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https://www.readbyqxmd.com/read/29317365/intraoperative-mri-guided-biopsy-in-the-diagnosis-of-suprasellar-langerhans-cell-histiocytosis
#1
Kate T Carroll, Bryson C Lochte, James Y Chen, Vivian S Snyder, Bob S Carter, Clark C Chen
Magnetic resonance imaging (MRI)-guided biopsy is an emerging diagnostic technique that holds great promise for otherwise difficult to access neuroanatomy. Here we describe MRI-guided biopsy of a suprasellar lesion located posterior and superior to the pituitary stalk. The approach was successfully implemented in a 38-year-old woman who had developed progressive visual deterioration. Intra-operative MRI revealed the need for trajectory adjustment due to an unintended, minor deviation in the burr hole entry point, demonstrating the benefit of an MRI-guided approach...
January 6, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29305568/novel-compound-heterozygous-variants-in-the-lhcgr-gene-identified-in-a-subject-with-leydig-cell-hypoplasia-type-1
#2
Yufei Xu, Yulin Chen, Niu Li, Xuyun Hu, Guoqiang Li, Yu Ding, Juan Li, Yiping Shen, Xiumin Wang, Jian Wang
BACKGROUND: Leydig cell hypoplasia (LCH) is a rare disease and one of the causes of male disorder of sexual differentiation (DSD). Inactivating mutations in the luteinizing hormone/chorionic gonadotropin receptor (LHCGR) gene account for the underlying LCH pathogenicity. This study aimed to analyze the clinical presentation and diagnosis as well as highlight the molecular characteristics of a subject with LCH type 1. CASE PRESENTATION: Clinical data were collected from the subject and analyzed...
January 6, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29296950/targeted-inhibition-of-the-mapk-pathway-emerging-salvage-option-for-progressive-life-threatening-multisystem-lch
#3
Alexandra Kolenová, Raphaela Schwentner, Gunhild Jug, Ingrid Simonitsch-Klupp, Christoph Kornauth, Lukáš Plank, Júlia Horáková, Ivana Bodová, Tomáš Sýkora, Lucia Geczová, Wolfgang Holter, Milen Minkov, Caroline Hutter
Single-agent vemurafenib leads to a rapid and sustained clinical response in severe multisystem LCH but does not eradicate the disease.Longitudinal assessment of BRAF V600E during treatment shows that clinical remission can occur despite significant amounts of mutated BRAF.
February 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29250688/pulmonary-langerhans-cell-histiocytosis-in-a-26-year-old-female-still-a-diagnostic-challenge
#4
Edith Simona Ianoşi, Mihaela Mureşan, Simona Gurzu, Eugenia Corina Budin, Ioan Jung, Tivadar Bara, Gabriela Jimborean
Langerhans cell histiocytosis (LCH) is a rare disorder caused by monoclonal Langerhans cells proliferation in bone, skin, lung, lymph nodes, liver, spleen, nervous or hematopoietic system. Pulmonary LCH is a diagnostic trap that is displayed on computed tomography (CT) as an interstitial disorder with honeycomb aspect. In this paper, we present an unusual case of a 26-year-old female that was hospitalized with progressive worsening dyspnea and history of recurrent pneumonia. Lung biopsy showed fibrosis of the interalveolar septa, architectural distortion and large cells with foamy cytoplasm and convoluted nuclei that were marked by CD68, S-100 and the specific antibody CD1a that allowed establishing the diagnosis of pulmonary LCH...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29221343/adult-onset-langerhans-cell-histiocytosis-of-the-sternum
#5
Yi-Jhih Huang, Kuan-Hsun Lin, Tai-Kuang Chao, Tsai-Wang Huang, Hsu-Kai Huang
Langerhans cell histiocytosis (LCH) is a rare disease with uncertain etiology that is more prevalent in children. LCH typically invades skeletal systems, but in rare cases, it has been reported in the ribs or sternum. Optimal treatment choices for single-site, skeletal LCH are still undefined. We report a case of adult-onset LCH of the sternum. The range of surrounding soft tissue invasion was confirmed by three-dimensional fusion and reconstruction of chest computed tomography and magnetic resonance images...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29209883/disseminated-juvenile-xanthogranuloma-occurring-after-treatment-of-langerhans-cell-histiocytosis-a-case-report
#6
Tae-Kyu Lee, Tae-Young Jung, Hee-Jo Baek, Seul-Kee Kim, Kyung-Hwa Lee, Sook Jung Yun
CASE PRESENTATION: An 11-year-old boy presented with a complaint of a painful temporal mass. Brain magnetic resonance imaging (MRI) showed a 3-cm-sized, homogeneously enhancing mass in the greater wing of the left sphenoid bone, which was diagnosed as Langerhans cell histiocytosis (LCH). Chemotherapy with vincristine and prednisolone was performed for 1 year. After 1 year and 11 months off treatment, he developed symptoms such as polydipsia and polyuria. Brain MRI showed thickening of the pituitary stalk with enhancement, suggestive of LCH involvement, and no recurrence in the sphenoid bone...
December 5, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29204304/langerhans-cell-histiocytosis-a-diagnostic-challenge-in-the-oral-cavity
#7
Mehmet Ali Altay, Alper Sindel, Öznur Özalp, Burak Kocabalkan, İrem Hicran Özbudak, Ramazan Erdem, Ozan Salim, Dale A Baur
Background: Langerhans cell histiocytosis (LCH) is a rare disorder of the reticuloendothelial system with unknown etiology. This report aims to present a case of LCH with diffuse involvement of the oral cavity and to raise awareness of the distinguishing features of this diagnostically challenging entity. Case Report: A 26-year-old male patient presented with complaints of teeth mobility, intense pain, and difficulty in chewing. Intraoral and radiological examinations revealed generalized gingival hyperplasia and severe teeth mobility with widespread alveolar bone loss...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/29198444/endocrine-and-metabolic-assessment-in-adults-with-langerhans-cell-histiocytosis
#8
L Montefusco, S Harari, D Elia, A Rossi, C Specchia, O Torre, G Adda, M Arosio
CONTEXT: Diabetes insipidus (DI) is one of most common complications of Langerhans cell histiocytosis (LCH) but prevalence of anterior pituitary deficiencies and metabolic alterations have not been clearly defined yet. OBJECTIVES: Evaluate prevalence of endocrine and metabolic manifestations in a cohort of patients affected by Pulmonary LCH. METHODS: Observational cross-sectional study on 18 adults (7 M/11 F, 42±12years) studied for complete basal and dynamic endocrine lab tests and glucose metabolism...
November 29, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/29194093/cdkn2a-b-deletion-and-double-hit-mutations-of-the-mapk-pathway-underlie-the-aggressive-behavior-of-langerhans-cell-tumors
#9
Luc Xerri, José Adélaïde, Cornel Popovici, Séverine Garnier, Arnaud Guille, Lenaïg Mescam-Mancini, Camille Laurent, Pierre Brousset, Carole Coze, Gérard Michel, Max Chaffanet, Reda Bouabdallah, Diane Coso, François Bertucci, Daniel Birnbaum
Langerhans cell histiocytosis (LCH) has a mostly favorable outcome, whereas Langerhans cell sarcoma (LCS) is an aggressive tumor. It is still unclear whether any specific molecular alterations could underlie the aggressive behavior of Langerhans cell proliferations. We used targeted next-generation sequencing and array-comparative genomic hybridization to profile 22 LCH samples from different patients together with 3 LCS samples corresponding to different relapses from the same patient. The third LCS relapse was a composite tumor including both B-cell chronic lymphocytic leukemia and LCS components...
November 29, 2017: American Journal of Surgical Pathology
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
#10
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/29164473/clinicopathological-and-immunohistochemical-study-of-head-and-neck-langerhans-cell-histiocytosis-from-latin-america
#11
Natália Rocha Bedran, Román Carlos, Bruno Augusto Benevenuto de Andrade, Ana Paula Silva Bueno, Mário José Romañach, Cristiane Bedran Milito
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplastic proliferation with variable clinical behavior caused by the accumulation of CD1a(+)/CD207(+) histiocytes, associated with a variable number of eosinophils, lymphocytes, plasma cells and multinucleated giant cells, most commonly observed in male children. LCH is uncommon in the head and neck region, occurring as ulcerated and reddened plaques or nodules that cause destruction of adjacent soft tissues and bone. The exact etiology of LCH is still unknown and controversial, with possible etiologic role of viruses, including Epstein-Barr virus (EBV)...
November 21, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/29157165/successful-cochlear-implantation-in-langerhans-cell-histiocytosis-a-rare-case
#12
Gaurav Gupta, Avani Jain, Mohnish Grover
An 8-year-old girl presented with a 1-year history of bilateral progressive hearing loss and vertigo for 6 months. High-resolution computed tomography of the temporal bones demonstrated multiple lytic lesions. Histopathology examination confirmed a diagnosis of Langerhans cell histiocytosis (LCH). She underwent chemotherapy for 12 months. Following treatment, she was in remission. However, the bilateral profound sensorineural hearing loss persisted. She underwent right cochlear implantation with very good functional audiological outcomes...
November 20, 2017: Cochlear Implants International
https://www.readbyqxmd.com/read/29125707/vascularized-humeral-periosteal-flap-to-treat-lateral-humeral-condyle-nonunion-an-anatomical-study-and-report-of-two-successfully-treated-pediatric-cases
#13
S Sergi Barrera-Ochoa, Francisco Soldado, Alfonso Rodriguez-Baeza, Paula Diaz-Gallardo, Felipe Hodgson, Pablo S Corona, Jorge Knörr
PURPOSE: Nonunion is a common complication of lateral condyle humeral (LCH) fractures in children. In situ fixation with a screw and bone grafting is the classically-recommended method of treatment. The purpose of this study is to analyze the feasibility of obtaining a vascularized periosteal flap obtained from the lateral humerus and based on the posterior collateral radial vessels (PCRV). Second, to report the results after the application in two pediatric cases. METHODS: Periosteal branches of PCRV were studied in ten upper limbs from fresh human cadavers...
November 10, 2017: Microsurgery
https://www.readbyqxmd.com/read/29118704/spontaneous-remission-of-severe-systemic-langerhans-cell-histiocytosis-with-bladder-involvement-a-case-study
#14
Isotta M Magaton, Alexandar Tzankov, Fatime Krasniqi, Christof Rottenburger, Rosanna Zanetti-Daellenbach, Peter Grendelmeier, Viola Heinzelmann-Schwarz, Michael Mayr, Fabienne D Schwab
Background: The clinical presentation of Langerhans cell histiocytosis (LCH) is heterogeneous ranging from single-organ involvement to systemic disease causing substantial morbidity and mortality. We describe an unusual course of severe multisystem LCH with spontaneous remission. Case Presentation: We report on a 45-year-old Caucasian woman with cervical cancer, FIGO stage IVB. Five months after the end of combined radiochemotherapy and brachytherapy, the patient was readmitted because of severe dysuria...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29107666/jak2-mutated-langerhans-cell-histiocytosis-associated-with-primary-myelofibrosis-treated-with-ruxolitinib
#15
Arturo Bonometti, Filippo Bagnoli, Daniele Fanoni, Luigia Venegoni, Laura Corti, Paola Bianchi, Elena Maria Elli, Giuseppe Isimbaldi, Vincenzo L'Imperio, Gianluca Nazzaro, Emanuela Passoni, Emilio Berti
The pathogenesis and cellular origin of Langerhans cell histiocytosis (LCH) are debated. Recently, mutations on MAPK and PI3K pathways have been linked to disrupted cell proliferation in LCH. Janus Kinase 2 (JAK2) mutations play the same role in Philadelphia-negative chronic myeloproliferative neoplasms. We describe the case of a patient affected by JAK2-positive Primary Myelofibrosis (PMF) who developed a clonally related LCH while in treatment with Ruxolitinib. JAK-inhibitors are well known to affect function and differentiation of different hematological lineages, including mononuclear phagocytes precursors...
October 28, 2017: Human Pathology
https://www.readbyqxmd.com/read/29107340/langerhans-cell-histiocytosis-a-neoplastic-disorder-driven-by-ras-erk-pathway-mutations
#16
REVIEW
Gary Tran, Thy N Huynh, Amy S Paller
Langerhans cell histiocytosis (LCH) is a disorder of myeloid neoplasia of dendritic cells that affects 1 in 200,000 children <15 years of age and even fewer adults. LCH presents with a spectrum of clinical manifestations. High-risk stratification is reserved for infiltration of blood, spleen, liver, and lungs. After decades of debate on the disease pathogenesis, a neoplastic mechanism is now favored on the basis of LCH cell clonality, rare cases of familial clustering, and recent evidence of mutations involving the Ras/Raf/MEK (mitogen-activated protein kinase kinase)/ERK (extracellular signal-regulated kinase) pathway in lesional biopsy specimens...
October 26, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29096034/cns-erdheim-chester-disease-a-challenge-to-diagnose
#17
REVIEW
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/29084059/insl3-expression-in-leydig-cell-hyperplasia-and-leydig-cell-tumors
#18
Nelli S Lakis, Kara A Lombardo, Shamlal Mangray, George J Netto, Daniela Salles, Andres Matoso
Insulin-like 3 (INSL3) is a hormone produced by Leydig cells (LCs) and leads to physiological testicular descent during embryonic development. We investigated the expression of INSL3 by immunohistochemistry in normal LCs, in Leydig cell tumor (LCT) (n=17 including 15 testes and 2 ovaries) and in Leydig cell hyperplasia (LCH) (n=10). Normally distributed LCs showed strong immunostaining in the cytoplasm in all cases. All 10 cases (100%) of LCH were strongly and diffusely positive in the intertubular areas. Six cases of LCH had nodules raging in size from 0...
October 27, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29074592/finding-active-lch-cells-in-the-blood
#19
Oussama Abla
No abstract text is available yet for this article.
October 26, 2017: Blood
https://www.readbyqxmd.com/read/29044633/extensive-nail-changes-in-a-toddler-with-multisystemic-langerhans-cell-histiocytosis
#20
Vimal Kumar, Dhanalakshmi Angappan, Julius Scott, Deenadayalan Munirathnam, Mukul Vij, Naresh Shanmugam
Langerhans cell histiocytosis (LCH) is a multisystem disorder involving various organs. Nail changes in LCH are extremely rare. We present this case report of extensive nail changes in an 18-month-old child with multisystem LCH.
October 17, 2017: Pediatric Dermatology
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