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    1 OF 212

    Erdheim-Chester Disease with atrial mass and perinephric calcification.
    Clin Case Rep 2017 Dec 2;5(12):2153-2154. Epub 2017 Nov 2.
    Division of RheumatologyMayo Clinic College of Medicine and ScienceRochesterMinnesota.
    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. Read More

    Current Concepts in Pathogenesis, Diagnosis, and Management of Smoking-Related Interstitial Lung Diseases.
    Chest 2017 Dec 5. Epub 2017 Dec 5.
    Dr. David E. and Bette H. Dines Professor of Pulmonary Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, 200 1(st) ST, SW, Rochester, MN 55905. Electronic address:
    Tobacco exposure results in various changes to the airways and lung parenchyma. While emphysema represents the more common injury pattern, in some individuals, cigarette smoke injures alveolar epithelial and other lung cells resulting in diffuse infiltrates and parenchymal fibrosis. Smoking can trigger interstitial injury patterns mediated via recruitment and inappropriate persistence of myeloid and other immune cells including eosinophils. Read More

    Adult-onset Langerhans cell histiocytosis of the sternum.
    J Thorac Dis 2017 Sep;9(9):E783-E786
    Division of Thoracic Surgery, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
    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. Read More

    Erdheim-Chester Disease with No Skeletal Bone Involvement and Massive Weight Loss.
    Case Rep Hematol 2017 30;2017:3862052. Epub 2017 Oct 30.
    Division of Hematology and HSCT, Department of Oncology, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
    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. Read More

    Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report.
    Childs Nerv Syst 2017 Dec 5. Epub 2017 Dec 5.
    Department of Dermatology, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Jeollanam-do, Republic of Korea.
    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. Read More

    Adult leukoencephalopathies with prominent infratentorial involvement can be caused by Erdheim-Chester disease.
    J Neurol 2017 Dec 4. Epub 2017 Dec 4.
    Unit of Neurodegerative and Neurometabolic Rare Diseases, IRCCS Foundation "Carlo Besta" Neurological Institute, Milan, Italy.
    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. Read More

    Langerhans Cell Histiocytosis: A Diagnostic Challenge in the Oral Cavity.
    Case Rep Pathol 2017 24;2017:1691403. Epub 2017 Oct 24.
    Department of Oral and Maxillofacial Surgery, Case Western Reserve University, School of Dental Medicine, Cleveland, OH, USA.
    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. Read More

    Sinus Histiocytosis With Massive Lymphadenopathy (Rosai Dorfman Disease): Diagnostic and Treatment Modalities for this Rare Entity Revisited.
    J Pediatr Hematol Oncol 2017 Dec 1. Epub 2017 Dec 1.
    Cook Children's Medical Center, Fort Worth, TX.
    Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy is a rare non-Langerhans' cell histiocytic disease resulting from the proliferation and accumulation of sinus histiocytes within lymph nodes. Extranodal involvement frequently occurs, which increases the morbidity and mortality of the disease. There is no clear consensus with regard to the most effective diagnostic and treatment modalities. Read More

    Treatment Outcome of Children With Multisystem Langerhans Cell Histiocytosis: The Experience of a Single Children's Hospital in Shanghai, China.
    J Pediatr Hematol Oncol 2017 Dec 1. Epub 2017 Dec 1.
    Key Laboratory of Pediatric Hematology & Oncology, Department of Hematology and Oncology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
    We reported the outcome of 150 children newly diagnosed with multisystem langerhans cell histiocytosis following a langerhans cell histiocytosis-II-based protocol (arm B). However, the continuation treatment was extended to 56 weeks and etoposide was omitted from the continuation treatment. Risk organ (RO) involvement was defined as: liver (≥3 cm with or without functional impairment); spleen (≥2 cm below the costal margin in the midclavicular line); hematopoietic system (hemoglobin <100 g/L, and/or white blood cell count <4. Read More

    Endocrine and metabolic assessment in adults with Langerhans cell histiocytosis.
    Eur J Intern Med 2017 Nov 29. Epub 2017 Nov 29.
    U.O. di Endocrinologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Dipartimento di Scienze Cliniche e di Comunità, Università di Milano, Milan, Italy.
    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. Read More

    Individualized Treatment Approaches for Langerhans Cell Histiocytosis.
    Br J Dermatol 2017 Nov 30. Epub 2017 Nov 30.
    Department of Dermatology, University Hospital Zürich, University of Zurich, Zurich, Switzerland.
    Langerhans cell histiocytosis (LCH) belongs to the rare histiocytic disorders, and has an estimated incidence of 1-2 cases per million adults [1]. Myeloid dendritic cells that express the same antigens (CD1a, CD207) as epidermal Langerhans cell seem to be the precursor cells for LCH [2]. Clinical presentation of patients with LCH may vary in site and extent of involvement. Read More

    CDKN2A/B Deletion and Double-hit Mutations of the MAPK Pathway Underlie the Aggressive Behavior of Langerhans Cell Tumors.
    Am J Surg Pathol 2017 Nov 29. Epub 2017 Nov 29.
    Cancer Research Center of Marseille (CRCM), INSERM U1068.
    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. Read More

    The histopathology of Erdheim-Chester disease: a comprehensive review of a molecularly characterized cohort.
    Mod Pathol 2017 Dec 1. Epub 2017 Dec 1.
    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
    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. Read More

    Intracranial Erdheim-Chester Disease mimicking parafalcine meningioma: report of two cases and review of the literature.
    World Neurosurg 2017 Nov 27. Epub 2017 Nov 27.
    Baylor College of Medicine, Department of Neurosurgery, Houston, TX, USA. Electronic address:
    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. Read More

    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.
    JAMA Oncol 2017 Nov 29. Epub 2017 Nov 29.
    Memorial Sloan Kettering Cancer Center, New York, New York.
    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. Read More

    Juvenile xanthogranuloma with angiomatous appearance and a peculiar immunophenotype.
    Pediatr Dermatol 2017 Nov 22. Epub 2017 Nov 22.
    Department of Dermatology, Hospital Universitario Fundacion Jiménez Diaz, Universidad Autónoma, Madrid, Spain.
    Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis in childhood. The clinical differential diagnosis of a solitary juvenile xanthogranuloma includes molluscum contagiosum, Spitz nevus, and melanoma. Lesions larger than 2 cm in diameter may be misdiagnosed as hemangiomas, but this is not typical of smaller juvenile xanthogranuloma. Read More

    Clinicopathological and Immunohistochemical Study of Head and Neck Langerhans Cell Histiocytosis from Latin America.
    Head Neck Pathol 2017 Nov 21. Epub 2017 Nov 21.
    Department of Pathology, School of Medicine, Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
    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). Read More

    Successful cochlear implantation in Langerhans cell histiocytosis: A rare case.
    Cochlear Implants Int 2017 Nov 20:1-4. Epub 2017 Nov 20.
    c Department of Otorhinolaryngology , SMS Medical College , Jaipur , Rajasthan , India.
    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). Read More

    A rapidly expanding calvarial Langerhans cell histiocytosis with low Ki-67 in an adult: a challenging diagnosis on magnetic resonance imaging.
    Neuroradiol J 2017 Jan 1:1971400917741905. Epub 2017 Jan 1.
    3 Bahçeşehir University School of Medicine, Turkey.
    Langerhans cell histiocytosis may have a wide variety of clinical presentations. The growth of localised form is usually slow and it mainly involves bones such as the skull, femur, spine, ribs, mandible and pelvis in children. The spectrum of clinical manifestations and magnetic resonance imaging findings of the disease may sometimes mimic infections as well as benign and malignant tumours. Read More

    Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.
    Clin Imaging 2017 Oct 31;49:17-36. Epub 2017 Oct 31.
    Department of Radiology, Division of Pediatric Radiology, Montefiore Medical Center, Bronx, NY, United States. Electronic address:
    Purpose: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs.

    Methods: We review the imaging findings in childhood diseases associated with dermatologic manifestations. Read More

    Spontaneous Remission of Severe Systemic Langerhans Cell Histiocytosis with Bladder Involvement: A Case Study.
    Case Rep Oncol 2017 Sep-Dec;10(3):876-884. Epub 2017 Oct 5.
    Department of Gynecology and Gynecological Oncology, Hospital for Women, University Hospital Basel, Basel, Switzerland.
    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. Read More

    Intradural Eosinophilic Granuloma Invading Skull: Case Report and Review of the Literature.
    Asian J Neurosurg 2017 Oct-Dec;12(4):698-700
    Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.
    Eosinophilic granuloma is a localized form of Langerhans cell histiocytosis, most commonly involving the skeletal system. Their origin from the dura is rare with only a handful of cases on record. We present one such rare case of an eosinophilic granuloma originating from the dura mater with secondary osseous invasion in an 11-year-old female child who presented with a swelling in the right parietal region. Read More

    JAK2-mutated langerhans cell histiocytosis associated with primary myelofibrosis treated with ruxolitinib.
    Hum Pathol 2017 Oct 28. Epub 2017 Oct 28.
    Department of Pathophysiology and Organ Transplantation, University of Milan, Via Festa del Perdono 7, (20122) Milan, Italy; Department of Dermatology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Via Pace 9, (20122) Milan, Italy.
    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. Read More

    Langerhans cell histiocytosis: A neoplastic disorder driven by Ras-ERK pathway mutations.
    J Am Acad Dermatol 2017 Oct 26. Epub 2017 Oct 26.
    Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address:
    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. Read More

    Oral manifestations of systemic disease.
    Gen Dent 2017 Nov-Dec;65(6):23-29
    On examination, the oral cavity may exhibit manifestations of underlying systemic disease and serve as an indicator of overall health. Systemic diseases with oral findings include autoimmune, hematologic, endocrine, and neoplastic processes. Autoimmune disease may manifest as oral ulcerations, changes in the salivary and parotid glands, and changes in the tongue. Read More

    CNS Erdheim-Chester Disease: A Challenge to Diagnose.
    J Neuropathol Exp Neurol 2017 Dec;76(12):986-996
    Department of Pathology; Department of Neurology; and Department of Neurosurgery, University of Colorado Denver, Aurora, Colorado.
    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). Read More

    [Langerhans cell histiocytosis with isolated sternum involvement. A clinical case].
    Arch Argent Pediatr 2017 Dec;115(6):e416-e419
    Departamento de Urgencias, Hospital de Niños "Ricardo Gutiérrez" de Buenos Aires, Ciudad Autónoma de Buenos Aires.
    The Langerhans Cell Histiocytosis (LCH) is a rare condition, characterized by the proliferation of dendritic cells. Its clinical presentation is variable and ranges from an isolated skin or bone disease, mainly the skull, to a life-threatening multisystemic disease. This case is about a healthy 8-year-old girl with a history of four days of severe sternum pain and no other symptomatology. Read More

    Genetic homogeneity of adult Langerhans cell histiocytosis lesions: Insights from BRAFV600E mutations in adult populations.
    Oncol Lett 2017 Oct 18;14(4):4449-4454. Epub 2017 Aug 18.
    Medical School, University of Buckingham, Buckingham MK18 1EG, UK.
    Langerhans cell histiocytosis (LCH) is a heterologous disease with a recognized disparity in incidence, affected sites and prognosis between adults and children. The recent identification of BRAFV600E mutations in LCH prompted the investigation of the frequency of these mutations in adult and childhood disease with the involvement of single or multiple sites in the present study. The study analysed the BRAFV600E status in a cohort of adult LCH patients by DNA sequencing, and performed a broader meta-analysis of BRAFV600E mutations in LCH in order to investigate any association with disease site and severity. Read More

    Pulmonary Langerhans' cell histiocytosis in adults.
    Adv Respir Med 2017 30;85(5):277-289. Epub 2017 Oct 30.
    3rd Lung Diseases Department, National Tuberculosis and Lung Disease Research Institute, Warsaw, Poland.
    Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived Langerhans' cells, which carry mutations of BRAF gene and/or NRAS, KRAS and MAP2K1 genes. It occurs predominantly in young smokers, without gender predominance. The disease is characterised by formation of eosinophilic granulomas with the presence of Langerhans' cells infiltrating and destroying distal airways. Read More

    ALK Fusions in a Wide Variety of Tumor Types Respond to Anti-ALK Targeted Therapy.
    Oncologist 2017 Oct 27. Epub 2017 Oct 27.
    Foundation Medicine, Inc., Cambridge, Massachusetts, USA.
    Background: Genomic fusions of the anaplastic lymphoma kinase gene (ALK) are a well-established therapy target in non-small cell lung cancer (NSCLC). From a survey of 114,200 clinical cases, we determined the prevalence of ALK rearrangements (rALK) in non-NSCLC tumors and report their responsiveness to therapies targeting ALK.

    Materials And Methods: Comprehensive genomic profiling of 114,200 relapsed and metastatic malignancies, including both solid tumors and hematolymphoid cancers, was performed using a hybrid-capture, adaptor ligation-based next-generation sequencing assay. Read More

    [Difficult diagnosis of Erdheim Chester disease revealed by central diabetes insipidus].
    Rev Med Liege 2017 Oct;72(10):436-438
    Service d'Endocrinologie-Diabétologie et Médecine interne, CHU Tahar Sfar, Mahdia, Tunisie.
    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. Read More

    Pulmonary Langerhans cell histiocytosis "de novo" after lung transplantation.
    Pathol Int 2017 Dec 20;67(12):632-637. Epub 2017 Oct 20.
    Pathology Service, University Hospital, "Marqués de Valdecilla", IDIVAL, Santander, Spain.
    A pulmonary Langerhans cell histiocytosis is presented in a 40 year-old woman two years after bilateral lung transplantation for emphysema without any signs of Langerhans cells proliferation in the explanted lungs. A microsatellite molecular analysis showed the proliferating cells were generated in a recipient cellular clone. The patient did not quit smoking after transplantation. Read More

    Medical Management of Pulmonary Hypertension with Unclear and/or Multifactorial Mechanisms (Group 5): Is There a Role for Pulmonary Arterial Hypertension Medications?
    Curr Hypertens Rep 2017 Oct 18;19(11):86. Epub 2017 Oct 18.
    Univ. Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France.
    Purpose Of Review: The purpose of this review was to outline the mechanisms and to review recent literature on pulmonary arterial hypertension (PAH) medications in group 5 pulmonary hypertension (PH).

    Recent Findings: The first steps in management are to understand the mechanisms and hemodynamic profile and to exclude chronic thromboembolic disease. Recent studies in the past 5 years have found that PAH medications may improve hemodynamics in patients with pre-capillary pulmonary hypertension due to sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and myeloproliferative disorders with dasatinib-induced PH. Read More

    Extensive nail changes in a toddler with multisystemic Langerhans cell histiocytosis.
    Pediatr Dermatol 2017 Nov 17;34(6):732-734. Epub 2017 Oct 17.
    Department of Pediatric Hepatology, Gleneagles Global Health City, Chennai, India.
    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. Read More

    Perianal Langerhans cell histiocytosis: a rare presentation in an adult male.
    Autops Case Rep 2017 Jul-Sep;7(3):38-43. Epub 2017 Sep 30.
    Menoufia University, Liver Institute. Shebein Elkom, Menoufia, Egypt.
    Langerhans cell histiocytosis (LCH) is a rare disease characterized by a proliferation of cells that show immunophenotypic and ultrastructural similarities with antigen-presenting Langerhans cells of mucosal sites and skin. LCH in adults is rare, and there are still many undiagnosed/misdiagnosed patients. We describe LCH involvement of the perianal region of a 33-year-old male with a previous history of diabetes insipidus. Read More

    Membranoproliferative glomerulonephritis associated with Rosai-Dorfman disease.
    Clin Nephrol Case Stud 2017 30;5:54-59. Epub 2017 Aug 30.
    Department of Pediatrics, Kinki University Faculty of Medicine, Osaka, Japan.
    Rosai-Dorfman disease is also known as sinus histiocytosis with massive lymphadenopathy. Extranodal Rosai-Dorfman disease has been reported in ~ 43% of cases; the most frequent extranodal sites - skin, soft tissue, bone, respiratory tract, and eye - are usually involved in association with lymphadenopathy. Lack of lymph node involvement is rare, especially when patients manifest renal disease. Read More

    A rare case of CD1a-negative Langerhans cell histiocytosis of the central nervous system in a child.
    Clin Case Rep 2017 Oct 1;5(10):1664-1667. Epub 2017 Sep 1.
    Cook Children's Medical CenterFort WorthTexas.
    Langerhans cell histiocytosis is a dendritic cell disorder with a wide spectrum of severity and presentations. Histopathology typically demonstrates a proliferation of Langerhans cells and a lymphohistiocytic inflammatory infiltrate with eosinophils. The diagnosis is supported by immunohistochemistry with the cell markers S100, CD1a, CD68, and Langerin [Blood, 126, 2015, 26 and N Engl J Med, 331, 1994, 154]. Read More

    Atlantoaxial Langerhans cell histiocytosis radiographic characteristics and corresponding prognosis analysis.
    J Craniovertebr Junction Spine 2017 Jul-Sep;8(3):199-204
    Department of Orthopaedics, Peking University Third Hospital, Beijing, PR China.
    Background: Langerhans cell histiocytosis (LCH) may affect atlas and axis, and there were very few published cases describing a characteristic of LCH of atlantoaxial.

    Objective: The objective of the study is to investigate the image manifestations of atlantoaxial LCH to improve the in-depth comprehension on it.

    Materials And Methods: A retrospective study was done of computed tomography (CT) and magnetic resonance imaging in atlas and axis and prognosis was analyzed. Read More

    Occult Langerhans Cell Histiocytosis in Clear Cell Renal Cell Carcinoma.
    Int J Surg Pathol 2017 Oct 1:1066896917735171. Epub 2017 Oct 1.
    1 Yeungnam University, Daegu, South Korea.
    Langerhans cell histiocytosis is a rare disease that is characterized by a localized or systemic proliferation of Langerhans dendritic cells and a wide spectrum of clinical presentations. We experienced an unusual case of occult Langerhans cell histiocytosis associated with clear cell renal cell carcinoma. A 62-year-old man underwent a partial nephrectomy for left renal mass. Read More

    Langerhans Cell Histiocytosis of the Thoracic Spine in an Adult.
    Korean J Spine 2017 Sep 30;14(3):109-111. Epub 2017 Sep 30.
    Department of Neurosurgery, Ewha Womans University College of Medicine, Seoul, Korea.
    We report a case of a 45-year-old man with a complaint of both leg weakness and hypoesthesia. Radiological evaluation revealed an osteolytic lesion of the ninth thoracic vertebra. The patient underwent posterior corpectomy with total excision of the tumor, mesh cage insertion with posterior screw fixation and subsequent radiotherapy. Read More

    Vulvar lesions in an 8-year-old girl: cutaneous manifestations of multisystem Langerhans cell histiocytosis.
    J Pediatr Adolesc Gynecol 2017 Oct 6. Epub 2017 Oct 6.
    Department of Obstetrics, Gynecology & Reproductive Sciences, Yale University School of Medicine, New Haven, CT.
    Background: Langerhans cell histiocytosis (LCH) is a rare localized or systemic disease characterized by proliferation of myeloid-derived dendritic cells. Vulvar lesions may be the herald symptom of LCH and may mimic other cutaneous lesions. Prognosis varies widely based on the extent and spread of disease. Read More

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