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IgG4-Related Disease: A Retrospective Chinese Study of Features and Treatment Response of 98 Patients Including 4 Rare Cases.

Curr Med Sci 2021 Apr 20;41(2):390-397. Epub 2021 Apr 20.

Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.

The features and treatment of 98 Chinese patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) referred to a single tertiary referring centre were reviewed. Patients diagnosed with IgG4-RD according to the comprehensive diagnostic criteria (CDC) were included in the retrospective study from May 2012 to March 2019. We collected data on clinical, laboratory, imaging, histological features and treatment. Read More

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Imaging of Bowel Lymphoma: A Pictorial Review.

Dig Dis Sci 2021 Apr 20. Epub 2021 Apr 20.

Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.

Intestinal lymphoma, although rare, is the second most common extra-nodal site of lymphoma, following stomach. It is usually secondary to systemic involvement and is predominantly of non-Hodgkin's subtype. In addition to the risk factors for lymphomas occurring elsewhere, certain risk factors are specific for intestinal lymphoma. Read More

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Clinical features and treatment outcomes of intraocular lymphoma: a single-center experience in China.

Int J Ophthalmol 2021 18;14(4):574-581. Epub 2021 Apr 18.

Department of Ophthalmology, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China.

Aim: To investigate the clinical manifestations, diagnostic approaches, treatments, and outcomes of intraocular lymphoma.

Methods: In this retrospective study, 16 patients (28 eyes) with intraocular lymphoma were recruited in the Department of Ophthalmology, Peking Union Medical College Hospital, from 2004 to 2019. All patients underwent comprehensive ophthalmic examinations. Read More

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Children with Kawasaki disease or Kawasaki-like syndrome (MIS-C/PIMS) at the time of COVID-19: are they all the same? Case series and literature review.

Reumatismo 2021 Apr 19;73(1):48-53. Epub 2021 Apr 19.

ASST G. Pini-CTO, Milan, Italy; Department of Clinical Sciences and Community Health, and Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan.

Since the coronavirus disease 2019 (COVID-19) outbreak started, children have been considered marginally involved compared to adults, with a quite significant percentage of asymptomatic carriers. Very recently, an overwhelming inflammatory activation, which shares clinical similarities with Kawasaki disease (KD), has been described in children exposed to COVID-19. We report three KD-like cases that occurred during the pandemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a highly affected area of Northern Italy. Read More

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Iron aggravates hepatic insulin resistance in the absence of inflammation in a novel db/db mouse model with iron overload.

Mol Metab 2021 Apr 16:101235. Epub 2021 Apr 16.

Department of Pediatric Hematology, Oncology and Immunology - University of Heidelberg, Heidelberg, Germany; Molecular Medicine Partnership Unit, Heidelberg, Germany. Electronic address:

Objective: The molecular pathogenesis of late complications associated with type 2 diabetes mellitus (T2DM) is not yet fully understood. While high glucose levels indicated by increased HbA1c only poorly explain disease progression and late complications, a pro-inflammatory status, oxidative stress and reactive metabolites generated by metabolic processes were postulated to be involved. Individuals with metabolic syndrome (MetS) frequently progress to T2DM, whereby 70% of T2DM patients show non-alcoholic fatty liver disease (NAFLD), the hepatic manifestation of MetS, and insulin resistance (IR). Read More

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Discriminating 'DRESS minor' from 'DRESS major': facial oedema aligns to the severe phenotype.

J Am Acad Dermatol 2021 Apr 16. Epub 2021 Apr 16.

Department of Dermatology, King's College Hospital, Denmark Hill, London, SE5 9RS, U.K.

Background: DRESS is a cutaneous and systemic drug allergy disorder. Patients exist on a severity spectrum with some developing a mild form of the disorder which fails to meet the RegiSCAR diagnostic criteria for DRESS.

Objective: We sought to determine if there were any cutaneous or dermatopathological features which discriminate between the mild form of DRESS ('DRESS minor') and the severe phenotype ('DRESS major'). Read More

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Collapsing Glomerulopathy as a Complication of Type I Interferon-Mediated Glomerulopathy in a Patient With RNASEH2B-Related Aicardi-Goutières Syndrome.

Am J Kidney Dis 2021 Apr 16. Epub 2021 Apr 16.

Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze;; Nephrology and Dialysis Unit, Meyer Children's Hospital, Firenze, Italy. Electronic address:

Aicardi-Goutières syndrome (AGS) is a well-characterised monogenic type I interferonopathy presenting with prominent neurological manifestations. Among extra-neurological features, renal involvement has only been described in one IFIH1-mutated patient who developed membranous nephropathy. The pathogenic role of augmented interferon signalling in tissues other than the central nervous system remains to be elucidated. Read More

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Myocarditis Presenting as Sudden Death in Infants and Children: A Single Centre Analysis by ESGFOR Study Group.

Pediatr Dev Pathol 2021 Apr 19:10935266211007262. Epub 2021 Apr 19.

Histopathology Department, Sheffield Children's NHS FT, Sheffield, UK.

Background: Acute myocarditis is an inflammatory disease of the heart mostly diagnosed in young people, which can present as sudden death. The etiology includes infectious agents (mostly viruses), systemic diseases and toxins. We aim to characterize infants and children with myocarditis at post-mortem presenting as sudden deaths. Read More

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Mortality in acute pancreatitis with persistent organ failure is determined by the number, type, and sequence of organ systems affected.

United European Gastroenterol J 2021 Mar;9(2):139-149

University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.

Background: Persistent organ failure (POF) is the strongest determinant of mortality in acute pancreatitis (AP). There is a paucity of data regarding the impact of different POF attributes on mortality and the role of different characteristics of systemic inflammatory response syndrome (SIRS) in the risk of developing POF.

Objective: We aimed to assess the association of POF dynamic features with mortality and SIRS characteristics with POF. Read More

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Mobile SAR signal N-hydroxypipecolic acid induces NPR1-dependent transcriptional reprogramming and immune priming.

Plant Physiol 2021 Apr 19. Epub 2021 Apr 19.

Institute for Molecular Ecophysiology of Plants, Department of Biology, Heinrich Heine University, Universitätsstraße 1, D-40225 Düsseldorf, Germany.

N-Hydroxypipecolic acid (NHP) accumulates in the plant foliage in response to a localized microbial attack and induces systemic acquired resistance (SAR) in distant leaf tissue. Previous studies indicated that pathogen inoculation of Arabidopsis (Arabidopsis thaliana) systemically activates SAR-related transcriptional reprogramming and a primed immune status in strict dependence of FLAVIN-DEPENDENT MONOOXYGENASE 1 (FMO1), which mediates the endogenous biosynthesis of NHP. Here, we show that elevations of NHP by exogenous treatment are sufficient to induce a SAR-reminiscent transcriptional response that mobilizes key components of immune surveillance and signal transduction. Read More

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Case Report of Two Patients With COVID-19 and Sacral Pressure Injuries Associated with Pyoderma Gangrenosum.

Adv Skin Wound Care 2021 Apr 16. Epub 2021 Apr 16.

In the Department of Plastic and Reconstructive Surgery, University Hospital Brussels, Belgium, Rania Elkhatib, MD, is Attending Physician; Gabriele Giunta, MD, FEBOPRAS, is Consultant; Valerie Hanssens, MSc, is Nurse Specialist; Ayush Kapila, MD, MRCS, is Resident; Randy De Baerdemaeker, MD, FEBOPRAS, is Consultant; Assaf Zeltzer, MD, PhD, is Consultant; and Moustapha Hamdi, MD, PhD, is Head of Department. The authors have disclosed no financial relationships related to this article. Submitted September 2, 2020; accepted in revised form October 28, 2020.

Abstract: With the continuous spread of the COVID-19 pandemic, an increasing number of patients have been admitted to the ICU with severe respiratory complications requing prolonged supine positioning. Recently, many case reports have been published regarding dermatological manifestations associated with the COVID-19 disease. Yet, there is little information about the clinical features. Read More

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Adult-onset asthma and periocular xanthogranuloma - A rare infiltrative disease of the orbit and eyelid.

Am J Ophthalmol Case Rep 2021 Jun 21;22:101043. Epub 2021 Mar 21.

Veterans Affairs Boston Healthcare System, Department of Ophthalmology, 150 South Huntington Avenue, Boston, MA, 02130, USA.

Purpose: To present a case of adult onset asthma with periocular xanthogranuloma (AAPOX), and discuss existing literature on adult orbital xanthogranulomatous diseases (AOXGDs) and their treatment.

Observations: A 63 year old male presented with progressive bilateral eyelid swelling with overlying yellow plaques associated with asthma. CT scan showed periorbital swelling with enlargement of the superior and lateral rectus muscles bilaterally. Read More

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Type I Interferonopathies in Children: An Overview.

Front Pediatr 2021 31;9:631329. Epub 2021 Mar 31.

Department of Pediatrics, University of Chieti, Chieti, Italy.

Notable advances in gene sequencing methods in recent years have permitted enormous progress in the phenotypic and genotypic characterization of autoinflammatory syndromes. Interferonopathies are a recent group of inherited autoinflammatory diseases, characterized by a dysregulation of the interferon pathway, leading to constitutive upregulation of its activation mechanisms or downregulation of negative regulatory systems. They are clinically heterogeneous, but some peculiar clinical features may lead to suspicion: a familial "idiopathic" juvenile arthritis resistant to conventional treatments, an early necrotizing vasculitis, a non-infectious interstitial lung disease, and a panniculitis associated or not with a lipodystrophy may represent the "interferon alarm bells. Read More

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Magnetic resonance lymphangiography in group 1 paediatric pulmonary arterial hypertension.

Pulm Circ 2021 Apr-Jun;11(2):20458940211004777. Epub 2021 Mar 30.

Instituto Oulton, Catholic University of Córdoba, Córdoba, Argentina.

Pulmonary hypertension could have thoracic lymphatic abnormalities caused by right ventricular failure. Since there is no description of such abnormalities, the purpose of this study was to investigate them with magnetic resonance. Prospective review magnetic resonance T2-weighted lymphangiography was performed between January 2017 and October 2019 through quantitative thoracic duct diameter, diameter index and qualitative lymphatic abnormalities types: 1 - little or none abnormalities, 2 - abnormalities in supraclavicular region, 3 - abnormalities extending into the mediastinum and 4 - abnormalities extending into the lung. Read More

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Serum Heparin-Binding Protein as a Potential Biomarker to Distinguish Adult-Onset Still's Disease From Sepsis.

Front Immunol 2021 31;12:654811. Epub 2021 Mar 31.

Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China.

Adult-onset Still's disease (AOSD) is a systemic, multifactorial, autoinflammatory disease for which the etiopathogenesis is not well understood. Given the similarities in clinical and laboratory features between this disease and sepsis, and the differences in treatment strategies for these two diseases, specific diagnostic markers are crucial for the correct diagnosis and management of AOSD. Previous studies have shown plasma heparin-binding protein (HBP) is a promising potential biomarker for AOSD; thus, this study aimed to detect serum HBP levels in patients with AOSD or sepsis to assess its potential as a biomarker for differential diagnosis. Read More

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Bacterial meningitis after dental extraction in a 17-year-old horse.

Can Vet J 2021 Apr;62(4):403-407

William R. Pritchard Veterinary Medical Teaching Hospital (Arndt, Heney, Wong), Department of Surgical and Radiological Sciences (Kilcoyne), Department of Medicine and Epidemiology (Magdesian), School of Veterinary Medicine, University of California, Davis, Davis, California 95616, USA.

Dental extractions in horses may result in bacteremia, which can lead to systemic complications. Bacterial meningitis following oral cheek tooth extractions in a 17-year-old Thoroughbred gelding is described in this report. The bacterial meningitis was confirmed by histopathology. Read More

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Inflammation in pediatric epilepsies: Update on clinical features and treatment options.

Epilepsy Behav 2021 Apr 15:107959. Epub 2021 Apr 15.

Department of Pediatric Neurology, Necker Enfants Malades Hospital, Reference Centre for Rare Epilepsies and Member of the ERN EpiCARE, Imagine Institute UMR1163, Paris Descartes University, Paris, France.

The role of inflammation is increasingly recognized in triggering or sustaining epileptic activity. In the last decades, increasing research has provided definite evidence to support the link between immunity, inflammatory process, and epilepsy. Neuro- and systemic inflammation play a pivotal role in driving epileptogenesis through different pathogenetic mechanisms: the activation of innate immunity in glia, neurons, and microvasculature, the brain mediated by blood-brain barrier (BBB) impairment, and the imbalance of pro- and anti-inflammatory molecules produced by both arms of immunity. Read More

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Sympathetic hyperactivity, hypertension, and tachycardia induced by stimulation of the ponto-medullary junction in humans.

Clin Neurophysiol 2021 Mar 24. Epub 2021 Mar 24.

Department of Neurosurgery, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.

Objective: The purpose of this study is to investigate changes in autonomic activities and systemic circulation generated by surgical manipulation or electrical stimulation to the human brain stem.

Methods: We constructed a system that simultaneously recorded microsurgical field videos and heart rate variability (HRV) that represent autonomic activities. In 20 brain stem surgeries recorded, HRV features and sites of surgical manipulation were analyzed in 19 hypertensive epochs, defined as the periods with transient increases in the blood pressure. Read More

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Academic integrity in nursing education: Policy review.

J Prof Nurs 2021 Mar-Apr;37(2):268-271. Epub 2020 Dec 17.

Mount Carmel College of Nursing, United States of America.

Background: Academic integrity is a matter of concern in nursing education. While cases of academic dishonesty are not public record, policies used to promote integrity in nursing education programs are widely available. A review of integrity policies in nursing programs explores common grounds in practices to promote integrity. Read More

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December 2020

Association of microRNA-125a with the clinical features, disease activity and inflammatory cytokines of juvenile-onset lupus patients.

Lupus 2021 Apr 17:9612033211010328. Epub 2021 Apr 17.

Human Genetics and Genome Research Division, Immunogenetics Department, National Research Centre, Giza, Egypt.

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with marked variation in its clinical presentation. Juvenile-onset SLE (jSLE) exhibits an aggressive clinical phenotype and severe complications. Dysregulated expression of microRNAs (miRs) in immune cells from patients with SLE has been found. Read More

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Left Ventricular Impaired Relaxation and Interstitial Myocarditis Identified in Sepsis-Associated Cardiac Dysfunction: Use of a Rodent Model.

Med Sci Monit 2021 Apr 18;27:e929512. Epub 2021 Apr 18.

Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia.

BACKGROUND Sepsis is a serious clinical problem that results from the systemic response of the body to infection. Left ventricular (LV) diastolic dysfunction is increasingly appreciated as a contributor to morbidity and mortality in sepsis. Animal models may offer a method of studying diastolic dysfunction while controlling for many potential clinical confounders, such as sepsis duration, premorbid condition, and therapeutic interventions. Read More

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Advanced image analytics predicting clinical outcomes in patients with colorectal liver metastases: A systematic review of the literature.

Surg Oncol 2021 Apr 15;38:101578. Epub 2021 Apr 15.

Amsterdam UMC, Vrije Universiteit Amsterdam, Department of Surgery, Cancer Center Amsterdam, De Boelelaan, 1117, Amsterdam, Netherlands.

Background: To better select patients with colorectal liver metastases (CRLM) for an optimal selection of treatment strategy (i.e. local, systemic or combined treatment) new prognostic models are warranted. Read More

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Pituitary metastasis unveiling a lung adenocarcinoma.

Endocrinol Diabetes Metab Case Rep 2021 Feb 26;2021. Epub 2021 Feb 26.

Endocrinology Department, Centro Hospitalar e Universitário do Porto, EPE, Porto, Portugal.

Summary: Pituitary metastasis (PM) can be the initial presentation of an otherwise unknown malignancy. As PM has no clinical or radiological pathognomonic features, diagnosis is challenging. The authors describe the case of a symptomatic PM that revealed a primary lung adenocarcinoma. Read More

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February 2021

Local and Regional Recurrences of Clinically Localized Renal Cell Carcinoma after Nephrectomy: A 15 Year Institutional Experience with Prognostic Features and Oncologic Outcomes.

Urology 2021 Apr 14. Epub 2021 Apr 14.

Department of Urology, The James Buchanan Brady Urological Institute, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address:

Objective: To evaluate outcomes for patients with local recurrence (LR) of clinically localized renal cell carcinoma (RCC) without concurrent systemic metastasis from our institution, an event that occurs rarely (1-3%) after surgery. LR may be a harbinger of poor outcomes, and the best management of these patients is unclear.

Materials/methods: We retrospectively reviewed patients surgically treated for clinically localized RCC (cT1-2N0M0) with subsequent LR (in the partial or radical nephrectomy bed) and/or regional recurrence (RR; in the abdomen distant from the direct site of surgery) without concurrent metastasis from our institutional database (2004-2018). Read More

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Systemic sclerosis-associated myositis features minimal inflammation and characteristic capillary pathology.

Acta Neuropathol 2021 Apr 17. Epub 2021 Apr 17.

Department of Neuropathology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Charitéplatz 1, 10117, Berlin, Germany.

Systemic sclerosis represents a chronic connective tissue disease featuring fibrosis, vasculopathy and autoimmunity, affecting skin, multiple internal organs, and skeletal muscles. The vasculopathy is considered obliterative, but its pathogenesis is still poorly understood. This may partially be due to limitations of conventional transmission electron microscopy previously being conducted only in single patients. Read More

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Biomarkers in COVID-19: An Up-To-Date Review.

Front Pediatr 2020 30;8:607647. Epub 2021 Mar 30.

Post Graduate Institute of Medical Education and Research, Chandigarh, India.

The ongoing pandemic of coronavirus disease 2019 (COVID-19) poses several challenges to clinicians. Timely diagnosis and hospitalization, risk stratification, effective utilization of intensive care services, selection of appropriate therapies, monitoring and timely discharge are essential to save the maximum number of lives. Clinical assessment is indispensable, but laboratory markers, or biomarkers, can provide additional, objective information which can significantly impact these components of patient care. Read More

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Investigation of type I interferon responses in ANCA-associated vasculitis.

Sci Rep 2021 Apr 15;11(1):8272. Epub 2021 Apr 15.

Department of Medical Gerontology, School of Medicine, Trinity Translational Medicine Institute, Trinity College Dublin, Dublin, Ireland.

Type I interferon (IFN) dysregulation is a major contributory factor in the development of several autoimmune diseases, termed type I interferonopathies, and is thought to be the pathogenic link with chronic inflammation in these conditions. Anti-neutrophil cytoplasmic antibody (ANCA)-Associated Vasculitis (AAV) is an autoimmune disease characterised by necrotising inflammation of small blood vessels. The underlying biology of AAV is not well understood, however several studies have noted abnormalities in type I IFN responses. Read More

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Peters Anomaly in Nail-Patella Syndrome: A Case Report and Clinico-Genetic Correlation.

Cornea 2021 Apr 14. Epub 2021 Apr 14.

The Centre of Excellence for Rare Eye Diseases, L V Prasad Eye Institute, Hyderabad, India; The Cornea Institute, L V Prasad Eye Institute, Hyderabad, India; Jasti V Ramanamma Children's Eye Care Center, L V Prasad Eye Institute, Hyderabad, India; Kallam Anji Reddy Molecular Genetics Laboratory, Brien Holden Eye Research Centre, L V Prasad Eye Institute, Banjara Hills, Hyderabad, India; Ophthalmic Pathology Services, L V Prasad Eye Institute, Hyderabad, India; University of Illinois Eye and Ear Infirmary, Chicago, IL; and Department of Ophthalmology, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.

Purpose: The purpose of this study was to report the clinicopathological features of Peters anomaly in a child with nail-patella syndrome.

Methods: Nail-patella syndrome (NPS) is a rare autosomal dominant connective tissue disorder characterized by several anomalies of the extremities, joints and nails, glomerulopathy, and rarely ocular involvement. NPS is caused by heterozygous loss-of-functional mutations in the LMX1B gene that encodes the LIM homeodomain proteins. Read More

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Paraproteinemias and Peripheral Nerve Disease.

Clin Geriatr Med 2021 May;37(2):301-312

Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA. Electronic address:

This article provides an overview of the clinical features, diagnosis, and treatment of the major paraprotein-related peripheral neuropathies, including monoclonal gammopathy of undetermined significance, Waldenström macroglobulinemia, POEMS syndrome, multiple myeloma, transthyretin amyloidosis, and light chain amyloidosis. For each paraprotein neuropathy, the epidemiology, demographics, systemic findings, and electrophysiologic features are presented. Pharmacologic treatment of transthyretin amyloid polyneuropathy also is reviewed. Read More

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Tumor Biology Impacts Survival in Surgically Managed Primary Hepatic Vascular Malignancies.

J Surg Res 2021 Apr 12;264:481-489. Epub 2021 Apr 12.

Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas; North Texas Veterans Affairs Medical Center, Dallas, Texas. Electronic address:

Background: Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database.

Materials And Methods: In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Read More

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