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    1 OF 208

    Killer cell immunoglobulin-like receptor 2DL4 is expressed in and suppresses the cell growth of Langerhans cell histiocytosis.
    Oncotarget 2017 Apr 7. Epub 2017 Apr 7.
    Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.
    Killer cell immunoglobulin-like receptor (KIR) 2DL4 (CD158d) is a receptor for human leukocyte antigen-G. The function of KIR2DL4 has been reported in human natural killer cell lymphoma and mastocytosis, but not in Langerhans cell histiocytosis (LCH). Herein, we examined the expression and function of KIR2DL4 in LCHs. Read More

    Clinical presentation and outcome of children with central diabetes insipidus associated with a self-limited or transient pituitary stalk thickening, diagnosed as infundibuloneurohypophysitis.
    Clin Endocrinol (Oxf) 2017 Apr 26. Epub 2017 Apr 26.
    Division of Pediatric Endocrinology, Vrije Universiteit Brussel, UZ Brussel Kinderziekenhuis, Brussels.
    Objective: Despite lymphocytic or autoimmune infundibuloneurohypophysitis (INH) is an increasingly recognized etiology in children with central diabetes insipidus, clinical data on epidemiology (clinical evolution, predisposing factors, complications), diagnosis and management of this entity are limited and mostly based on published case reports. The aim of this study was to gain a broader insight in the natural history of this disease by analyzing the clinical presentation, radiological pituitary stalk changes, associated autoimmunity and hormonal deficiencies in children with CDI and a self-limiting or transient stalk thickening (ST), diagnosed as autoimmune INH, during the last 15 years in four Belgian university hospitals.

    Design And Patients: The medical files of nine CDI patients with a ST at initial presentation and no signs of Langerhans cell histiocytosis or germinoma at presentation and/or during follow up of more than 1. Read More

    Circulating cell-free BRAF(V600E) as a biomarker in children with Langerhans cell histiocytosis.
    Br J Haematol 2017 Apr 25. Epub 2017 Apr 25.
    French Reference Centre for Langerhans Cell Histiocytosis, Trousseau Hospital, Paris, France.
    The BRAF(V600E) mutation is reported in half of patients with Langerhans cell histiocytosis (LCH). This study investigated the detection of the BRAF(V600E) allele in circulating cell-free (ccf) DNA in a paediatric LCH cohort. Children with BRAF(V600E) -mutated LCH were investigated to detect ccf BRAF(V600E) at diagnosis (n = 48) and during follow-up (n = 17) using a picolitre-droplet digital PCR assay. Read More

    Late outcomes in children with Langerhans cell histiocytosis.
    Arch Dis Child 2017 Apr 25. Epub 2017 Apr 25.
    Department of Paediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong.
    Introduction: Langerhans cell histiocytosis (LCH) is a rare disease with diverse clinical courses. Despite improvement in survival outcomes in the recent decades, sequelae of the disease remain a concern. This study aimed to provide information on the long-term outcomes in patients with LCH, particularly on the sequelae and any associated factors. Read More

    Evaluation of pituitary uptake incidentally identified on 18F-FDG PET/CT scan.
    Oncotarget 2017 Feb 16. Epub 2017 Feb 16.
    Department of Nuclear Medicine, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
    The clinical significance of pituitary uptake on routine whole body 18F-fluorodeoxyglucose (FDG) positron emission tomography/computer tomography (PET/CT) is not completely characterized. We seek to assess the potential differential diagnosis/underlying etiology of pituitary FDG uptake incidentally identified on routine PET/CT scans. A total of 24,007 PET/CT whole body scans in recent 5 years were retrospectively reviewed. Read More

    Clinical Spectrum, Quality of life, BRAF Mutation Status and Treatment of Skin Involvement in Adult Langerhans Cell Histiocytosis.
    Acta Derm Venereol 2017 Apr 19. Epub 2017 Apr 19.
    Assistance Publique- Hôpitaux de Paris, , Département de Dermatologie, Hôpital Saint-Louis, , Paris, France.
    Langerhans cell histiocytosis is a rare histiocytic disorder for which skin involvement and management are poorly described in adults. The aim of this retrospective monocentric study in a national reference centre is to describe the clinical characteristics, quality of life, BRAF mutation status and outcomes of skin involvement in adult patients with Langerhans cell histiocytosis. Twenty-five patients (14 females, mean age 47 years) were included, with a median follow-up of 33 months (range 4-420 months). Read More

    Nuclear Medicine Techniques in Pediatric Bone Imaging.
    Semin Nucl Med 2017 May 12;47(3):190-203. Epub 2017 Jan 12.
    Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA. Electronic address:
    An important mandate when imaging pediatric patients is the reduction of radiation exposure to the lowest possible level consistent with good quality diagnostic imaging, so individual selection of radiopharmaceutical dosage and imaging technique is essential. Although the logistics of image acquisition and the approach to interpretation mirror the more common use in adults, the challenges of imaging young children typically require greater attention to patient preparation, positioning, and supervision during imaging, with the use of parental or family engagement in the procedure, gentle restraint, and occasionally the need for sedation. Because the normal bone scan appearance varies dramatically with age as osseous structures mature, it is critical that the interpreting physician be well versed in the range of normal variability at each developmental stage. Read More

    Fine Needle Aspiration of Langerhans Cell Histiocytosis: A Cytopathologic Study of 37 Cases.
    Acta Cytol 2017 Apr 14. Epub 2017 Apr 14.
    Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei, Taiwan.
    Objective: Langerhans cell histiocytosis (LCH) is an uncommon neoplasm of dendritic cells and predominantly occurs in children and young adults. The study aims to evaluate cytopathologic features and current diagnostic concepts in a large series of LCH on fine needle aspiration (FNA).

    Study Design: We retrospectively searched the pathology archives of The Johns Hopkins Hospital (JHH) and Emory University Hospital (EUH) to identify all FNA cases diagnosed as LCH in a period of 21 years. Read More

    Pulmonary langerhans cell histiocytosis case with diabetes insipidus and tuberculosis.
    Niger J Clin Pract 2017 Apr;20(4):503-506
    Pamukkale University, Medicine Faculty, Pathology Department, Denizli, Turkey.
    A 19-year-old male patient was observed due to having central diabetes insipidus (DI) for five years. He had a history of smoking 5-10 cigarettes a day for two years, but stopped smoking from the last month. The computerized tomography revealed thin-walled cystic lesions in different sizes more dominantly in the upper lobes and consolidated areas in the left upper and lower lobes. Read More

    an aggressive multifocal primary CNS histiocytosis with PTPN11 (Shp2) mutation.
    Neuropathol Appl Neurobiol 2017 Apr 12. Epub 2017 Apr 12.
    Department of Pathology and Laboratory Medicine, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada.
    Primary histiocytic tumors of the CNS are rare. The current WHO classification (2016) included 5 entities: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and histiocytic sarcoma (HS) (1). The diagnosis usually is made based on the tumor differentiation as to the counterpart in normal histiocyte development. Read More

    Hemosiderotic Juvenile Xanthogranuloma.
    Am J Dermatopathol 2017 Mar 16. Epub 2017 Mar 16.
    *Department of Cellular Pathology, Hospital El Bierzo, Cellular Pathology, Ponferrada, Spain; †Biomedical Investigation Institute of A Coruña, CellCOM-ST Group, A Coruña, Spain; ‡Department of Cellular Pathology, Hospital de la Reina, Cellular Pathology, Ponferrada, Spain; §Department of Histopathology, Children's Birmingham Hospital, Birmingham, United Kingdom; ¶Department of Histopathology, Birmingham Children's Hospital, Birmingham, United Kingdom; ‖Department of Histopathology, University Children's Hospital, Dublin, Ireland; and **Department of Histopathology, Hospital Niño Jesus, Madrid, Spain.
    Juvenile xanthogranuloma is a non-Langerhans cell lesion mostly limited to the skin but occasionally presenting in extracutaneous locations or associated with systemic conditions. Lesions need to be distinguished mainly from dermatofibroma, xanthoma, Langerhans cell histiocytosis, or reticulohistiocytoma. Herein, we present a hemosiderotic variant of juvenile xanthogranuloma in a 12-year-old girl, which we have not found described in literature. Read More

    Bone pathologic fracture revealing an unusual association: coexistence of Langerhans cell histiocytosis with Rosai-Dorfman disease.
    BMC Clin Pathol 2017 7;17. Epub 2017 Apr 7.
    Departement of pathology, Hassan II University hospital, Fès, Morocco.
    Background: The coexistence of Rosai-Dorfman disease (RDD) with Langerhans cell histiocytosis (LCH) is very rare, as to date only 17 cases have been reported in the english literature. The pathophysiology of this uncommon co-occurrence still remains enigmatic and a subject of various speculations.

    Case Presentation: We report a case of a 30-year-old female patient who presented with a pathologic fracture of the left proximal femur. Read More

    Langerhans-cell histiocytosis of the cervical spine in an adult patient: Case report and review of the literature.
    J Orthop 2017 Jun 25;14(2):264-267. Epub 2017 Mar 25.
    Department of Orthopedics and Trauma Surgery, Medical University of Graz, Austria.
    Langerhans-cell histiocytosis (LCH) is a rare, benign bone tumor, usually occurring in children and younger adults under 20 years old. Only a few cases of solitary bone lesions of the adult spine are reported in literature, therapeutic guidelines or treatment regimens for lesions of the adult spine are not established yet to our knowledge. Read More

    Spontaneous and complete regeneration of a vertebra plana after surgical curettage of an eosinophilic granuloma.
    Eur Spine J 2017 Mar 30. Epub 2017 Mar 30.
    Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
    Purpose: The eosinophilic granuloma is a unifocal or multifocal Langerhans cell histiocytosis characterized by an expanding proliferation of Langerhans cells in bones. Skeletal LCH is a rare condition, and vertebral regeneration in cases of vertebral body collapse is even rarer. We report the case of a girl with spontaneous complete healing. Read More

    Langerhans cell histiocytosis masquerading as acute appendicitis: Case report and review.
    World J Gastrointest Endosc 2017 Mar;9(3):139-144
    Mohammad M Karimzada, Daniel DeUgarte, Dennis Y Kim, Department of Surgery, Harbor-UCLA Medical Center, Torrance, CA 90502, United States.
    Langerhans cell histiocytosis (LCH) is a rare syndrome characterized by unifocal, multifocal unisystem, or disseminated/multi-system disease that commonly involves the bone, skin, lymph nodes, pituitary, or sometimes lung (almost exclusively in smokers) causing a variety of symptoms from rashes and bone lesions to diabetes insipidus or pulmonary infiltrates. We present a previously unreported case of gastrointestinal LCH as well as a novel characteristic lesion affecting the colon of a young woman who presented with signs and symptoms mimicking acute on chronic appendicitis. Immunohistochemical analysis of appendectomy specimen and nodular specimens on colonoscopy demonstrated S-100, CD1a, and langerin reactivity. Read More

    Cranium-Penetrating Mass Detected by Ultrasound Expedited Management of Langerhans Cell Histiocytosis.
    Pediatr Emerg Care 2017 Apr;33(4):290-292
    From the *New York Methodist Hospital, Brooklyn, NY; and †Department of Emergency Medicine, Denver Health, Denver, CO.
    A 6-year-old boy presented to the pediatric emergency department with a unilateral 5 × 3-cm superficial mass on the postauricular region growing for 1 month. Point-of-care ultrasound was used to evaluate the mass, which revealed a complex cystic mass penetrating the temporal bone. After confirmatory magnetic resonance imaging, the patient was transferred for neurosurgical evaluation, and the tumor was excised. Read More

    A confusing case report of pulmonary langerhans cell histiocytosis and literature review.
    Open Med (Wars) 2016 15;11(1):178-182. Epub 2016 Jun 15.
    Department of Pathology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.
    Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare disease. From the insidious onset and nonspecific manifestations, it is difficult to diagnose PLCH. To help improve the diagnosis and therapy options of adult PLCH, we present this case report and literature review about a confusing case of PLCH. Read More

    Osteopontin: its potential role in cancer of children and young adults.
    Biomark Med 2017 Apr 14;11(4):389-402. Epub 2017 Mar 14.
    Department of Pediatrics, Hematology & Oncology, Medical University of Gdansk, 7 Debinki Street, 80-211 Gdansk, Poland.
    Objective: Osteopontin (OPN) is aglyco-phosphoprotein, involved in tissue remodeling, inflammation and boneresorption. In various adult neoplasms OPN was shown to correlate with cancer progression, invasiveness and metastasis.

    Aim: to define the role of OPN in malignancies of children and young adults. Read More

    Fluorrine-18-fluorodeoxyglucose positron emission tomography/computed tomography of adult liver Langerhans cell histiocytosis.
    Hell J Nucl Med 2017 Jan-Apr;20(1):97-99. Epub 2017 Mar 20.
    Department of Nuclear Medicine, Jinling Hospital, Medical school of Nanjing University, Nanjing, Jiangsu Province,210002, China.
    Objective: Adult liver Langerhans cell histiocytosis (LCH) is an extremely rare desease. This paper reports a 40 years old male patient who was diagnosed as liver LCH though ultrasound-guided liver biopsy. The initial Fluorrine-18- fluorodeoxyglucose positron emission tomography/ computed tomography ((18)F-FDG PET/CT) showed multiple nodular low-density lesions in liver without obvious elevated (18)F-FDG uptake. Read More

    Optimal Diagnostic Yield Achieved With On-site Pathology Evaluation of Fine-Needle Aspiration-Assisted Core Biopsies for Pediatric Osseous Lesions: A Single-Center Experience.
    Arch Pathol Lab Med 2017 Mar 16. Epub 2017 Mar 16.
    Context: - Image-guided, fine-needle aspiration-assisted core needle biopsy with an on-site evaluation by a pathologist (FNACBP) of osseous lesions is not a common practice in pediatric institutions.

    Objectives: - To evaluate the diagnostic adequacy and accuracy of FNACBP for pediatric osseous lesions and to compare the adequacy with procedures that do not use fine-needle aspiration.

    Design: - Six-year, retrospective review of 144 consecutive children biopsied for osseous lesions with and without fine-needle aspiration assistance. Read More

    A Rare Case of Solitary Giant Congenital Juvenile Xanthogranuloma.
    J Cutan Med Surg 2017 Feb 1:1203475417690718. Epub 2017 Feb 1.
    3 Hôpital de Maria, Maria, QC, Canada.
    Juvenile xanthogranuloma (JXG) is the most frequent form of non-Langerhans cell histiocytosis. We present a case of giant congenital JXG in a 7-week-old boy, who had a firm and incompressible lesion, measuring 3 × 4 cm in diameter, on his right flank. The clinical appearance of the lesion and the ultrasound results suggested a vascular tumor, such as a hemangioma. Read More

    WHO 2016 Classification: Changes and Advancements in the Diagnosis of Miscellaneous Primary CNS Tumours.
    Neuropathol Appl Neurobiol 2017 Mar 12. Epub 2017 Mar 12.
    Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
    This short review highlights significant changes and recent findings incorporated to varying extent in the WHO 2016 definition of a variety of tumours, including peripheral nerve sheath tumours, meningiomas, mesenchymal non-meningothelial tumours, melanocytic tumours, lymphomas and histiocytic tumours, germ cell tumours and non-neuroendocrine pituitary tumours. Most notable classification changes include: adding "hybrid nerve sheath tumours" to the spectrum of benign nerve sheath tumours; an updated definition of atypical meningioma (WHO grade II), including cases with brain invasion; recognizing dural solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) as a single tumour entity characterized by NAB2 and STAT6 gene fusions for which the term solitary SFT/HPC was chosen; recognizing that pituitary granular cell tumour, spindle cell oncocytoma, and pituicytoma all share nuclear expression of TTF-1, possibly representing a spectrum of a single nosological entity derived from posterior pituitary glial cells. The most significant diagnostic markers which have emerged include: inactivation of NF1, CDKN2A, and PRC2 components, SUZ12 and EED in MPNST, leading to neurofibromin and H3K27me3 expression loss; GNAQ and GNA11 mutations in CNS primary melanocytic tumours; BRAFV600E mutation in histiocytic tumours (Langerhans cell histiocytosis and Erdheim-Chester disease) and papillary craniopharyngioma, which provides both a diagnostic marker in the appropriate pathological setting and a therapeutic target. Read More

    Perianal Lesions in Children: An Updated Review.
    Am J Clin Dermatol 2017 Mar 13. Epub 2017 Mar 13.
    Department of Dermatology, State University of New York Downstate Medical Center, 10 West, 800 Poly Place, Brooklyn, NY, 11203, USA.
    Perianal lesions in children are common reasons for dermatology clinic visits and a well-defined approach to diagnosis and management is helpful to the practicing clinician. In this article, we review and update various etiologies of perianal lesions in the pediatric population, including infectious, papulosquamous, vascular, and neoplastic. We provide a standard initial approach to diagnosis and updates on current management. Read More

    Non-odontogenic tumors of the facial bones in children and adolescents: role of multiparametric imaging.
    Neuroradiology 2017 Apr 13;59(4):327-342. Epub 2017 Mar 13.
    Division of Radiology, Department of Imaging and Medical Informatics, Geneva University Hospital, University of Geneva, Rue Gabrielle Perret Gentil 4, 1211, Geneva, Switzerland.
    Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin. Read More

    Denosumab for the treatment of adult multisystem Langerhans cell histiocytosis.
    Metabolism 2017 Apr 12;69:107-111. Epub 2017 Jan 12.
    Department of Pathophysiology, National University of Athens, 75 Mikras Asias St., 11527, Athens, Greece.
    Purpose: An etiological treatment is currently lacking for Langerhans Cell Histiocytosis (LCH). Receptor activator of nuclear factor κB ligand (RANKL) appears to play a central role in the lesional immunological process inducing compensatory osteoprotegerin (OPG) activation. In a preliminary study we aimed to evaluate for the first time the use of denosumab, a RANKL inhibitor, as a targeted treatment strategy in LCH in order to support and enhance endogenous OPG action in order to control or alter the lesional immunological process. Read More

    Multifocal Eosinophilic Granuloma of Jaws and Skull with Classical and Unusual Radiographic/Imaging Findings.
    J Clin Diagn Res 2017 Jan 1;11(1):ZD09-ZD11. Epub 2017 Jan 1.
    Senior Lecturer, Department of Oral Medicine and Radiology, CKS Teja Institute of Dental Sciences and Research , Tirupati-Andhra Pradesh, India .
    Eosinophilic granuloma is basically a disorder of reticuloendothelial system and is one of the variants of langerhans cell histiocytosis. Multifocal eosinophilic granuloma affecting jaws and skull is relatively a rare disorder. We hereby report a case of multifocal eosinophilic granuloma involving mandible, maxilla and several skull bones. Read More

    Diagnosis and treatment of cystic lung disease.
    Korean J Intern Med 2017 Mar 28;32(2):229-238. Epub 2017 Feb 28.
    Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.
    Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. Read More

    (18)F-FDG PET/CT in follow-up evaluation in pediatric patients with Langerhans histiocytosis.
    Rev Esp Med Nucl Imagen Mol 2017 Mar 2. Epub 2017 Mar 2.
    Unidad PET, CETIR-ERESA, Esplugues de Llobregat, Barcelona, España.
    Purpose: We evaluated the impact of (18)F-FDG PET/CT in identifying sites of active disease and to assess therapeutic follow up in a group of pediatric patients with Langerhans cell histiocytosis (LCH).

    Method: During 2007-2013, 13 (18)F-FDG PET/CT studies were performed for follow-up in 7 patients with a diagnosis of LCH (4 female, 3 male; 1-12 years-old). PET findings were analyzed and correlated with the CT and MRI. Read More

    DIAGNOSIS OF ENDOCRINE DISEASE: Expanding the cause of hypopituitarism.
    Eur J Endocrinol 2017 Jun 3;176(6):R269-R282. Epub 2017 Mar 3.
    School of MedicineUniversity of Belgrade, Belgrade, Serbia
    Hypopituitarism is defined as one or more pituitary hormone deficits due to a lesion in the hypothalamic-pituitary region. By far, the most common cause of hypopituitarism associated with a sellar mass is a pituitary adenoma. A high index of suspicion is required for diagnosing hypopituitarism in several other conditions such as other massess in the sellar and parasellar region, brain damage caused by radiation and by traumatic brain injury, vascular lesions, infiltrative/immunological/inflammatory diseases (lymphocytic hypophysitis, sarcoidosis and hemochromatosis), infectious diseases and genetic disorders. Read More

    Idiopathic thrombocytopenic purpura in childhood, Langerhans cell histiocytosis in adulthood: More than a chance association?
    Med J Malaysia 2017 Feb;72(1):50-52
    Putrajaya Hospital, Department of Medicine, Endocrine Unit, Malaysia.
    Described herein, a case of Langerhans cell histiocytosis (LCH) in an adult with Idiopathic Thrombocytopenic Purpura (ITP) diagnosed at age ten. She presented with cranial diabetes insipidus, later developed hypogonadotrophic hypogonadism and multiple cervical lympadenopathy from which histopathology of excisional biopsy confirmed LCH. Magnetic resonance imaging showed thickened pituitary stalk. Read More

    Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation: An adult case report.
    Medicine (Baltimore) 2017 Mar;96(9):e5994
    Organ Transplant Center, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
    Background: Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis. LCH diseases may occur in a single organ or multisystem organ. The patients with multisystem involvement usually have a poor prognosis. Read More

    Successful treatment of histiocytic sarcoma with cladribine and high-dose cytosine arabinoside in a child.
    Int J Hematol 2017 Feb 28. Epub 2017 Feb 28.
    Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Chuou-ku, Niigata, Niigata, 951-8510, Japan.
    Histiocytic sarcoma, a rare hematopoietic neoplasm with evidence of histiocytic differentiation, is often refractory to conventional chemotherapy and radiotherapy, and its prognosis is generally dismal. The optimal management of this malignancy has not been established. We report a case of 8-year-old girl with histiocytic sarcoma involving the left femur. Read More

    Management of eosinophilic granuloma in pediatric patients: surgical intervention and surgery combined with postoperative radiotherapy and/or chemotherapy.
    Childs Nerv Syst 2017 Apr 28;33(4):583-593. Epub 2017 Feb 28.
    Department of Spine Surgery, Xiangya Hospital of Central South University, Xiangya Road 87, Changsha, Hunan Province, 410008, China.
    Background: Eosinophilic granuloma (EG) of spine in pediatric patients presents kinds of clinical manifestation and a difficult management scenario. The choice of treatment, issues of surgical intervention versus conservative treatment, combination therapy or single treatment, all these factors, including neurological deficits, spinal stability, long-term complications, and continued skeletal growth, must be considered.

    Methods: From 2008 to the 2015, 31 pediatric patients of spinal EG were retrospectively reviewed. Read More

    Molecular analysis of BRAF V600E mutation in multiple nodules of pulmonary Langerhans cell histiocytosis.
    Virchows Arch 2017 Apr 20;470(4):429-435. Epub 2017 Feb 20.
    Institute of Pathology, St. Vincent's Hospital Karlsruhe, Suedendstrasse 37, 76137, Karlsruhe, Germany.
    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related histiocytic disorder with variable clinical symptoms. Like in other non-pulmonary Langerhans cell proliferations, PLCH has recently been shown to harbour BRAF V600E mutations in a significant subset of cases, thus challenging the concept of PLCH being a reactive disorder. Here, we analysed 38 formalin-fixed and paraffin-embedded PLCH nodules of nine patients for BRAF mutation using two different molecular methods. Read More

    The use of BRAF V600E mutation-specific immunohistochemistry in pediatric Langerhans cell histiocytosis.
    Hematol Oncol 2017 Feb 20. Epub 2017 Feb 20.
    Department of Pathology, Texas Children's Hospital, Houston, TX, USA.
    BRAF p.V600E mutations are detected in greater than 50% of pediatric Langerhans cell histiocytosis (LCH) lesions. However, the use of mutation-specific BRAF V600E immunohistochemistry (IHC) as a surrogate for molecular testing in pediatric LCH is unknown. Read More

    A Unique Case of Increased 18F-FDG Metabolic Activity in the Soft Tissues of the Bilateral Upper Thighs Due to Immunizations in a Pediatric Patient.
    World J Nucl Med 2017 Jan-Mar;16(1):59-61
    Department of Radiology, Division of Nuclear Medicine, Naval Medical Center San Diego, San Diego, California, USA.
    A case of a 7-month-old white female who was referred for 18F-fluorodeoxyglucose (FDG) Positron emission tomography/computed tomography (PET/CT) initial evaluation of a lytic skull lesion with presumed diagnosis of Langerhans cell histiocytosis is described. Incidentally, she was found to have hypermetabolic nodules in the soft tissues of her anterior thighs. Read More

    [Zygomatic localization of Langerhans cell histiocytosis - a case report].
    Wiad Lek 2016 ;69(6):829-831
    Katedra i Zakład Podstawowych Nauk Medycznych, Wydział Zdrowia Publicznego w Bytomiu, Śląski Uniwersytet Medyczny w Katowicach, Polska.
    Langerhans cell histocytosis is a rare disease. LCH is reported mainly in children aged less than 15 years, however it may appear at any age. In this work, the case of 17-month boy is reported. Read More

    Histiocytoses: emerging neoplasia behind inflammation.
    Lancet Oncol 2017 Feb;18(2):e113-e125
    Service de Médecine Interne 2, Centre National de Référence Maladies Auto Immunes Systémiques Rares, Institut E3M, Hôpital Pitié-Salpêtrière, Paris, France.
    Histiocytoses are disorders characterised by inflammation and the accumulation of cells derived from the monocyte and macrophage lineages, which results in tissue damage. Although they are often considered rare disorders with protean clinical manifestations, considerable advances in the understanding of their genetics have led to increased clinical recognition of these conditions, and fuelled further insights into their pathogenesis. In this Review, we describe insights into the cells of origin, molecular pathology, clinical features, and treatment strategies for some of the most common histiocytic disorders, including Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Read More

    Common Skin Conditions in Children: Neonatal Skin Lesions.
    FP Essent 2017 Feb;453:11-17
    University of North Carolina Chapel Hill School of Medicine Dermatology Residency Program, 410 Market St. Suite 400 CB#7715, Chapel Hill, NC 27516.
    Skin findings during the initial month of life are ubiquitous. One study estimated that more than 95% of newborns have cutaneous findings, which often are distressing to parents but frequently are benign and self-limited. Among them are milia, cutis marmorata, congenital dermal melanocytosis, and the benign neonatal pustular eruptions (eg, benign cephalic pustulosis, erythema toxicum neonatorum, transient neonatal pustular melanosis). Read More

    Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes.
    JCI Insight 2017 Feb 9;2(3):e89473. Epub 2017 Feb 9.
    Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center.
    Many patients with histiocytic disorders such as Langerhans cell histiocytosis (LCH) or Erdheim-Chester disease (ECD) have treatment-refractory disease or suffer recurrences. Recent findings of gene mutations in histiocytoses have generated options for targeted therapies. We sought to determine the utility of prospective sequencing of select genes to further characterize mutations and identify targeted therapies for patients with histiocytoses. Read More

    Traumatic Rib Injury: Patterns, Imaging Pitfalls, Complications, and Treatment.
    Radiographics 2017 Mar-Apr;37(2):628-651. Epub 2017 Feb 10.
    From the School of Medicine and Dentistry, University of Rochester Medical Center, 601 Elmwood Ave, Box 648, Rochester, NY 14642.
    The ribs are frequently affected by blunt or penetrating injury to the thorax. In the emergency department setting, it is vital for the interpreting radiologist to not only identify the presence of rib injuries but also alert the clinician about organ-specific injury, specific traumatic patterns, and acute rib trauma complications that require emergent attention. Rib injuries can be separated into specific morphologic fracture patterns that include stress, buckle, nondisplaced, displaced, segmental, and pathologic fractures. Read More

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