanthogranuloma-report-of-two-cases
#14
Mônica Simões Israel, Roman Carlos, Fábio Ramôa Pires
Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs in the skin of children. Extracutaneous involvement is rare, and few cases affecting the oral cavity have been reported. The purpose of the present study was to report two cases of oral juvenile xanthogranuloma affecting the lower lip of a 14-year-old girl and the soft palate of a second 14-month-old girl, both present as yellowish papules. The lesions were surgically excised, and histological and immunohistochemical analysis showed a proliferation of non-Langerhans cells histiocytes and foamy cells, fulfilling the morphologic features of juvenile xanthogranuloma...
May 15, 2017: Pediatric Dentistry
https://www.readbyqxmd.com/read/28567509/-clinical-and-histological-spectrum-of-palisaded-granulomatous-dermatitides-granuloma-annulare-necrobiosis-lipoidica-rheumatoid-nodules-and-necrobiotic-xanthogranuloma
#15
REVIEW
B Weidenthaler-Barth
The palisading granulomatous dermatitides comprise a group of different skin diseases with similar histomorphologic forms of granuloma in the skin. Histopathologically characteristic are areas in the reticular dermis and subcutaneous fat with degenerated bundles of collagen surrounded by histiocytes and multinucleate giant cells aligned in a palisade (necrobiotic granuloma). Within the center of palisaded granulomas, mucin or fibrin can be found. The skin diseases presenting histologically with palisading granuloma are granuloma annulare and necrobiosis lipoidica...
July 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28551613/erdheim-chester-disease-comprehensive-review-of-molecular-profiling-and-therapeutic-advances
#16
REVIEW
Faysal Haroun, Kristen Millado, Imad Tabbara
The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features. However, evolving knowledge regarding the molecular and genetic aberrations in addition to common clinical features of ECD support the classification of ECD together with Langerhans cell histiocytosis (LCH). Accordingly, ECD can be thought of as an inflammatory myeloid clonal disorder based on the detection of various activating mutations along the mitogen activated protein kinase-extracellular signal regulated kinase (MAPK-ERK) pathway with most notable variant being a valine to a glutamic acid substitution at amino acid 600 in the B-rapidly accelerated fibrosarcoma protein (BRAFV600E)...
June 2017: Anticancer Research
https://www.readbyqxmd.com/read/28522113/neonatal-glaucoma-associated-with-juvenile-xanthogranuloma-case-report
#17
A Ramos Suárez, I Ayet Roger, A Serra Castanera
CASE REPORT: The case concerns a 22 day-old male child with juvenile xanthogranuloma (JXG), which manifested as a unilateral glaucoma and with fibrinous haemorrhagic exudate in the anterior chamber affecting the angle of the right eye. Despite a high level of suspicion, the definitive diagnosis was not possible until the infant reached the age of 10 months, after the appearance of the skin lesions typical of this condition and histopathological study of them. CONCLUSION: JXG is a rare disease, characterised by yellowish skin lesions on the trunk, neck, or head...
May 15, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28512266/activating-mapk1-erk2-mutation-in-an-aggressive-case-of-disseminated-juvenile-xanthogranuloma
#18
Rikhia Chakraborty, Oliver A Hampton, Harshal Abhyankar, Daniel J Zinn, Amanda Grimes, Brooks Skull, Olive Eckstein, Nadia Mahmood, David A Wheeler, Dolores Lopez-Terrada, Tricia L Peters, John M Hicks, Tarek Elghetany, Robert Krance, Poulikos I Poulikakos, Miriam Merad, Kenneth L McClain, Carl E Allen, Donald W Parsons
Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder that is usually benign and self-limiting. We present a case of atypical, aggressive JXG harboring a novel mitogen-activated protein kinase (MAPK) pathway mutation in the MAPK1 gene, which encodes mitogen-activated protein kinase 1 or extracellular signal-regulated 2 (ERK2). Our analysis revealed that the mutation results in constitutive ERK activation that is resistant to BRAF or MEK inhibitors but susceptible to an ERK inhibitor. These data highlight the importance of identifying specific MAPK pathway alterations as part of the diagnostic workup for patients with histiocytic disorders rather than initiating empiric treatment with MEK inhibitors...
July 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28504206/braf-v600e-mutation-in-pediatric-intracranial-and-cranial-juvenile-xanthogranuloma
#19
Piti Techavichit, Darintr Sosothikul, Thiamjit Chaichana, Chinachote Teerapakpinyo, Paul Scott Thorner, Shanop Shuangshoti
Juvenile xanthogranuloma (JXG) is a cutaneous form of non-Langerhans cell histiocytosis (LCH), primarily affecting children. The lesion is presumed to originate from either macrophages or dermal dendritic cells. JXG can rarely present as an isolated intracranial lesion and, in contrast to the dismal outcome of patients with systemic disease, cranial JXG has been shown to carry a more favorable prognosis. Here, we report for the first time 3 pediatric cases of JXG with a BRAF V600E mutation, 2 with intracranial lesions and one with cranial lesions...
May 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28500693/the-clinical-spectrum-of-xanthomatous-lesions-of-the-eyelids
#20
REVIEW
Can Baykal, Algun Polat Ekinci, Kurtulus D Yazganoglu, Nesimi Buyukbabani
Yellowish papules, nodules, or plaques, namely "xanthomatous" lesions, may be seen on the eyelids in the course of various disorders. The prototype is "xanthelasma palpebrarum" (XP) that is localized only to the eyelids and may be associated with hyperlipidemia. On the other hand, different types of normolipemic disorders may also cause xanthomatous eyelid lesions. Among these, Langerhans cell histiocytosis, diffuse normolipemic xanthoma, and non-Langerhans cell histiocytoses (papular xanthoma, juvenile xanthogranuloma, xanthoma disseminatum, adult-onset xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, and reticulohistiocytosis) can be listed...
May 13, 2017: International Journal of Dermatology
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