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https://www.readbyqxmd.com/read/28717292/conservative-management-of-unicystic-ameloblastoma-in-young-patients-a-prospective-single-center-trial-and-review-of-literature
#1
Mitsu Meshram, Lalit Sagarka, Jigar Dhuvad, Sonal Anchlia, Siddharth Vyas, Harsh Shah
AIMS: The study intends to evaluate the clinical outcome of treating young patients of unicystic ameloblastoma, by various conservative treatment modalities such as marsupialization, enucleation with bone curettage and aid in establishing sound treatment guidelines. MATERIALS AND METHODS: A case series of fifteen patients is presented of unicystic ameloblastoma, who presented to our Department over the past 6 years (2010-2015) and conservative method of treatment elected in all the patients...
September 2017: Journal of Maxillofacial and Oral Surgery
https://www.readbyqxmd.com/read/28711573/distribution-and-impact-of-comorbidities-on-survival-and-leukemic-transformation-in-myeloproliferative-neoplasm-associated-myelofibrosis-a-retrospective-cohort-study
#2
Justyna Bartoszko, Tony Panzarella, Caroline Jane McNamara, Anthea Lau, Aaron D Schimmer, Andre C Schuh, Hassan Sibai, Dawn Maze, Karen W L Yee, Rebecca Devlin, Vikas Gupta
BACKGROUND: We sought to describe the distribution and impact of comorbidities on outcomes in patients with myelofibrosis, a disease characterized by aberrant bone marrow function with eventual fibrosis. Comorbidities were scored using the Adult Comorbidity Evaluation-27 (ACE-27) and the Hematopoietic Cell Transplant Comorbidity Index (HCT-CI), in which a score ≥ 3 indicates severe comorbidities. PATIENTS AND METHODS: We conducted a retrospective study of 306 patients with a confirmed diagnosis of myelofibrosis...
June 29, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28702412/megakaryocytic-morphology-in-janus-kinase-2-v617f-positive-myeloproliferative-neoplasm
#3
Shuchi Ghai, Sharada Rai
CONTEXT: Alterations in megakaryocyte morphology are the hallmark of myeloproliferative neoplasms (MPNs). These neoplasm are also associated with Janus kinase 2 (JAK2) V617F mutation in nearly 95% patients with polycythemia vera (PV), 40% patients of essential thrombocythemia (ET) and 50% patients of myelofibrosis (MF). The utility of megakaryocyte morphology in these disorders in correlation with JAK2 V617F remains unresolved. AIMS: The aim of the study was to assess the morphology of megakaryocytes in bone marrow aspirates (BMAs) and bone marrow biopsies of patients of BCR-ABL negative MPNs with JAK2 V617F mutation...
April 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28699668/poems-syndrome-2017-update-on-diagnosis-risk-stratification-and-management
#4
Angela Dispenzieri
DISEASE OVERVIEW: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy...
August 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28697683/metastatic-sclerosing-epithelioid-fibrosarcoma-in-bone-marrow
#5
Rana Asakra, Shane Zaidi, Khin Thway
Sclerosing epithelioid fibrosarcoma (SEF) is an aggressive neoplasm thought to be related to low-grade fibromyxoid sarcoma, which typically occurs in middle-aged adults in the deep soft tissues of the lower extremities and trunk. It comprises nests and cords of relatively uniform epithelioid polygonal cells with clear or eosinophilic cytoplasm in densely sclerotic stroma, and it is typically associated with EWSR1 gene rearrangements, and most commonly EWSR1-CREB3L1 fusions. As primary SEF can arise in bone, and bone is also a common metastatic site for SEF, its recognition at this site is important...
July 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28686765/cutaneous-myoepithelial-carcinoma-with-disseminated-metastases
#6
Gina E Johnson, Keith Stevens, Annie O Morrison, Lauren Stuart, Jerad M Gardner, Mack Rachal, Douglas C Parker
Primary cutaneous neoplasms of myoepithelial differentiation are uncommon. Cutaneous myoepithelial carcinomas are rare. We report a case of cutaneous myoepithelial carcinoma in a 47-year-old man with a history of end-stage renal disease and renal transplant 19 years prior who presented to the hospital with a 3-month history of diffuse bone pain and an ulcerated scalp mass with multiple satellite lesions. This case illustrates a rare instance of metastatic disease from primary cutaneous myoepithelial carcinoma...
June 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28685840/european-leukemianet-study-on-the-reproducibility-of-bone-marrow-features-in-masked-polycythemia-vera-and-differentiation-from-essential-thrombocythemia
#7
Hans Michael Kvasnicka, Attilio Orazi, Juergen Thiele, Giovanni Barosi, Carlos E Bueso-Ramos, Alessandro M Vannucchi, Robert P Hasserjian, Jean-Jacques Kiladjian, Umberto Gianelli, Richard Silver, Tariq I Mughal, Tiziano Barbui
The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data...
July 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28679986/virus-specific-cytotoxic-t-cells-in-chronic-active-epstein-barr-virus-infection
#8
Haruna Shibayama, Ken-Ichi Imadome, Erika Onozawa, Akiho Tsuzura, Osamu Miura, Takatoshi Koyama, Ayako Arai
Chronic active Epstein-Barr virus infection (CAEBV) is a disease characterized by clonally proliferating and activated EBV-infected T or NK cells accompanied by chronic inflammation and T- or NK-cell neoplasms. However, the mechanism for developing CAEBV has not been clarified to date. Because the decreased number or inactivation of EBV-specific cytotoxic T lymphocytes (CTLs) resulted in the development of EBV-positive B-cell neoplasms, we investigated the number of CTLs in CAEBV patients using the tetrameric complexes of HLA-restricted EBV-specific peptides...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28674741/new-tumour-entities-in-the-4th-edition-of-the-world-health-organization-classification-of-head-and-neck-tumours-odontogenic-and-maxillofacial-bone-tumours
#9
REVIEW
Paul M Speight, Takashi Takata
The latest (4th) edition of the World Health Organization Classification of Head and Neck tumours has recently been published with a number of significant changes across all tumour sites. In particular, there has been a major attempt to simplify classifications and to use defining criteria which can be used globally in all situations, avoiding wherever possible the use of complex molecular techniques which may not be affordable or widely available. This review summarises the changes in Chapter 8: Odontogenic and maxillofacial bone lesions...
July 3, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28671156/overview-of-extremity-musculoskeletal-neoplasms-at-the-ahmadu-bello-university-teaching-hospital-zaria-nigeria
#10
Maitama Mohammed Inuwa, Lawal Yau Zakariyau, Dahiru I Ismail, Ejagwulu S Friday, Aniko A Ibrahim, Abdulmalik A Mohammed
BACKGROUND: The burden of managing patients with musculoskeletal neoplasms in the West African sub-region is quite significant. This is largely due to late presentation, delay in making diagnosis, and failure of obtaining consent to certain surgical procedures. Improvements in diagnosis and treatment over the years have however increased life and limb survival for many patients. This study was aimed at evaluating the clinicopathologic pattern of neoplasm as it affects the upper and lower limbs with a view to determining the most common types, the most involved sites of the body and the age at presentation of these tumors...
July 2017: Annals of African Medicine
https://www.readbyqxmd.com/read/28670078/b-lymphoblastic-lymphoma-of-the-rectum
#11
Puthillathu Unnikrishnan, Geetha Narayanan, Bhavya S Kumar, Nandini Devi
Lymphoblastic lymphoma is a neoplasm of immature cells committed to the B-cell or T-cell lineage. B-lymphoblastic lymphoma usually involves lymph nodes and extranodal sites, such as the skin, bone, and soft tissue. The rectum is a very rare site of involvement in B-lymphoblastic lymphoma. We describe a 26-year-old man who presented with bleeding per rectum and fecal incontinence. Endoscopy showed a large nodular friable lesion narrowing the entire rectal lumen. Biopsy was diagnostic of B-lymphoblastic lymphoma...
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28669446/inflammation-and-myeloproliferative-neoplasms
#12
REVIEW
Federico Lussana, Alessandro Rambaldi
Myeloproliferative neoplasms (MPN) include three main entities: Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Myelofibrosis (MF). MPN represent a unique model of the relationship between the clonal development of a hematologic malignancy and chronic inflammation. The neoplastic clone is the main driver of this inflammatory reaction as demonstrated by the curative effect of allogeneic stem cell transplantation which leads not only to a complete restore of the hematopoiesis, but also to regression of bone marrow fibrosis...
June 29, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28667395/-intra-axial-brain-tumors-in-adults-on-the-basis-of-the-2016-who-classification
#13
REVIEW
N Peitgen, P Papanagiotou
CLINICAL/METHODICAL ISSUE: The influence of the World Health Organization (WHO) classification from 2016 on the radiological diagnosis for tumors of the central nervous system (CNS) in adults. STANDARD RADIOLOGICAL METHODS: Computed tomography (CT), magnetic resonance imaging (MRI) and MR spectroscopy. PRACTICAL RECOMMENDATIONS: In order to come as close as possible to the correct diagnosis of CNS tumors, MRI is the long-standing accepted method of choice that can in some cases be supported by the use of CT to demonstrate calcification or bone destruction...
June 30, 2017: Der Radiologe
https://www.readbyqxmd.com/read/28659241/primary-melanoma-of-the-third-metatarsal
#14
Vivek Ajit Singh, Chiao Yee Lim, Ho Choon Yan, Nazarina Abdul Rahman
Melanoma is a well-known malignant neoplasm of the skin, although it can also arise from other structures. Bone metastasis is not an uncommon event associated with melanoma, although primary osseous melanoma is very rare. In the present report, we describe a case of primary melanoma arising from the left third metatarsal in an adult male. The lesion was treated with surgical excision without adjunct chemotherapy, and recurrence developed approximately 12 months after the foot surgery. The patient died of the cancer 34 months after it had been identified...
June 25, 2017: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
https://www.readbyqxmd.com/read/28656936/blastic-plasmacytoid-dendritic-cell-leukemia-in-a-black-malian
#15
Y L Diallo, J Cucherousset, O Samassekou, M Doumbere, Y Cissoko, D T Menta, N Ouologuem, M Ouologuem, M Konate, A T Sidibe
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy, that most commonly manifests as cutaneous lesions. A 19-year-old Malian female was admitted to the Unit of Medicine of Hopital du Mali with anemia, fever, weakness, and weight loss. On physical examination she was wasted, pale, febrile (37.4°C), and had inguinal and axillary lymphadenopathies. The complete blood count found pancytopenia with Hemoglobin level of 4.8 g/dL, Leucocytes count of 1900/μL (neutrophil: 300/μL), and platelets count of 56 000/μL...
June 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28656712/uterine-tumour-resembling-ovarian-sex-cord-tumour-utrosct-first-report-of-a-large-series-with-follow-up
#16
Michelle Moore, W Glenn McCluggage
AIMS: Uterine tumour resembling ovarian sex cord tumour (UTROSCT) is an uncommon mesenchymal neoplasm of uncertain histogenesis. While it is considered a neoplasm of uncertain but low malignant potential, there is limited evidence for this since there are no large studies with follow-up. We aimed to determine the clinical behaviour of this uncommon neoplasm and investigate clinicopathological parameters which predict behaviour. METHODS AND RESULTS: From a series of 34 cases of UTROSCT, mainly from consultation practice, we obtained follow-up information which was obtained by contacting referring pathologists and clinicians...
June 28, 2017: Histopathology
https://www.readbyqxmd.com/read/28643220/denosumab-an-emerging-therapy-in-pediatric-bone-disorders
#17
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
#18
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
#19
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
#20
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
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