5,329 results match your criteria Paget Disease


Determinants of Survival in Skull Base Osteosarcoma: A National Cancer Database Study.

World Neurosurg 2021 May 8. Epub 2021 May 8.

Department of Otolaryngology - Head and Neck Surgery, University of California, Irvine, Orange, CA, USA;; Department of Neurological Surgery, University of California, Irvine, Orange, CA, USA;. Electronic address:

Objective: Osteosarcomas of the skull base are rare and aggressive tumors that are most commonly treated with primary surgical resection and adjuvant chemoradiation. Using the National Cancer Database (NCDB), we analyzed demographic and clinical prognosticators for overall survival (OS).

Methods: The NCDB was queried for cases of histologically confirmed skull base osteosarcoma treated between 2004 and 2015, excluding patients receiving palliation or having less than 1 month of follow-up. Read More

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Definitive Radiotherapy for Penoscrotal Extramammary Paget's Disease: A Case Report with Long-Term Follow-Up.

Clin Med Insights Case Rep 2021 10;14:11795476211009251. Epub 2021 Apr 10.

Department of Radiology, Saiseikai Shiga Hospital, Ritto, Shiga, Japan.

Penoscrotal extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma in older male patients, and no effective nonsurgical treatment strategies are currently available. The aim of this study was to report the usefulness of external radiotherapy (RT) for penoscrotal EMPD in an inoperable elderly patient. This report presents the treatment of an 89-year-old man with widespread penoscrotal EMPD. Read More

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Adipophilin expression in skin lesions with clear cell histology.

J Clin Pathol 2021 May 5. Epub 2021 May 5.

Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China

Aims: Clear cells formed due to depositions of glycogen or lipids in the cytoplasm commonly occur in various tissues. Adipophilin (ADP), a lipid regulatory protein, is closely related to lipid droplets. This study aims to examine adipophilin expression in clear cells of various skin lesions. Read More

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Isolation and Characterization of Human Adipocyte-Derived Extracellular Vesicles using Filtration and Ultracentrifugation.

J Vis Exp 2021 Apr 19(170). Epub 2021 Apr 19.

Division of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford.

Extracellular vesicles (EVs) are lipid enclosed envelopes that carry biologically active material such as proteins, RNA, metabolites and lipids. EVs can modulate the cellular status of other cells locally in tissue microenvironments or through liberation into peripheral blood. Adipocyte-derived EVs are elevated in the peripheral blood and show alterations in their cargo (RNA and protein) during metabolic disturbances, including obesity and diabetes. Read More

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Tocilizumab in patients admitted to hospital with COVID-19 (RECOVERY): a randomised, controlled, open-label, platform trial.

Authors:

Lancet 2021 May;397(10285):1637-1645

Background: In this study, we aimed to evaluate the effects of tocilizumab in adult patients admitted to hospital with COVID-19 with both hypoxia and systemic inflammation.

Methods: This randomised, controlled, open-label, platform trial (Randomised Evaluation of COVID-19 Therapy [RECOVERY]), is assessing several possible treatments in patients hospitalised with COVID-19 in the UK. Those trial participants with hypoxia (oxygen saturation <92% on air or requiring oxygen therapy) and evidence of systemic inflammation (C-reactive protein ≥75 mg/L) were eligible for random assignment in a 1:1 ratio to usual standard of care alone versus usual standard of care plus tocilizumab at a dose of 400 mg-800 mg (depending on weight) given intravenously. Read More

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J Pharmacol Exp Ther 2021 Apr 30. Epub 2021 Apr 30.

Gavin Herbert Eye Institute, Dept. of Ophthalmology, University of California, Irvine, United States

CB-5083 is an inhibitor of p97/valosin-containing protein (VCP), for which Phase I trials for cancer were terminated due to adverse effects on vision, such as photophobia and dyschromatopsia. Lower dose CB-5083 could combat inclusion body myopathy with early-onset Paget disease; and frontotemporal dementia or multisystem proteinopathy, caused by gain-of-function mutations in VCP. We hypothesized that the visual impairment in the cancer trial was due to CB-5083's inhibition of phosphodiesterase-6 (PDE6), which mediates signal transduction in photoreceptors. Read More

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Epigenetic analysis of Paget's disease of bone identifies differentially methylated loci that predict disease status.

Elife 2021 Apr 30;10. Epub 2021 Apr 30.

College of Health and Life Sciences, Hamad Bin Khalifa University, Doha, Qatar.

Paget's Disease of Bone (PDB) is characterized by focal increases in disorganized bone remodeling. This study aims to characterize PDB associated changes in DNA methylation profiles in patients' blood. Meta-analysis of data from the discovery and cross-validation set, each comprising of 116 PDB cases and 130 controls, revealed significant differences in DNA methylation at 14 CpG sites, 4 CpG islands, and 6 gene-body regions. Read More

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Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse.

Cells 2021 Apr 8;10(4). Epub 2021 Apr 8.

Mechanisms in Cell Biology and Disease Group, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, SA 5000, Australia.

Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disease with significant neurological and skeletal pathologies. Respiratory dysfunction is a secondary pathology contributing to mortality in MPS IIIA patients. Pulmonary surfactant is crucial to optimal lung function and has not been investigated in MPS IIIA. Read More

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Structural insight into mutations at 155 position of valosin containing protein (VCP) linked to inclusion body myopathy with Paget disease of bone and frontotemporal Dementia.

Saudi J Biol Sci 2021 Apr 19;28(4):2128-2138. Epub 2021 Feb 19.

Department of Neurology, Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou 563000, China.

Mutations in have been implicated in the pathology linked to inclusion body myopathy, paget disease of bone and frontotemporal dementia (IBMPFD). VCP is an essential component of AAA-ATPase superfamily involved in various cellular functions. Advanced analysis was performed using prediction based servers to determine the most deleterious mutation. Read More

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Temporal Variations in Respiratory Syncytial Virus Epidemics, by Virus Subtype, 4 Countries.

Emerg Infect Dis 2021 May;27(5):1537-1540

Temporal variation of respiratory syncytial virus (RSV) epidemics was recently reported to be determined by the dominant RSV subtype. However, when we repeated the analysis for 4 countries in the Northern and Southern Hemispheres, the dominant subtype did not seem to affect temporal variation of RSV epidemics. Read More

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Anogenital syringocystadenocarcinoma papilliferum in situ or anaplastic extramammary Paget disease? A unifying concept and review of the literature.

J Cutan Pathol 2021 Apr 24. Epub 2021 Apr 24.

Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.

Extramammary Paget disease (EMPD) is a rare cutaneous malignancy that typically involves the genital skin and can be primary or associated with an underlying internal malignancy. The typical histopathological appearance of EMPD consists of single or small aggregates of cells with abundant pale cytoplasm and large pleomorphic nuclei, known as Paget cells, scattered throughout the epidermis. We report a case of anogenital EMPD occurring in a 53-year-old man with unusual histopathologic findings of marked epidermal acanthosis, acantholysis, intraepidermal glandular differentiation, and prominent plasma cell-rich fibrovascular cores. Read More

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Vulvar Paget's disease and stromal invasion: Clinico-pathological features and survival outcomes.

Surg Oncol 2021 Apr 16;38:101581. Epub 2021 Apr 16.

Gynecology and Obstetrics 1, Department of Surgical Sciences, City of Health and Science, University of Torino, Torino, Italy.

Purpose: To evaluate clinico-pathological features, treatments and survival outcomes of vulvar Paget's disease (VPD).

Methods: We retrospectively reviewed VPD diagnosed between 1983 and 2018 at the Department of Surgical Sciences, Sant'Anna Hospital, Turin. Clinico-pathological characteristics and surgical treatment outcomes were investigated according to the depth of invasion. Read More

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Recommendations for respiratory syncytial virus surveillance at national level.

Eur Respir J 2021 Apr 22. Epub 2021 Apr 22.

Statens Serum Institut, Copenhagen, Denmark.

Respiratory syncytial virus (RSV) is a common cause of acute lower respiratory tract infections (ALRI) and hospitalisations among young children and is globally responsible for many deaths in young children, especially in infants below 6 months of age. Furthermore, RSV is a common cause of severe respiratory disease and hospitalisation among the elderly. The development of new candidate vaccines and monoclonal antibodies highlights the need for reliable surveillance of RSV. Read More

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Ectopic extramammary Paget disease in thoracic location.

Dermatol Online J 2021 Mar 15;27(3). Epub 2021 Mar 15.

Dermatology Team, Institute of Oncology, Fundación Arturo López Pérez (FALP), Santiago.

Extramammary Paget disease (EMPD) is a rare skin cancer that affects areas with a high concentration of apocrine glands including genital, axillary, and anal skin. When it affects other locations it is called ectopic extramammary Paget disease (E-EMPD) and is uncommon. To date, there are only 45 case reports to the best of our knowledge. Read More

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High-Frequency Ultrasound for Evaluation of the Pathological Invasion Level of Extramammary Paget Disease.

J Ultrasound Med 2021 Apr 15. Epub 2021 Apr 15.

Department of Medical Ultrasound, Shanghai Skin Disease Hospital, Ultrasound Research and Education Institute, Tongji University School of Medicine, Shanghai, China.

Objectives: Pathological invasion level of extramammary Paget disease (EMPD) is strongly related with its risk staging, treatment, and prognosis. However, the current evaluation before treatments fails to evaluate pathological invasion level of EMPD. High-frequency ultrasound (HFUS) may play a key role to solve this problem. Read More

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The role of simulation in training breast surgeons: a systematic review.

Ann R Coll Surg Engl 2021 May 14;103(5):318-323. Epub 2021 Apr 14.

St Andrew's Centre for Plastic Surgery and Burns, Broomfield Hospital, Chelmsford, UK.

Background: As surgical education has evolved, most curricula have favoured a competency-based approach over traditional apprenticeship models. Surgical simulation can be a useful aide in the training of both oncological and reconstructive breast surgery trainees. This review investigates the extent to which simulation of breast surgery procedures has been validated as a training tool. Read More

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Clinical course in two children with Juvenile Paget's disease during long-term treatment with intravenous bisphosphonates.

Bone Rep 2021 Jun 9;14:100762. Epub 2021 Mar 9.

Department of Pediatrics, Division of Rare Diseases, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany.

Juvenile Paget disease (JPD) is an ultra-rare disease, characterized by loss of function of osteoprotegerin. Osteoprotegerin inhibits osteoclast activation via the receptor activator of nuclear factor κB (RANK) pathway. Severely affected children suffer from bone deformities and pain and require long term anti-resorptive treatment. Read More

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Survival analysis of extramammary Paget's disease (EMPD) in a tertiary hospital in Taiwan.

World J Surg Oncol 2021 Apr 12;19(1):110. Epub 2021 Apr 12.

Division of Plastic and Reconstructive Surgery, Department of Surgery, Taipei Veterans General Hospital, 19F, No.201 Shih-Pai RD Sec 2, Taipei, Taiwan.

Background: This study aimed to investigate the survival analysis of extramammary Paget's disease (EMPD) in a Taiwanese population and to provide data for comparison with other studies in various locations and racial populations.

Methods: We retrospectively analyzed the medical records of 63 patients with EMPD who were surgically treated from 2002 to 2019 at a single institution. The primary endpoint was the 5-year overall survival rate of EMPD, and the secondary endpoint was recurrence-free 5-year survival. Read More

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Tumor Microenvironment Proteomics: Lessons From Multiple Myeloma.

Front Oncol 2021 23;11:563384. Epub 2021 Mar 23.

Department of Experimental and Clinical Oncology, Discipline of Hematology and Hemotherapy, Federal University of São Paulo, UNIFESP, São Paulo, Brazil.

Although the "seed and soil" hypothesis was proposed by Stephen Paget at the end of the 19th century, where he postulated that tumor cells (seeds) need a propitious medium (soil) to be able to establish metastases, only recently the tumor microenvironment started to be more studied in the field of Oncology. Multiple myeloma (MM), a malignancy of plasma cells, can be considered one of the types of cancers where there is more evidence in the literature of the central role that the bone marrow (BM) microenvironment plays, contributing to proliferation, survival, migration, and drug resistance of tumor cells. Despite all advances in the therapeutic arsenal for MM treatment in the last years, the disease remains incurable. Read More

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Robot-assisted transthoracic first rib resection for venous thoracic outlet syndrome.

Vascular 2021 Apr 9:1708538121997332. Epub 2021 Apr 9.

Cardiovascular Sciences, Department of Vascular Surgery, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, Netherlands.

Background: Venous thoracic outlet syndrome (vTOS) is caused by external compression of the subclavian vein at the costoclavicular junction. It can be subdivided in McCleery Syndrome and Paget-Schroetter Syndrome (PSS). To improve the venous outflow of the arm and to prevent recurrent thrombosis, first rib resection with venolysis of the subclavian vein can be performed. Read More

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Acute kidney injury and nephrotic syndrome associated with eltrombopag therapy in chronic idiopathic thrombocytopenic purpura.

BMJ Case Rep 2021 Apr 7;14(4). Epub 2021 Apr 7.

James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, Norfolk, UK.

A 77-year-old man was admitted with severe acute kidney injury and nephrotic syndrome. He was started on eltrombopag for chronic idiopathic thrombocytopenic purpura 6 weeks earlier. An ultrasound of the kidneys was normal and an auto-antibody screen was negative. Read More

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Becker nevus syndrome: A 2020 update.

Authors:
Daniele Torchia

J Am Acad Dermatol 2021 Apr 2. Epub 2021 Apr 2.

Department of Dermatology, James Paget University Hospital, Gorleston-on-Sea, Great Yarmouth, United Kingdom. Electronic address:

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An unusual secondary extramammary Paget's disease of vulva.

Indian J Sex Transm Dis AIDS 2020 Jul-Dec;41(2):210-212. Epub 2020 Nov 11.

Department of OBG, Father Muller Medical College Hospital, Mangalore, Karnataka, India.

Extramammary Paget's disease (EMPD) of the vulva is a rare disease that occurs mainly in postmenopausal women. It can be primary or secondary. Prognosis of primary EMPD is good, but when secondary, we need to find the underlying malignancy as the prognosis depends on it. Read More

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November 2020

New advances in Amyotrophic Lateral Sclerosis genetics: Towards gene therapy opportunities for familial and young cases.

Rev Neurol (Paris) 2021 Mar 30. Epub 2021 Mar 30.

Institut du Cerveau et de la Moelle épinière, ICM, Inserm U1127, CNRS UMR7225, Sorbonne Université, UPMC Univ Paris 6 UMRS1127, 75013 Paris, France. Electronic address:

Due to novel gene therapy opportunities, genetic screening is no longer restricted to familial cases of ALS (FALS) cases but also aplies to the sporadic populations (SALS). Screening of four main genes (C9orf72, SOD1, TARDBP and FUS) identified the causes in 15% of Amyotrophic Lateral Sclerosis (ALS) patients (two third of the familial cases and 8% of the sporadic ones) but their respective contribution to ALS phenotype varies according the age of disease onset. The genetic overlap between ALS and other diseases is expanding and includes frontotemporal dementia, Paget's Disease of Bone, myopathy for adult cases, HSP and CMT for young cases highlighing the importance of retrieving the exhaustive familial history for each indivdual with ALS. Read More

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Aberrant Melan-A Expression in Extramammary Paget Disease.

Am J Dermatopathol 2021 Mar 30. Epub 2021 Mar 30.

Department of Dermatology, UConn Health, Farmington, CT.

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Treatment of Metastatic Extramammary Paget Disease with Combination Ipilimumab and Nivolumab: A Case Report.

Case Rep Oncol 2021 Jan-Apr;14(1):430-438. Epub 2021 Mar 12.

Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA.

Metastatic primary cutaneous extramammary Paget disease (EMPD) is a rare clinical entity with a 5-year survival <10% and no standard therapy. We report the first case to our knowledge of metastatic EMPD with treatment response to checkpoint inhibitor immunotherapy. The patient had diffusely metastatic disease and previously progressed on cytotoxic chemotherapy and a molecularly targeted agent. Read More

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Multifocal nodular steatosis mimicking malignancy.

Postgrad Med J 2021 Mar 29. Epub 2021 Mar 29.

Gastroenterology, James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, UK.

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Utility of photodynamic diagnosis plus reflectance confocal microscopy in detecting the margins of extramammary Paget disease.

Indian J Dermatol Venereol Leprol 2021 Mar-Apr;87(2):207-213

Department of Dermatologic Surgery, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing.

Background: Due to the clinically poorly delineated unclear margin of extramammary Paget disease, the recurrence rate after surgical resection is high.

Aims: To compare photodynamic diagnosis and photodynamic plus reflectance confocal microscopy diagnosis in determining the tumor margins in patients with extramammary Paget disease.

Methods: Thirty-six patients with histopathologically confirmed primary extramammary Paget disease between January 2017 to June 2018 were included in the study. Read More

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January 2020