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    Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review.
    Brain Dev 2018 Jun 12. Epub 2018 Jun 12.
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
    Background: Langerhans cell histiocytosis (LCH) is a clonal disease with focal or disseminated lesions that may compress the surrounding tissues, including the spinal cord. Because few reports have described the spinal symptoms as the first manifestation of pediatric LCH, the long-term neurological outcomes remain unclear.

    Case Report And Literature Review: We report a 21-month-old boy who presented with sudden-onset paraplegia. Read More
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    Langerhans cell histiocytosis of bone in an adult: A case report.
    Radiol Case Rep 2018 Apr 28;13(2):310-314. Epub 2018 Jan 28.
    Moffitt Cancer Center, 12902 Magnolia Drive, Tampa, FL 33612, USA.
    Langerhans cell histiocytosis (LCH) may clinically manifest in a variety of ways due to its ability to involve nearly every organ system. LCH may present as a single bone lesion, skin rash, or as invasive disseminated disease and occurs typically in the pediatric and adolescent population, affecting both males and females. Independent of its clinical presentation and severity, LCH lesions share the common histology of CD1a/CD207 dendritic cells along with an inflammatory infiltrate, and, based upon improved scientific understanding, is now classified as a myeloproliferative neoplasm. Read More
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    Childhood cancer registration in New Zealand: A registry collaboration to asssess and improve data quality.
    Cancer Epidemiol 2018 Jun 11;55:104-109. Epub 2018 Jun 11.
    Starship Blood and Cancer Centre, Starship Children's Hospital, Auckland, New Zealand.
    Aim: To evaluate the completeness and accuracy of child cancer registration in New Zealand.

    Methods: Registrations for children aged 0-14 diagnosed between 1/1/2010 and 31/12/2014 were obtained from the New Zealand Cancer Registry (NZCR) and the New Zealand Children's Cancer Registry (NZCCR). Six key data fields were matched using National Health Index numbers in order to identify and resolve registration discrepancies. Read More
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    [Thickening of the pituitary stalk in children and adolescents with central diabetes insipidus: Causes and consequences].
    An Pediatr (Barc) 2018 Jun 9. Epub 2018 Jun 9.
    Servicio de Pediatría y Endocrinología, Hospital Infantil Universitario Niño Jesús, Madrid, España; Departamento de Pediatría, Universidad Autónoma de Madrid, Madrid, España; CIBER Fisiopatología de la Obesidad y Nutrición, Instituto de Salud Carlos III, Madrid, España.; IMDEA Instituto de Alimentación, CEI UAM + CSIC, Madrid, España.
    Background: Central diabetes insipidus (CDI) is a rare disorder in children. The aetiology of CDI in childhood is heterogeneous. The aim of this study is to illustrate the importance of a careful clinical and neuro-radiological follow-up of the pituitary and hypothalamus region in order to identify the aetiology and the development of associated hormonal deficiencies. Read More
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    Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review.
    J Neurol Surg A Cent Eur Neurosurg 2018 Jun 11. Epub 2018 Jun 11.
    Department of Neurosurgery, Inselspital, Bern University Hospital, Bern, Switzerland.
    A 36-year-old man was diagnosed with Langerhans cell histiocytosis (LCH) of the cervical spine with a unifocal expansive osteolytic lesion of C4. The surgical management with a 2-year follow-up and a review of the literature on LCH of the cervical spine are presented. Although a rare condition, LCH is an important differential diagnosis of any osteolytic lesion in the cervical spine with localized pain in a young adult patient. Read More
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    Immune Thrombocytopenia in a Child With Refractory Langerhans Cell Histiocytosis Following Cladribine Containing Therapy.
    J Pediatr Hematol Oncol 2018 Jun 8. Epub 2018 Jun 8.
    Children's Haematology and Oncology Centre, Christchurch Hospital, Canterbury District Health Board, Christchurch, New Zealand.
    In this report, we present a young infant with multisystem Langerhans cell histiocytosis, who after cladribine and cytarabine salvage treatment developed immune thrombocytopenia (IT). On review of the literature, there were no previous reports of Langerhans cell histiocytosis-associated IT. Treatment of the IT with intravenous immunoglobulin and oral corticosteroids was unsuccessful. Read More
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    A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge.
    Case Rep Hematol 2018 16;2018:7865325. Epub 2018 May 16.
    Division of Blood and Marrow Transplant, University of Louisville, Louisville, KY, USA.
    Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocyte disorder most commonly characterized by multifocal osteosclerotic lesions of the long bones demonstrating sheets of foamy histiocyte infiltrates on biopsy with or without histiocytic infiltration of extraskeletal tissues. ECD can be difficult to diagnose since it is a very rare disease that can affect many organ systems. Diagnosis is based on the pathologic evaluation of involved tissue interpreted within the clinical context. Read More
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    Oral manifestation of Langerhans cell histiocytosis: a case report.
    BMC Oral Health 2018 Jun 8;18(1):106. Epub 2018 Jun 8.
    Clinic of Cranio-Maxillofacial and Oral Surgery, University of Zurich, University Hospital Zurich, Plattenstrasse 11, CH-8032, Zurich, Switzerland.
    Background: Bone necrosis of the jaw is a serious condition with a broad differential diagnosis of pathologies such as cutaneous histiocytosis, bone metastases or malignant tumours. In addition to the most common cause, medication related osteonecrosis of the jaw (MRONJ), one must consider a number of other causes, such as histiocytosis. Langerhans cell histiocytosis (LCH) is a histiocytic disorder with a large spectrum of clinical manifestations and with possible involvement of a variety of organs. Read More
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    Indeterminate Dendritic Cell Tumor: A Report of 2 New Cases Lacking the ETV3-NCOA2 Translocation and a Literature Review.
    Am J Dermatopathol 2018 May 31. Epub 2018 May 31.
    Department of Pathology, University of Virginia Medical Center.
    Indeterminate dendritic cell tumor (IDCT) is a cutaneous proliferation of histiocytes that share morphologic and immunophenotypic properties with Langerhans cells. IDCT was recently included in the updated WHO classification of tumors of hematopoietic and lymphoid tissues. Recent studies have shown that some cases of IDCT demonstrate an ETV3-NCOA2 translocation, supporting the idea that IDCT is a clonal neoplasm. Read More
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    Rare childhood hybrid histiocytosis of the central nervous system-diagnosed by stereotactic brain biopsy with marked treatment response to clofarabine.
    Childs Nerv Syst 2018 Jun 5. Epub 2018 Jun 5.
    Department of Neurosurgery, Rigshospitalet University Hospital, Blegdamsvej 9, 2100, Copenhagen, Denmark.
    Histiocytosis is a heterogeneous group of disease entities, comprised by two main categories, namely Langerhans and non-Langerhans cell histiocytoses. Central nervous system involvement in histiocytosis is considered very rare and is often secondary to affection of anatomically related bone structures and/or multi-organ disease. We present a never-before described case of rare childhood histiocytosis with hybrid features of Langerhans cell histiocytosis and juvenile xanthogranuloma confined to the central nervous system in a 2- and a half-year-old boy with distinct treatment response to clofarabine. Read More
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    Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review.
    Hormones (Athens) 2018 Mar 16;17(1):119-125. Epub 2018 Apr 16.
    Medical Faculty, University of Nis, Nis, Serbia.
    Introduction: Langerhans cell histiocytosis (LCH) localised in the hypothalamic-pituitary region (HPR) is very rare, especially in adults. Diabetes insipidus (DI) is considered to be a hallmark of HPR LCH, while anterior pituitary abnormalities are usually seen as consequences of surgery, radiotherapy or chemotherapy.

    Case Description: We present a patient with localised HPR LCH with dominant anterior pituitary dysfunction and tumour mass effects but without DI. Read More
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    Head and Neck Sinus Histiocytosis with Massive Lymphadenopathy Radiology-Pathology Correlation.
    Head Neck Pathol 2018 May 31. Epub 2018 May 31.
    Department of Radiology, University of Chicago, 5841 S Maryland Avenue, Chicago, IL, 60637, USA.
    Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare, benign type of non-Langerhans cell histiocytosis. The radiological findings are often nonspecific, potentially mimicking malignancies. The diagnosis is ultimately made based on pathology, in which the lymph nodes are characterized by a dilated subcapsular sinus filled with histiocytes that can exhibit emperipolesis. Read More
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    Immunohistochemical Biomarkers in Diagnosis of Hematolymphoid Neoplasms of Endocrine Organs.
    Endocr Pathol 2018 Jun;29(2):176-188
    Hematopathology Service, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY, 10065, USA.
    The hematolymphoid infiltrations are challenging lesions in endocrine organs and tissues. The fourth edition of WHO classification of tumors of endocrine organs and the fourth edition of WHO classification of tumors of hematopoietic and lymphoid tissues are recently published. The updates in both fields include some new disease descriptions and prognostic markers. Read More
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    Erdheim-Chester Disease Presenting as an Anterior Mediastinal Tumor without Skeletal Involvement.
    Korean J Thorac Cardiovasc Surg 2018 Jun 5;51(3):223-226. Epub 2018 Jun 5.
    Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine.
    Erdheim-Chester disease (ECD) is a form of non-Langerhans cell histiocytosis that most commonly involves the skeletal system. We report an unusual case of ECD presenting as an anterior mediastinal tumor without skeletal involvement. A 60-year-old man with no remarkable medical history was referred for evaluation of a mediastinal mass. Read More
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    Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature.
    Case Rep Radiol 2018 5;2018:2952084. Epub 2018 Apr 5.
    Department of Medical Imaging, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada.
    Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment. Read More
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    Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature.
    Case Rep Ophthalmol Med 2018 4;2018:1529281. Epub 2018 Apr 4.
    Department of Ophthalmology, VMMC & Safdarjung Hospital, Ring Road, Ansari Nagar, New Delhi 110029, India.
    A 2-year-old male child presented with a painless progressive mass in the inferolateral aspect of right orbit of three-month duration. Differential leucocyte count revealed raised eosinophil count (13%). On radiological examination, CT scan showed 25 × 27 mm round well-defined smooth-outlined homogenously enhancing extraconal mass arising from the zygomatic bone at the inferotemporal periorbital area of right orbit with bone erosion. Read More
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    Cutaneous squamous cell carcinoma, thyroid cancer and Langerhans cell histiocytosis in a patient with X-linked recessive Mendelian susceptibility to mycobacterial diseases with a nuclear factor-κB essential modifier mutation.
    J Dermatol 2018 May 24. Epub 2018 May 24.
    Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan.
    Nuclear factor (NF)-κB essential modifier (NEMO), also known as IκB kinase subunit-γ (IKKγ), is a pivotal molecule in the NF-κB signaling pathway. Mutations of NEMO cause incontinentia pigmenti and X-linked ectodermal dysplasia with immunodeficiency. Mendelian susceptibility to mycobacterial diseases (MSMD), which confers an almost selective predisposition to mycobacterial infection, is also caused by NEMO mutations. Read More
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    Langerhans cell histiocytosis at L5 vertebra treated with en bloc vertebral resection: a case report.
    World J Surg Oncol 2018 May 22;16(1):96. Epub 2018 May 22.
    Spine Lab, Department of Orthopedic Surgery, The First Affiliated Hospital, School of Medicine, Zhejiang University, The second floor of Building 3, 79# Qingchun Road, Hangzhou, 310003, China.
    Background: Langerhans cell histiocytosis (LCH) in adult lumbar spine is extremely rare, and optimal treatments remain unclear. In literature, only a few cases of lumbar spine LCH were treated using surgery but en bloc vertebral resection has not been used.

    Case Presentation: A 50-year-old man presented with unbearable radiating pain at his right leg. Read More
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    Vemurafenib in Langerhans cell histiocytosis: report of a pediatric patient and review of the literature.
    Oncotarget 2018 Apr 24;9(31):22236-22240. Epub 2018 Apr 24.
    Division of Pediatric Hematology and Oncology, Hospital for Children and Adolescents, Johann Wolfgang Goethe-University, Frankfurt, Germany.
    Selective BRAF inhibitors such as vemurafenib have become a treatment option in patients with Langerhans cell Histiocytosis (LCH). To date, only 14 patients receiving vemurafenib for LCH have been reported. Although vemurafenib can stabilize the clinical condition of these patients, it does not seem to cure the patients, and it is unknown, when and how to stop vemurafenib treatment. Read More
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    Clinical resistance associated with a novel MAP2K1 mutation in a patient with Langerhans cell histiocytosis.
    Pediatr Blood Cancer 2018 May 16:e27237. Epub 2018 May 16.
    Institute of Molecular Medicine, Phoenix Children's Hospital, Phoenix, Arizona.
    Patients with Langerhans cell histiocytosis (LCH) harbor BRAF V600E and activating mutations of MAP2K1/MEK1 in 50% and 25% of cases, respectively. We evaluated a patient with treatment-refractory LCH for mutations in the RAS-RAF-MEK-ERK pathway and identified a novel mutation in the MAP2K1 gene resulting in a p.L98_K104 > Q deletion and predicted to be auto-activating. Read More
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    Juvenile Xanthogranuloma in a Pediatric Patient with Langerhans Cell Histiocytosis.
    Ocul Oncol Pathol 2018 Apr 4;4(3):141-144. Epub 2017 Oct 4.
    Department of Ophthalmology, Baylor College of Medicine, Houston, TX, USA.
    Purpose: To report a case of juvenile xanthogranuloma that simulated a chalazion and to discuss the association between juvenile xanthogranuloma and Langerhans cell histiocytosis.

    Method: Case report and review of literature.

    Results: A 13-year-old boy with a prior history of Langerhans cell histiocytosis was referred to our clinic for a possible chalazion. Read More
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    Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis.
    Saudi J Ophthalmol 2018 Jan-Mar;32(1):52-55. Epub 2018 Feb 16.
    Department of Ophthalmology, The Ottawa Hospital and University of Ottawa, Ottawa, Ontario, Canada.
    Langerhans cell histiocytosis (LCH) is a rare multi-system disease. It presents infrequently as a childhood orbital tumor, and can mimic more common inflammatory orbital disease processes. We report the clinical, histopathological, and electron microscopic findings of orbital LCH in an 18-month-old child, along with a review of the recent literature regarding molecular pathogenetic analysis of LCH. Read More
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    Histiocytic lesions of the orbit: A study of 9 cases.
    Saudi J Ophthalmol 2018 Jan-Mar;32(1):40-44. Epub 2018 Mar 15.
    Department of Ophthalmology, Ankara University Faculty of Medicine, Ankara, Turkey.
    Purpose: To describe the clinical presentation, treatment, and outcome of patients with histiocytic lesions of the orbit.

    Methods: Retrospective study of 9 patients treated and followed up between October 2001 and January 2018.

    Results: Eight patients in our series were males and one patient was female. Read More
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    Langerhans cell histiocytosis in children: Diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up.
    J Am Acad Dermatol 2018 Jun;78(6):1047-1056
    ClearlyDerm Center for Dermatology, Department of Clinical Biomedical Science, Florida Atlantic University, Boca Raton, Florida.
    A definitive diagnosis of Langerhans cell histiocytosis (LCH) requires a combination of clinical presentation, histology, and immunohistochemistry. The inflammatory infiltrate contains various proportions of LCH cells, the disease hallmark, which are round and have characteristic "coffee-bean" cleaved nuclei and eosinophilic cytoplasm. Positive immunohistochemistry staining for CD1a and CD207 (langerin) are required for a definitive diagnosis. Read More
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    Langerhans cell histiocytosis in children: History, classification, pathobiology, clinical manifestations, and prognosis.
    J Am Acad Dermatol 2018 Jun;78(6):1035-1044
    ClearlyDerm Center for Dermatology, Department of Clinical Biomedical Science, Florida Atlantic University, Boca Raton, Florida.
    Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway. When disease involves the skin, LCH most commonly presents as a seborrheic dermatitis or eczematous eruption on the scalp and trunk. Evaluation for involvement of other organ systems is essential, because 9 of 10 patients presenting with cutaneous disease also have multisystem involvement. Read More
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    Expression of the transcription factor ZBTB46 distinguishes human histiocytic disorders of classical dendritic cell origin.
    Mod Pathol 2018 May 9. Epub 2018 May 9.
    Department of Pathology, Stanford University School of Medicine, Stanford, CA, 94305, USA.
    Distinguishing classical dendritic cells from other myeloid cell types is complicated by the shared expression of cell surface markers. ZBTB46 is a zinc finger and BTB domain-containing transcription factor, which is expressed by dendritic cells and committed dendritic cell precursors, but not by plasmacytoid dendritic cells, monocytes, macrophages, or other immune cell populations. In this study, we demonstrate that expression of ZBTB46 identifies human dendritic cell neoplasms. Read More
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    Langerhans cell histiocytosis presenting as Crohn's disease: a case report.
    Int J Colorectal Dis 2018 May 8. Epub 2018 May 8.
    Department of Pathology, Centre Hospitalier de l'Universite de Montreal, 1100 rue Sanguinet, Pavillon F, Montreal, QC, H2X 0C2, Canada.
    Purpose: We describe an exceptional case of Langerhans cell histiocytosis (LCH) that presented as Crohn's disease and primary sclerosing cholangitis.

    Methods: The patient's clinical, endoscopic, and histologic data from the Centre Hospitalier de l'Universite de Montreal were reviewed, as well as the literature on LCH involving the digestive tract and the liver, with a focus on the similarities with Crohn's disease and primary sclerosing cholangitis.

    Results: A 39 years-old man first presented with anal fissures and deep punctiform colonic ulcers. Read More
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    F FDG-PET/CT for the Evaluation of Primary Eosinophilic Granuloma of the Hypothalamus.
    J Nucl Med Technol 2018 May 3. Epub 2018 May 3.
    University of Arizona, United States.
    A 21-year-old man presented with polyuria and polydipsia and was discovered to have diabetes insipidus due to eosinophilic granuloma of the hypothalamus. F FDG-PET/CT for metastatic work-up revealed an intensely FDG avid hypothalamic mass and no other sites of disease. Read More
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    The role of parental and perinatal characteristics on Langerhans cell histiocytosis: characterizing increased risk among Hispanics.
    Ann Epidemiol 2018 Apr 17. Epub 2018 Apr 17.
    Department of Pediatrics, Section of Hematology-Oncology, Baylor College of Medicine, Houston, TX; Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Houston, TX. Electronic address:
    Purpose: Potential roles of inherited and environmental risk factors in pathogenesis of Langerhans cell histiocytosis (LCH), a myeloid neoplastic disorder, are undefined. We therefore evaluated the role of parental and perinatal factors on the risk of this childhood cancer.

    Methods: Information on LCH cases (n = 162) for the period 1995-2011 was obtained from the Texas Cancer Registry. Read More
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    Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease.
    Blood 2018 May 2. Epub 2018 May 2.
    Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY, United States.
    Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by accumulation of activated histiocytes within affected tissues. RDD, which now belongs to the R group of the 2016 revised histiocytoses classification, is a widely heterogeneous entity with a range of clinical phenotypes occurring in isolation or in association with autoimmune or malignant diseases. Recent studies have found NRAS, KRAS, MAP2K1 and ARAF mutations in lesional tissues, raising the possibility of a clonal origin in some forms of RDD. Read More
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    Insulin-like growth factor-1 receptor expression in pediatric tumors: a comparative immunohistochemical study
    Turk J Med Sci 2018 Apr 30;48(2):419-423. Epub 2018 Apr 30.
    Background/aim: Insulin-like growth factor-1 receptor (IGF-1R) is a pivotal receptor tyrosine kinase involved in the cell cycle and malignant tumor transformation. It is differentially expressed in various types of tumors. We aimed to determine the expression of IGF- 1R in different pediatric tumors and to shed light on possible new indications of anti-IGF-1R treatment approaches. Read More
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    Langerhans Cell Histiocytosis of the Gastrointestinal Tract - A Rare Entity.
    Cureus 2018 Feb 26;10(2):e2227. Epub 2018 Feb 26.
    Division of Gastroenterology and Hepatology, Academic Affiliate of the Icahn School of Medicine, Clinical Affiliate of the Mount Sinai Hospital.
    Langerhans cell histiocytosis (LCH) is an idiopathic and rare disease that ranges in clinical severity based on location and organ involvement. LCH most commonly affects the skin and bones. The involvement of the gastrointestinal tract (GI) in adults is exceedingly rare and only 10 cases have been reported in the literature. Read More
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    MAPK/ERK signal pathway alterations in patients with Langerhans Cell Histiocytosis.
    Klin Onkol 2018 ;31(2):130-136
    Background: Clinical outcomes of Langerhans cell histiocytosis (LCH) are highly variable. It has been suggested that mitogen-activated protein kinase (MAPK) /extracellular signal-regulated kinases (ERK) signaling pathway might be activated in LCH patients.

    Materials And Methods: We investigated KRAS, BRAF and NRAS mutations in patients with LCH by qPCR. Read More
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    The application of x-ray, computed tomography, and magnetic resonance imaging on 22 pediatric Langerhans cell histiocytosis patients with long bone involvement: A retrospective analysis.
    Medicine (Baltimore) 2018 Apr;97(17):e0411
    Department of Hematology, Children's Hospital of Nanjing Medical University, Nanjing, China.
    The studies focusing on x-ray, computed tomography (CT), and magnetic resonance imaging (MRI) in pediatric Langerhans cell histiocytosis (LCH) patients were still rare. Therefore, we aimed to evaluate the application of x-ray, CT, and MRI in pediatric LCH patients with long bone involvement.Total 22 pediatric LCH patients were included in this study. Read More
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    Response to Trametinib of a Pulmonary Langerhans Cell Histiocytosis Harboring a MAP2K1 Deletion.
    Am J Respir Crit Care Med 2018 Apr 25. Epub 2018 Apr 25.
    Assistance Publique-Hopitaux de Paris, Hôpital Saint Louis, Centre National de Référence des Histiocytoses, Paris, Please Select, France.
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    Cutaneous Langerhans cell histiocytosis presenting with hypopigmented lesions: Report of two cases and review of literature.
    Pediatr Dermatol 2018 Apr 25. Epub 2018 Apr 25.
    Department of Dermatology, State University of New York Downstate Medical Center, Brooklyn, NY, USA.
    Langerhans cell histiocytosis is a rare group of disorders that results from the abnormal proliferation and accumulation of dendritic-derived cells in various organs of the body, such as the skin and bones. Hypopigmented macules are a rare cutaneous presentation of Langerhans cell histiocytosis that may pose a diagnostic dilemma when no other findings of Langerhans cell histiocytosis are present at the time of examination. We present 2 cases of the hypopigmented variant of Langerhans cell histiocytosis, including a case with histopathologic features of regression, and a review of the literature. Read More
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    Nodular macroregenerative tissue as a pattern of regeneration in cholangiopathic disorders.
    Pediatr Radiol 2018 Jul 19;48(7):932-940. Epub 2018 Apr 19.
    Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., MLC 5031, Cincinnati, OH, 45229, USA.
    Background: Published case series have described central hepatic macroregenerative nodules or masses as a common feature of Alagille syndrome. Our experience suggests this regenerative pattern can be seen more generally in cholangiopathic disorders.

    Objective: To define the frequency of central regenerative tissue in Alagille syndrome and other cholangiopathic disorders and to describe the typical appearance of such regenerative tissue. Read More
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    [BRAF-V600E mutation and its clinical significance in children with Langerhans cell histiocytosis].
    Zhongguo Dang Dai Er Ke Za Zhi 2018 Apr;20(4):290-294
    Department of Pediatrics, West China Second University Hospital, Sichuan University/Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610041, China.
    Objective: To investigate the clinical significance of BRAF-V600E mutation in children with Langerhans cell histiocytosis (LCH).

    Methods: Real-time fluorescence quantitative PCR was used to detect BRAF-V600E mutation in paraffin-embedded tissue samples from 26 children with LCH. A retrospective analysis was performed for the association of BRAF-V600E mutation with clinical features and prognosis of children with LCH. Read More
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    Concurrent Mastoid Cellulitis and Langerhans Cells Histiocytosis: 
A Challenging Diagnosis.
    Oman Med J 2018 Mar;33(2):167-170
    Department of Otorhinolaryngology, Tengku Ampuan Rahimah Hospital, Klang, Selangor, Malaysia.
    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder, which commonly arises in the bone and may involve other systems. To date, the diagnosis of temporal bone LCH remains a challenge as it may masquerade as a common ear infection. We report a case of a child who presented to us with persistent bilateral ear discharge for four months and was not responding to treatment. Read More
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    Frequency of MAP2K1, TP53, and U2AF1 Mutations in BRAF-mutated Langerhans Cell Histiocytosis: Further Characterizing the Genomic Landscape of LCH.
    Am J Surg Pathol 2018 Jul;42(7):885-890
    Department of Pathology, University of Chicago, Chicago, IL.
    Langerhans cell histiocytosis is a proliferative disorder of neoplastic Langerhans cells with activating mutations in the Erk signaling pathway. TP53 and U2AF1 mutations have been implicated in other myelomonocytic malignancies and we hypothesized that mutations in these genes may cosegregate in LCH patients according to BRAF mutation status. Towards this end, we collected cases with a pathologic diagnosis of Langerhans cell histiocytosis from Stanford University Hospital. Read More
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    A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis.
    Arch Soc Esp Oftalmol 2018 Apr 8. Epub 2018 Apr 8.
    Unidad de Órbita y Oculoplástica, Servicio de Oftalmología, Hospital Universitario de Gerona Dr. Josep Trueta, Gerona, España.
    Clinical Case: A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Read More
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    Dermoscopy in the diagnosis of juvenile xanthogranuloma.
    An Bras Dermatol 2018 Jan-Feb;93(1):138-140
    Dermatology Department at Hospital Santa Casa de Curitiba - Curitiba (PR), Brazil.
    Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. Read More
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    Oral juvenile xanthogranuloma in a child: Clinical, histological and immunohistochemical profile of a rare entity.
    J Cutan Pathol 2018 Apr 6. Epub 2018 Apr 6.
    Oral Pathology Section, Universidad Autónoma de Guadalajara (UAG), Zapopan, Mexico.
    Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis (non-LCH) affecting normolipemic infants and children most frequently in the first year of life, often showing spontaneous regression within 3 to 6 years. Classic JXG is characterized by a yellowish asymptomatic papule or nodule, often located in the skin of the head, neck and upper trunk. Oral JXG has been reported, but is rare. Read More
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    CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.
    Cancer 2018 Jun 6;124(12):2607-2620. Epub 2018 Apr 6.
    Texas Children's Cancer Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
    Background: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH.

    Methods: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions. Read More
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    Craniofacial Manifestations of Systemic Disorders: CT and MR Imaging Findings and Imaging Approach.
    Radiographics 2018 May-Jun;38(3):890-911. Epub 2018 Apr 6.
    From the Departments of Radiology (V.C.A.A., M.N.C., H.K., A.F., O.S.), Otolaryngology-Head and Neck Surgery (O.S.), and Radiation Oncology (O.S.), Boston University Medical Center, Boston University School of Medicine, 820 Harrison Ave, 3rd Floor, Boston, MA 02118.
    Many systemic diseases or conditions can affect the maxillofacial bones; however, they are often overlooked or incidentally found at routine brain or head and neck imaging performed for other reasons. Early identification of some conditions may significantly affect patient care and alter outcomes. Early recognition of nonneoplastic hematologic disorders, such as thalassemia and sickle cell disease, may help initiate earlier treatment and prevent serious complications. Read More
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