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https://www.readbyqxmd.com/read/28643220/denosumab-an-emerging-therapy-in-pediatric-bone-disorders
#1
REVIEW
Alison M Boyce
PURPOSE OF REVIEW: Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand (RANKL), and has emerged as an important novel therapy for skeletal disorders. This article examines the use of denosumab in children. RECENT FINDINGS: Considerable safety and efficacy data exists for denosumab treatment of adults with osteoporosis, bone metastases, and giant cell tumors. Pediatric data is limited; however, evidence suggests denosumab may be beneficial in decreasing bone turnover, increasing bone density, and preventing growth of certain skeletal neoplasms in children...
June 22, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28642604/turning-the-tide-in-myelodysplastic-myeloproliferative-neoplasms
#2
REVIEW
Michael W N Deininger, Jeffrey W Tyner, Eric Solary
Myelodysplastic syndromes/myeloproliferative neoplasms (MDS/MPN) are aggressive myeloid malignancies recognized as a distinct category owing to their unique combination of dysplastic and proliferative features. Although current classification schemes still emphasize morphology and exclusionary criteria, disease-defining somatic mutations and/or germline predisposition alleles are increasingly incorporated into diagnostic algorithms. The developing picture suggests that phenotypes are driven mostly by epigenetic mechanisms that reflect a complex interplay between genotype, physiological processes such as ageing and interactions between malignant haematopoietic cells and the stromal microenvironment of the bone marrow...
June 23, 2017: Nature Reviews. Cancer
https://www.readbyqxmd.com/read/28641898/sclerosing-odontogenic-carcinoma-current-diagnostic-and-management-considerations-concerning-a-most-unusual-neoplasm
#3
REVIEW
M Hanisch, D Baumhoer, S Elges, L F Fröhlich, J Kleinheinz, S Jung
Sclerosing odontogenic carcinoma (SOC) is a primary intraosseous carcinoma of the jaws that has been listed as a separate entity for the first time in the latest version of the World Health Organization classification of Head and Neck Tumours (2017). The aim of this study was to analyse and interpret the existing literature on SOC in the context of a clinical case treated in the authors' department. A systematic search of the PubMed database was performed in accordance with the PRISMA guidelines, yielding nine cases of SOC reported so far...
June 19, 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28641661/-clinical-and-pathologic-features-of-myeloid-sarcoma
#4
Ya-Jun Jiang, Hong-Xia Wang, Wan-Chuan Zhuang, Hao Chen, Chang Zhang, Xiu-Mei Li, Gui-Hua Zhu, Yao He
OBJECTIVE: To explore the clinicopathologic features, differential diagnosis and therapy of myeloid sarcoma. METHODS: The clinical data including clinical manifestations, laboratorial tests, histopathologicical examination, immunohistochemistry and clinical prognosis of 10 patients with myeloid sarcoma were analyzed retrospectively. Among 10 patients, 5 male and 5 female, aged 23 to 71 years old (median = 36 years). RESULTS: 2 cases of myeloid sarcoma were secondary from chronic myeloid leukemia, and 1 cases of myeloid sarcoma occurred after the allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia, and the others lacked the anamnesis of malignancies...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28641100/bruton-s-tyrosine-kinase-btk-as-a-promising-target-in-solid-tumors
#5
REVIEW
J Molina-Cerrillo, T Alonso-Gordoa, P Gajate, E Grande
Bruton's tyrosine kinase (BTK) is a non-receptor intracellular kinase that belongs to the TEC-family tyrosine kinases together with bone marrow-expressed kinase (BMX), redundant-resting lymphocyte kinase (RLK), and IL-2 inducible T-Cell kinase (ITK). All these proteins play a key role in the intracellular signaling of both B and T lymphocytes. Recently, some preclinical data have demonstrated that BTK is present in certain tumor subtypes and in other relevant cells that are contributing to the tumor microenvironment such as dendritic cells, macrophages, myeloid derived suppressor cells and endothelial cells...
June 9, 2017: Cancer Treatment Reviews
https://www.readbyqxmd.com/read/28640953/overview-of-transgenic-mouse-models-of-myeloproliferative-neoplasms-mpns
#6
Andrew Dunbar, Abbas Nazir, Ross Levine
Myeloproliferative neoplasms (MPNs) are a class of hematologic diseases characterized by aberrant proliferation of one or more myeloid lineages and progressive bone marrow fibrosis. In 2005, seminal work by multiple groups identified the JAK2V617F mutation in a significant fraction of MPN patients. Since that time, murine models of JAK2V617F have greatly enhanced the understanding of the role of aberrant JAK-STAT signaling in MPN pathogenesis and have provided an in vivo pre-clinical platform that can be used to develop novel therapies...
June 22, 2017: Current Protocols in Pharmacology
https://www.readbyqxmd.com/read/28640138/an-unusual-presentation-of-multiple-myeloma-with-unilateral-sudden-vision-loss-a-case-report
#7
Pei-Wen Wu, Ta-Jen Lee, Jim-Ray Chen, Chien-Chia Huang
RATIONALE: Plasma cell neoplasms are categorized by neoplastic proliferation of a single clone of plasma cells which produce a monoclonal immunoglobulin. Plasma cell neoplasms can present as a solitary plasmacytoma or as multiple myeloma. Both of them can progress to multiple myeloma. Once a diagnosis of plasmacytoma has been made, thorough examinations should be carried out for identifying the disease entity. PATIENT CONCERNS AND DIAGNOSES: Herein, we describe an extraordinary rare case of multiple myeloma with initial presentation of a left sphenoid neoplasm resulting in left-sided headache and rapid deterioration of visual acuity...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28639881/suspected-lateral-periodontal-cyst-presenting-concurrently-with-canine-acanthomatous-ameloblastoma-in-a-2-year-old-standard-poodle
#8
Jennifer Tjepkema, Cynthia Bell
Lateral periodontal cysts (LPCs) are odontogenic epithelial cysts composed of nonkeratinized epithelial cells that are in the category of developmental cysts, rather than inflammatory cysts. Lateral periodontal cysts are rare in both people and domestic animals; they are associated with vital teeth and located lateral to a tooth root. Lateral periodontal cysts are typically asymptomatic lesions that are characterized radiographically as a unilocular lucency with well-defined corticated borders. Canine acanthomatous ameloblastoma (CAA) is the most common odontogenic neoplasm in dogs and rarely presents as a cystic lesion...
January 1, 2017: Journal of Veterinary Dentistry
https://www.readbyqxmd.com/read/28639598/haematological-profile-of-patients-with-mixed-phenotype-acute-leukaemia-from-a-tertiary-care-centre-of-north-india
#9
Manupriya Sharma, Man Updesh Singh Sachdeva, Parveen Bose, Neelam Varma, Subhash Varma, R K Marwaha, Pankaj Malhotra
BACKGROUND & OBJECTIVES: Mixed-phenotype acute leukaemia (MPAL) is a rare neoplasm with no definite treatment protocols and a distinctly poor outcome. Advancement in polychromatic flow cytometry has made its identification easier. This prospective study was designed to identify cases of MPAL and study their clinical presentation and haematological profile in a tertiary care hospital in north India. METHODS: Ethylenediaminetetraacetic acid (EDTA)-anticoagulated bone marrow aspirate samples of patients diagnosed as acute leukaemia (AL) on the basis of morphology were utilized for immunophenotyping...
February 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/28635683/blastic-plasmacytoid-dendritic-cell-neoplasm-with-pulmonary-involvement-and-atypical-skin-lesion
#10
Cyndi Myrelle da Silva Barros Romão, Cláudio José Dos Santos Júnior, Luiz Arthur Calheiros Leite, Maria Jordana Rocha Gomes Alves, Nathalia Silva Araújo, Anderson F L Castro, Muriel Silva Moura, Vitória Mikaelly da Silva Gomes, Arthur Moacir Costa Sampaio Batinga, João Antonio S Queiroz, Natanael Barbosa Dos Santos
BACKGROUND Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematodermic malignancy neoplasm with highly aggressive course and poor prognosis. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood.  CASE REPORT Here, we report the case of a 51-year-old woman who presented a violaceus skin lesion on the lateral region of the right thigh, weight loss, fever, and lymphadenopathies. Computed tomography (CT) displayed thoracic and abdominal lymph node and alveolar bleeding...
June 21, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28633797/sponge-rubber-revealed-two-years-after-penetrating-injury-a%C3%A2-case%C3%A2-report
#11
Sandor Roth, Maja Zaninovic, Aron Roth
The present report illustrates a very rare case report of a foreign body granuloma of the foot associated with a 2-year period of a retained spongeous rubber foreign body after a penetrating injury. Because it appeared almost 2 years after the injury, the history of trauma was minimized, the foreign body had migrated from the plantar to the dorsal side of the foot, and the radiographic appearance resembled a malignant process, it was difficult to rule out the possibility of a malignant tumor. This case shows the weakness of magnetic resonance imaging in detecting spongeous rubber in the body...
July 2017: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
https://www.readbyqxmd.com/read/28631687/-acquired-atresia-and-stenosis-of-the-external-acoustic-meatus
#12
D S Kondratchikov, Kh M Diab, V S Korvyakov, L I Terekhina
Acquired atresia of the external acoustic meatus is a rare pathological condition characterized by obliteration of the medial part of the external acoustic canal by a soft fibrous plug. The present article presents an overview of a series of cases of acquired atresia and stenosis of the external acoustic meatus with the description of etiology, pathogenesis, and methods for the treatment of this condition. In the majority of the cases, atresia develops at the final stage of granulation external otitis with or without an accompanying dermatological pathology...
2017: Vestnik Otorinolaringologii
https://www.readbyqxmd.com/read/28627100/subcutaneous-extraskeletal-osteosarcoma-in-a-metatarsal-footpad-in-a-cat
#13
Ramón Almela, Wolf von Bomhard, Agustina Ansón, Ursula Mayer
BACKGROUND: Extraskeletal osteosarcomas (ESOSAs) are rare neoplasms in humans and animals. In cats, ESOSA has been reported to arise from orbital, ocular, intestinal, mammary and subcutaneous locations. Subcutaneous ESOSA occurs most commonly at sites used for vaccination including interscapular, dorsal lumbar or thigh areas. Previous reports of feline cases have not documented the use of advanced diagnostic imaging to exclude a primary bone tumour. OBJECTIVE: To describe the clinicopathological and advanced imaging findings of a subcutaneous ESOSA occurring in a metatarsal footpad of a cat and to report the one year follow-up status...
June 18, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28626633/mediastinal-hemangioendothelioma-case-report-and-review-of-the-literature
#14
Davide Patrini, Laura Scolamiero, Reena Khiroya, David Lawrence, Elaine Borg, Martin Hayward, Nikolaos Panagiotopoulos
BACKGROUND: Epithelioid haemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that can arise in the lung, liver, soft tissues or, less commonly, bone. Due to its low prevalence of less than one in a million and its non-specific clinical features, EHE is often misdiagnosed and managed inappropriately. Here we discuss the case of a 58 year-old gentleman with mediastinal EHE and review existing literature on pulmonary EHE (PEH). CASE HISTORY: A 58 year-old gentleman presented to our outpatient Clinic with chest discomfort and palpitations...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28626490/soft-tissue-sarcomas-in-the-head-and-neck-25-years-of-experience
#15
Juan Francisco Liuzzi, Maribel Da Cunha, Daniuska Salas, Saul Siso, Esteban Garriga
Sarcomas are infrequent and heterogeneous tumours. They represent 1-2% of all malignant neoplasms in adults and between 4% and 10% of head and neck cancers. METHODS: The research was retrospective, descriptive, and cross-sectional. RESULTS: A study population of 62 patients with a mean age of 44 years was obtained; the most frequent location was the soft tissues of the neck (25.3%) and the mean tumour size was 7.1 cm; the most frequent diagnosis was undifferentiated pleomorphic sarcoma (25...
2017: Ecancermedicalscience
https://www.readbyqxmd.com/read/28623913/aggressive-natural-killer-cell-leukemia-or-extranodal-nk-t-cell-lymphoma-a-case-with-nasal-involvement
#16
Xiaoke Jin, Youhai Xu, Jun Zhang, Guangxi Li, Dongping Huang, Yuqiong Yang, Hesheng He
BACKGROUND: Aggressive natural killer cell leukemia/lymphoma (ANKL) is a rare and highly aggressive NK cell neoplasm with a short clinical course and poor prognosis and is often misdiagnosed and confused with NK/T cell lymphoma (NKTL), which has a very different prognosis. Here, we present a case with nasal and bone marrow involvement, provide a literature review and make a differential diagnosis. CASE PRESENTATION: A 41-year-old male presented nasal congestion pharyngalgia, palatal perforation, high fever and multiorgan dysfunction...
June 17, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28622153/extraskeletal-osteosarcoma-outcomes-and-the-role-of-chemotherapy
#17
Jonas Paludo, Karen Fritchie, Candace L Haddox, Peter S Rose, Carola A S Arndt, Randolph S Marks, Evanthia Galanis, Scott H Okuno, Steven I Robinson
OBJECTIVES: Extraskeletal osteosarcoma (EO) is a malignant neoplasm that produces osteoid, bone, and chondroid material without direct attachment to bone or periosteum. Surgical resection is the mainstay of treatment; the role of chemotherapy is not well defined. Therefore, we evaluated the impact of chemotherapy in the survival of patients with EO. METHODS: All EO patients seen at Mayo Clinic between 1990 and 2014 were assessed. Forty-three patients were included after all archived pathology slides were reviewed to confirm the diagnosis of EO...
June 15, 2017: American Journal of Clinical Oncology
https://www.readbyqxmd.com/read/28614212/phosphaturic-mesenchymal-tumors-clinicopathologic-immunohistochemical-and-molecular-analysis-of-22-cases-expanding-their-morphologic-and-immunophenotypic-spectrum
#18
Abbas Agaimy, Michael Michal, Simion Chiosea, Fredrik Petersson, Ladislav Hadravsky, Glenn Kristiansen, Raymund E Horch, Jan Schmolders, Arndt Hartmann, Florian Haller, Michal Michal
Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm of uncertain histogenesis that has been linked to tumor-induced osteomalacia (TIO) since 1959. The neoplastic cells produce increased amount of FGF23 which results in TIO via uncontrolled renal loss of phosphate (phosphaturia), and consequently diminished bone mineralization. To date, ∼300 cases have been reported. Although there is increasing evidence that PMT can be diagnosed by reproducible histopathologic features, firm diagnosis has been often restricted to cases associated with TIO and, hence, diagnosis of "nonphosphaturic variants" remained challenging...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28611040/%C3%AE-catenin-is-a-candidate-therapeutic-target-for-myeloid-neoplasms-with-del-5q
#19
Liping Li, Yue Sheng, Wenshu Li, Chao Hu, Nupur Mittal, Kaoru Tohyama, Amber Seba, Youyang Zhao, Howard Ozer, Tongyu Zhu, Zhijian Qian
Deletion of the chromosome 5q [del(5q)] is one of the most common cytogenetic abnormalities observed in patients with de novo myelodysplastic syndromes (MDS) and therapy-related MDS or acute myeloid leukemia (t-MDS/tAML). Emerging evidence indicates that activation of the Wnt/β-catenin pathway contributes to the development of myeloid neoplasms with del(5q). Whether β-catenin is a potential therapeutic target for myeloid neoplasms with del(5q) has yet to be evaluated. Here we report that genetic deletion of a single allele of β-catenin rescues ineffective hematopoiesis in an Apc haploinsufficient mouse model, which recapitulates several characteristic features of the pre-leukemic stage of myeloid neoplasms with a -5/del(5q)...
June 13, 2017: Cancer Research
https://www.readbyqxmd.com/read/28602585/janus-kinase-2-inhibitor-fedratinib-in-patients-with-myelofibrosis-previously-treated-with-ruxolitinib-jakarta-2-a-single-arm-open-label-non-randomised-phase-2-multicentre-study
#20
Claire N Harrison, Nicolaas Schaap, Alessandro M Vannucchi, Jean-Jacques Kiladjian, Ramon V Tiu, Pierre Zachee, Eric Jourdan, Elliott Winton, Richard T Silver, Harry C Schouten, Francesco Passamonti, Sonja Zweegman, Moshe Talpaz, Joanne Lager, Zhenming Shun, Ruben A Mesa
BACKGROUND: Myelofibrosis is a chronic myeloproliferative neoplasm characterised by splenomegaly, cytopenias, bone marrow fibrosis, and debilitating symptoms including fatigue, weight loss, and bone pain. Mutations in Janus kinase-2 (JAK2) occur in approximately 50% of patients. The only approved JAK2 inhibitor for myelofibrosis is the dual JAK1 and JAK2 inhibitor, ruxolitinib. 58-71% of patients treated with ruxolitinib in clinical trials so far have not achieved the primary endpoint of 35% or more reduction in spleen volume from baseline assessed by MRI or CT...
June 8, 2017: Lancet Haematology
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