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    10603 results match your criteria Langerhans Cell Histiocytosis

    1 OF 213

    Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma.
    Rom J Ophthalmol 2017 Jul-Sep;61(3):229-236
    Opthalmology Clinic, "Sf. Spiridon" University Hospital, Iași, Romania.
    Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. Read More

    Congenital Langerhans cell histiocytosis presenting in a 27-week-gestation neonate.
    Pediatr Dermatol 2018 Feb 13. Epub 2018 Feb 13.
    Section of Dermatology, University of Chicago, Chicago, IL, USA.
    Langerhans cell histiocytosis is exceedingly rare in premature infants, and the few cases reported suggest a poor prognosis with systemic involvement. We present a case of Langerhans cell histiocytosis limited to a single cutaneous lesion, presenting in a 27-week-gestation infant, which is the youngest gestational age of reported Langerhans cell histiocytosis cases. The lesion showed spontaneous resolution by 41 weeks corrected gestational age, and systemic involvement was absent, demonstrating a mild course of skin-only Langerhans cell histiocytosis in a premature infant. Read More

    Solving a Mystery . . . 8 Years Later.
    J Investig Med High Impact Case Rep 2018 Jan-Dec;6:2324709617752962. Epub 2018 Jan 24.
    Mayo Clinic, Rochester, MN, USA.
    Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis with multisystem involvement and insidious symptoms. In this article, we describe an interesting case of Erdheim-Chester disease that was eventually diagnosed 8 years after symptoms initially started. Read More

    Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature.
    Leuk Res 2018 Feb 2;67:39-44. Epub 2018 Feb 2.
    Cancer Research Foundation of New York, USA. Electronic address:
    Background: There is mounting evidence that Langerhans cell histiocytosis (LCH) and acute myeloid leukemia (AML) are hematopoietic neoplasms that arise from the same myeloid precursor cell. In addition, studies suggest a relationship between LCH and primary idiopathic myelofibrosis (MF). Furthermore familial LCH, AML, and MF have each been reported. Read More

    Multidisciplinary approach in a case of Hand-Schüller-Christian disease with maxillary involvement.
    Spec Care Dentist 2018 Feb 8. Epub 2018 Feb 8.
    Department of Clinical and Experimental Medicine, University of Foggia, Foggia, Italy.
    Langerhans cell histiocytosis (LCH) is a childhood pathology with a peak of incidence ranging from 1 to 4 years of age, though diagnosis is often made in adult age. LCH is clinically classified into three types: eosinophilic granuloma, Hand-Schuller-Christian disease and Abt-Letterer-Siwe disease. We report a case of Hand-Schüller-Christian disease with diabetes insipidus, skull and maxillary involvement in a 16-year-old boy referred to our observation for gradual increase in mobility of the teeth and subsequent gradual loss of the second premolars and the first molars of the upper jaw. Read More

    Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement.
    Int J Radiat Oncol Biol Phys 2018 Mar 6;100(3):670-678. Epub 2017 Nov 6.
    Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York. Electronic address:
    Purpose: To characterize the efficacy and safety of radiation therapy in a contemporary Langerhans cell histiocytosis (LCH) cohort and to explore whether there are sites at higher risk for local recurrence.

    Patients And Methods: Between 1995 and 2015 we identified 39 consecutive LCH patients who were treated primarily with radiation therapy. Patients were staged by single/multisystem involvement and established risk organ criteria. Read More

    Langerhans cell histiocytosis as an unusual cause of back pain in a child: A case report and review of literature.
    J Craniovertebr Junction Spine 2017 Oct-Dec;8(4):384-386
    Department of Pediatrics, Division of Pediatric Hemato Oncology, Sri Ramachandra University, Chennai, Tamil Nadu, India.
    Low back pain in children and adolescents are usually attributed to mechanical causes and faulty positions. Although most of them are self-limiting, physicians should be aware of the red flag signs that warrant complete evaluation to rule out malignant causes of back pain. As delay in the diagnosis of vertebral lytic lesion may have sequelae in the growing children, pain disproportionate to the signs should have low threshold levels for evaluation. Read More

    [Emphysema, did you say emphysema?]
    Rev Mal Respir 2018 Feb 2. Epub 2018 Feb 2.
    Service de pneumologie, hôpital Pasteur, universite Côte-d'Azur, FHU OncoAge, centre hospitalier universitaire de Nice, 06000 Nice, France.
    Introduction: Chronic obstructive pulmonary disease (COPD) is a common condition that may initially look simple but may conceal other diseases capable of accelerating its natural history or even simulating it. We describe four cases presenting as COPD with emphysema that were reclassified on the basis of certain clinical characteristics and the radiological pattern.

    Case Reports: A 52 year old never smoking woman presenting with emphysema was eventually diagnosed as having lymphangioleiomyomatosis on the basis of an abdominal CT scan showing kidney angiomyolipomas. Read More

    Xanthoma Disseminatum Presenting with Hoarseness.
    Iran J Otorhinolaryngol 2017 Nov;29(95):365-368
    Department of Pathology, JIPMER, Puducherry, India.
    Introduction: Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder with unknown etio-pathology. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs with a predilection for flexures and the face.

    Case Report: We report a patient who presented with disseminated xanthomatous papules and nodules involving the face, neck, trunk, axilla, groin, and oral cavity, along with hoarseness of voice. Read More

    Emergent presentation of Langerhans cell histiocytosis in a pediatric patient: Acute cerebellar involvement causing obstructive hydrocephalus requiring posterior fossa decompression.
    Indian J Radiol Imaging 2017 Oct-Dec;27(4):432-435
    Department of Radiology, University of Florida, Gainesville, Florida, USA.
    Langerhans cell histiocytosis (LCH) is a disorder of the monocyte-macrophage system that can be unifocal or systemic. Here, we present a pediatric case who initially presented with osseous LCH but again presented 6 years later emergently with cerebellar symptoms, cerebellar mass and obstructive hydrocephalus. Patient underwent biopsy of the cerebellum which was path proven intracranial LCH. Read More

    Reconstruction of a severe mandibular pathological fracture caused by Langerhans cell histiocytosis using a free fibula osteocutaneous flap:a case report.
    Case Reports Plast Surg Hand Surg 2018 10;5(1):9-13. Epub 2018 Jan 10.
    Department of Plastic Surgery, Kobe University Graduate School of MedicineKobeHyogoJapan.
    Langerhans cell histiocytosis (LCH) is a rare disease in which Langerhans cells, which are bone marrow-derived antigen-presenting cells, proliferate in single or multiple organs. We successfully treated a patient with unifocal LCH of the mandible with malocclusion due to a severe pathological fracture, using reconstruction with a vascularised free bone. Read More

    An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy.
    Expert Opin Pharmacother 2018 Feb 22;19(3):233-242. Epub 2018 Jan 22.
    a Department of Specialized Pediatrics , Sigmund Freud Private University , Vienna , Austria.
    Introduction: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasia driven by activation mutations alongside the MAPK pathway. Its broad spectrum of clinical manifestations and diverse course and clinical outcome, make interpretation of trial data difficult. Use of different stratification systems further complicates comparison among trials. Read More

    Histiocytosis - cutaneous manifestations of hematopoietic neoplasm and non-neoplastic histiocytic proliferations.
    J Eur Acad Dermatol Venereol 2018 Jan 16. Epub 2018 Jan 16.
    Department of Dermatology, University Hospital Zürich, Zürich, Switzerland.
    Histiocytoses are rare disorders characterized by the accumulation of cells derived from macrophages, dendritic cells or monocytes in various tissues. There is a broad spectrum of disease manifestations with some subtypes commonly showing skin lesions, while in others, the skin is rarely involved. Here, we describe cutaneous manifestations of histiocytoses belonging to the Langerhans group (L group), the group of cutaneous and mucocutaneous histiocytoses (C group) and the group of Rosai-Dorfman disease (RDD) and related histiocytoses (R group) according to the current classification. Read More

    Reflectance Confocal Microscopy for the Diagnosis of Langerhans Cell Histiocytosis.
    Br J Dermatol 2018 Jan 16. Epub 2018 Jan 16.
    Department of Dermatology, Archet 2 Hospital, Nice, France.
    Langerhans cell histiocytosis (LCH) is a rare proliferative disease occurring in young children. The most affected organs are the bones and the skin (1). The diagnosis of LCH relies on histopathology. Read More

    Expression of enhancer of zeste homolog 2 (EZH2) protein in histiocytic and dendritic cell neoplasms with evidence for p-ERK1/2-related, but not MYC- or p-STAT3-related cell signaling.
    Mod Pathol 2018 Jan 12. Epub 2018 Jan 12.
    Brigham and Women's Hospital and Harvard Medical School, Department of Pathology, Boston, MA, USA.
    EZH2 is an important enzymatic subunit of the epigenetic regulator polycomb repressive complex 2 (PRC2), which controls gene silencing through post-translational modification, and is overexpressed in various carcinomas and hematopoietic neoplasms. We found that the majority of cases of histiocytic and dendritic cell neoplasms, including histiocytic sarcoma, follicular dendritic cell sarcoma, Langerhans cell histiocytosis, and interdigitating dendritic cell sarcoma, show strong EZH2 expression by immunohistochemical staining, in contrast to benign histiocytic lesions and normal cellular counterparts, which did not show EZH2 expression, suggesting that this molecule may function as an oncogenic protein in these neoplasms. We correlated EZH2 expression with that of p-ERK1/2, MYC, and p-STAT3, potential regulators of EZH2, and found that 60-80% of these cases showed strong p-ERK1/2 expression, and only a minority of cases showed positivity for MYC or p-STAT3 in neoplastic cells. Read More

    Ninety-year-old man with hypereosinophilia, lymphadenopathies and pruritus.
    BMJ Case Rep 2018 Jan 10;2018. Epub 2018 Jan 10.
    Department of Oncology, Centro Hospitalar de Lisboa Ocidental EPE, Lisboa, Portugal.
    We report a case of a 90-year-old man with hypereosinophilia, lymphadenopathies and skin lesions, namely lichenification and pruritus. An aetiological investigation was performed, and a bone marrow (BM) biopsy and aspirate showed a hypercellular marrow with hypereosinophilia without dysmorphia or abnormal elements, and the BM and inguinal node's immunophenotyping denied any presence of abnormal lymphoid cell population. The inguinal node biopsy revealed a multinodular proliferation of large cells S100 and CD1a, and a diagnosis of Langerhans cell histiocytosis was made. Read More

    Intraoperative MRI-guided biopsy in the diagnosis of suprasellar Langerhans Cell Histiocytosis.
    World Neurosurg 2018 Jan 6. Epub 2018 Jan 6.
    Moores Cancer Center, University of California, San Diego, California, USA; Department of Neurosurgery, University of California, San Diego, California, USA. Electronic address:
    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. Read More

    Development of pulmonary Langerhans cell histiocytosis in a patient with established adenocarcinoma of the lung.
    J Thorac Dis 2017 Dec;9(12):E1079-E1083
    Division of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, Baltimore, MD, USA.
    Newly-appearing lung nodules on surveillance imaging in patients with pre-existing lung cancer can present a diagnostic dilemma when attempting to differentiate between metastatic disease, infection, and other inflammatory conditions. Here we report a case of an//metastatic adenocarcinoma patient who underwent lung biopsy for evaluation of upper-lobe predominant lung nodules revealed to represent pulmonary Langerhans cell histiocytosis (PLCH). The patient was a heavy smoker and admitted to increase her smoking habit after initially learning about her diagnosis with lung cancer. Read More

    Classic Hodgkin lymphoma with osseous involvement mimicking Langerhans cell histiocytosis in a child.
    Hum Pathol 2018 Jan 4. Epub 2018 Jan 4.
    Department of Anatomic Pathology, Hirosaki University Graduate School of Medicine, Hirosaki 036-8562, Japan. Electronic address:
    Hodgkin lymphoma (HL) commonly presents the superficial lymphadenopathy. In addition, HL cells can arise in various organs including the liver and spleen as an extranodal lymphoma. HL in bone is unusual at the initial diagnosis, although some cases show late stage localization of lymphoma cells to bone. Read More

    Fever and Skin Involvement at Diagnosis Predicting the Intractable Langerhans Cell Histiocytosis: 40 Case-Series in a Single Center.
    J Pediatr Hematol Oncol 2017 Dec 29. Epub 2017 Dec 29.
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University.
    Langerhans cell histiocytosis (LCH) occurs as a clonal disease with enigmatic immune responses. LCH patients occasionally present with fever, although the significance remains elusive. We investigated the predicting factors for developing intractable disease of refractory and/or reactivated LCH. Read More

    Pulmonary langerhans cell histiocytosis - insight into the incidence of alfa-1-antitrypsin (A1ATD) deficiency alleles.
    Adv Respir Med 2017 ;85(6):297-300
    3rd Lung Diseases Department, National Tuberculosis and Lung Disease Research Institute, Płocka 26, Warsaw, Poland.
    Introduction: The alpha-1 antitrypsin deficiency (A1ATD) is one of the three most common genetic disorders in Caucasians. It considerably increases the risk of progressive obstructive lung diseases, mostly chronic obstructive pulmonary disease. There is no data regarding prevalence of main, clinically most important A1ATD alleles PI*Z and PI*S in patients with pulmonary Langerhans cell histiocytosis (PLCH). Read More

    Erdheim-Chester Disease Involving Lymph Nodes and Liver Clinically Mimicking Lymphoma: A Case Report.
    J Pathol Transl Med 2017 Dec 27. Epub 2017 Dec 27.
    Department of Pathology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
    Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis and multisystem disease. First described in 1930, there are no more than 750 cases reported. The etiology remains unknown, but a majority of cases of ECD and Langerhans cell histiocytosis were found to have clonal mutations involving genes of the MAPK pathway. Read More

    BRAF V600E Mutation Across Multiple Tumor Types: Correlation Between DNA-based Sequencing and Mutation-specific Immunohistochemistry.
    Appl Immunohistochem Mol Morphol 2017 Dec 21. Epub 2017 Dec 21.
    Department of Pathology, University of New Mexico Health Sciences.
    The B-Raf proto-oncogene (BRAF) encodes a cytoplasmic serine/threonine kinase with a key role in regulating the mitogen-activated protein kinase signal transduction pathway. An activating missense mutation in codon 600 of exon 15 (V600E) of BRAF gene has been identified in multiple neoplasms including melanoma, colorectal carcinoma, papillary thyroid carcinoma, hairy cell leukemia, and Langerhans cell histiocytosis. Patients with BRAF V600E-mutated melanoma respond to FDA-approved BRAF inhibitors. Read More

    Congenital Langerhans cell histiocytosis: a good prognosis disease?
    An Bras Dermatol 2017 ;92(5 Suppl 1):40-42
    Department of Hematology, Instituto de Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro (IPPMG-UFRJ) - Rio de Janeiro (RJ), Brazil.
    Langerhans cell histiocytosis is rare and more frequent in children. The skin is affected in 50% of the cases and is the only site in 10%. Its course varies from self-limited and localized forms to severe multisystemic forms. Read More

    RAF/MEK/extracellular signal-related kinase pathway suppresses dendritic cell migration and traps dendritic cells in Langerhans cell histiocytosis lesions.
    J Exp Med 2018 Jan 20;215(1):319-336. Epub 2017 Dec 20.
    Department of Oncological Sciences, Icahn School of Medicine at Mount Sinai, New York, NY
    Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia characterized by granulomatous lesions containing pathological CD207dendritic cells (DCs) with constitutively activated mitogen-activated protein kinase (MAPK) pathway signaling. Approximately 60% of LCH patients harbor somaticV600E mutations localizing to CD207DCs within lesions. However, the mechanisms drivingV600ELCH cell accumulation in lesions remain unknown. Read More

    Pulmonary Langerhans cell histiocytosis in a 26-year-old female: still a diagnostic challenge.
    Rom J Morphol Embryol 2017 ;58(3):1047-1050
    Department of Pathology, University of Medicine and Pharmacy of Tirgu Mures, Romania;
    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. Read More

    Mixed histiocytosis: A case report and published work review.
    J Dermatol 2017 Dec 15. Epub 2017 Dec 15.
    Department of Dermatovenereology, West China Hospital, Sichuan University, Chengdu, China.
    Histiocytoses are a group of heterogeneous diseases that encompass Langerhans cell histiocytosis and non-Langerhans cell histiocytosis. Cutaneous plane xanthoma is a non-Langerhans cell histiocytic disorder characterized by the presence of yellow-orange plaques on the face, neck, upper trunk and extremities. It can appear in association with several systemic diseases (including dyslipidemias, paraproteinemias, cardiovascular diseases and lymphoproliferative disorders), but is rarely connected with Langerhans cell histiocytoses. Read More

    Retraction: 'Imaging characteristics of liver Langerhans cell histiocytosis in children' by Hui Liu, Xueying Long, Xiaoyi Wang, Wenzheng Li and Yigang Pei.
    • Authors:
    Pediatr Int 2017 09;59(9):1034
    The above article from Pediatrics International, published as an Accepted Article online on April 28, 2017 in Wiley Online Library (http://wileyonlinelibrary.com) has been withdrawn by agreement between the authors, the Journal Editor-in-Chief, Atsushi Manabe, and John Wiley & Sons Australia, Ltd, on behalf of the Japan Pediatric Society. The withdrawal has been agreed at the request of the authors who have identified some serious problems that need to be addressed and who have found that some important information is missing. Read More

    Prognostic factors for histiocytic and dendritic cell neoplasms.
    Oncotarget 2017 Nov 19;8(58):98723-98732. Epub 2017 Oct 19.
    Department of Pathology, Kurume University, School of Medicine, Kurume, Japan.
    Histiocytic and dendritic cell neoplasms are rare and poorly studied. We report the clinical characteristics and prognostic factors in such cases in Japan. We investigated the clinical characteristics and survival inadult patients with histiocytic and dendritic cell neoplasms. Read More

    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.
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN.
    Tobacco exposure results in various changes to the airways and lung parenchyma. Although emphysema represents the more common injury pattern, in some individuals, cigarette smoke injures alveolar epithelial cells 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 2018 Feb 4;265(2):273-284. 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 2018 Jan;40(1):e9-e12
    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 2018 Feb;42(2):150-159
    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 2018 Feb 28;110:365-370. Epub 2017 Nov 28.
    Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA. Electronic address:
    Background: 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

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