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https://www.readbyqxmd.com/read/29332607/the-role-of-t2-weighted-gradient-echo-in-the-diagnosis-of-tumefactive-intrahepatic-extramedullary-hematopoiesis-in-myelodysplastic-syndrome-and-diffuse-hepatic-iron-overload-a-case-report-and-review-of-the-literature
#1
Abel A Belay, Andrew M Bellizzi, Alan H Stolpen
BACKGROUND: Extramedullary hematopoiesis is the proliferation of hematopoietic cells outside bone marrow secondary to marrow hematopoiesis failure. Extramedullary hematopoiesis rarely presents as a mass-forming hepatic lesion; in this case, imaging-based differentiation from primary and metastatic hepatic neoplasms is difficult, often leading to biopsy for definitive diagnosis. We report a case of tumefactive hepatic extramedullary hematopoiesis in the setting of myelodysplastic syndrome with concurrent hepatic iron overload, and the role of T2*-weighted gradient-echo magnetic resonance imaging in differentiating extramedullary hematopoiesis from primary and metastatic hepatic lesions...
January 15, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29327708/myeloproliferative-neoplasms-with-concurrent-bcr-abl1-translocation-and-jak2-v617f-mutation-a-multi-institutional-study-from-the-bone-marrow-pathology-group
#2
Craig R Soderquist, Mark D Ewalt, David R Czuchlewski, Julia T Geyer, Heesun J Rogers, Eric D Hsi, Sa A Wang, Carlos E Bueso-Ramos, Attilio Orazi, Daniel A Arber, Elizabeth O Hexner, Daria V Babushok, Adam Bagg
Myeloproliferative neoplasms arise from hematopoietic stem cells with somatically altered tyrosine kinase signaling. Classification of myeloproliferative neoplasms is based on hematologic, histopathologic and molecular characteristics including the presence of the BCR-ABL1 and JAK2 V617F. Although thought to be mutually exclusive, a number of cases with co-occurring BCR-ABL1 and JAK2 V617F have been identified. To characterize the clinicopathologic features of myeloproliferative neoplasms with concomitant BCR-ABL1 and JAK2 V617F, and define the frequency of co-occurrence, we conducted a retrospective multi-institutional study...
January 12, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29325354/-excision-of-giant-desmoid-in-the-abdominal-wall-method-of-abdominal-wall-reconstruction-and-follow-up-of-long-termed-effect
#3
Y Fei, J Y Li
Objective: To explore the ideal procedure of excision and repair for giant desmoid in the abdominal wall and long-termed follow-up results. Methods: Clinical and follow-up data of 24 patients with giant desmoid in the abdominal wall underwent radical removal and immediate abdominal wall reconstruction in Diagnostic and Therapeutic Center of Hernia and Abdominal Wall Diseases, First Affiliated Hospital of People's Liberation Army General Hospital from October 2006 to October 2016 were analyzed retrospectively...
January 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29323085/cd19-negative-b-lineage-acute-lymphoblastic-leukemia-a-diagnostic-and-therapeutic-challenge
#4
Shashi Bansal, Upendra Sharma, Akansha Jain, Richa Sharma, Bhargav Yagnik
B-lineage acute lymphoblastic leukemia (B-ALL) is an aggressive neoplasm of B-lymphocyte precursors that express the pan B-cell marker CD19 in all the cases. Rarely, a case may be assigned as B-lineage even if CD19 is negative. Here, a 16-year-old male presented with complaints of pain abdomen, on and off fever, joint pain, and hepatosplenomegaly for 2 months. Bone marrow examination was suggestive of acute leukemia with numerous leukoblasts on aspiration. On flow cytometry, gated blast population was negative for CD19, cytoCD3, and myeloperoxidase MPO and positive for CD34, TdT, HLA-DR, CD22, CD79a, and CD10...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29323084/fibroblast-growth-factor-receptor-1-associated-myeloproliferative-neoplasm-and-t-lymphoblastic-lymphoma
#5
Gayathri Gopan, T M Anoop, N P Prakash, Rakul Nambiar, R Krishnachandran
Myeloid and lymphoid hematological malignancies with eosinophilia and abnormalities of fibroblast growth factor receptor-1 (FGFR1) result from the formation of abnormal fusion genes that encode constitutively activated tyrosine kinases. The WHO classification (2008) of hematolymphoid neoplasms recognizes a category of myeloid and lymphoid neoplasms with eosinophilia and abnormalities of FGFR1. Here, we present the case of a 30-year-old-woman who was diagnosed with T-lymphoblastic lymphoma from lymph node biopsy and myeloproliferative neoplasm with eosinophilia from bone marrow studies...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29316907/expression-of-mmp-9-decreases-metastatic-potential-of-chondrosarcoma-an-immunohistochemical-study
#6
Dominik Malcherczyk, Thomas J Heyse, Bilal F El-Zayat, Vanessa Kunzke, Roland Moll, Susanne Fuchs-Winkelmann, Jürgen R J Paletta
BACKGROUND: Chondrosarcoma is the second most common primary malignant bone tumor. Because of their heterogeneity, with differences in invasive and metastatic behavior, it is important to identify biological markers that will allow for a more accurate estimation of prognosis in patients with these tumors. Matrix metalloproteinases (MMP) play a crucial role in tumor progression, invasion and metastasis. The mechanism of tumor progression dependent of MMPs is complex and influences malignant transformation, angiogenesis and tumor growth at the primary and metastatic sites...
January 9, 2018: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/29314691/mastocytosis-pathogenesis-clinical-manifestation-and-treatment
#7
Nicola Wagner, Petra Staubach
The term mastocytosis designates a group of rare disorders characterized by typical skin lesions, frequently associated episodes of anaphylaxis, and clinical symptoms related to the release of various mediators. Dermatologists/allergists are frequently the first to establish the diagnosis. The condition is based on clonal mast cell proliferation, usually in the skin or bone marrow and only rarely in the gastrointestinal tract or other tissues. In general, mastocytosis has a good prognosis in terms of life expectancy...
January 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/29314186/aggressive-nk-cell-leukaemia-and-extranodal-nk-t-cell-lymphoma-are-two-distinct-diseases-that-differ-in-their-clinical-presentation-and-cytogenetic-findings
#8
Ching-Fen Yang, Chih-Yi Hsu, Donald Ming-Tak Ho
AIMS: Distinguishing between aggressive NK-cell leukaemia (ANKCL) and extranodal NK/T-cell lymphoma (ENKTCL) with secondary bone marrow involvement is challenging, as they are rare bone marrow NK/T-cell neoplasms and share similar features. METHODS AND RESULTS: We studied bone marrow NK/T-cell neoplasms by classifying them into those with no extramedullary mass (group 1, 8 cases) and those with extramedullary mass (group 2, 13 cases). Both groups showed similar clinical presentations and pathological features...
January 4, 2018: Histopathology
https://www.readbyqxmd.com/read/29312715/multidisciplinary-approach-to-chest-wall-resection-and-reconstruction-for-chest-wall-tumors-a-single-center-experience
#9
Elisa Scarnecchia, Valeria Liparulo, Alessandra Pica, Giuseppe Guarro, Carmine Alfano, Francesco Puma
Background: Chest wall resection and reconstruction (CWRR) is quite challenging in surgery, due to evolution in techniques. Neoplasms of the chest wall, primary or secondary, have been considered inoperable for a long time. Thanks to evolving surgical techniques, reconstruction after extensive chest wall resection is possible with good functional and aesthetic results. Methods: In our single-center experience, seven cases of extensive CWRR for tumors were performed with a multidisciplinary approach by both thoracic and plastic surgeons...
December 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29311096/early-detection-and-evolution-of-pre-leukemic-clones-in-therapy-related-myeloid-neoplasms-following-autologous-sct
#10
Gerbrig Berger, Leonie I Kroeze, Theresia N Koorenhof-Scheele, Aniek O de Graaf, Kenichi Yoshida, Hiroo Ueno, Yuichi Shiraishi, Satoru Miyano, Eva van den Berg, Hein Schepers, Bert A van der Reijden, Seishi Ogawa, Edo Vellenga, Joop H Jansen
Therapy-related myeloid neoplasms (tMNs) are severe adverse events that can occur following treatment with autologous hematopoietic stem cell transplantation (ASCT). This study aimed to investigate the development of tMN following ASCT at the molecular level by whole exome sequencing (WES) and targeted deep sequencing (TDS) in sequential (pre-) tMN samples. WES identified a significantly higher number of mutations in tMN as compared to de novo MDS (median 27 vs 12, p=0.001). The mutations found in tMN did not carry a clear ageing-signature, unlike the mutations found in de novo MDS, indicating a different mutational mechanism...
January 8, 2018: Blood
https://www.readbyqxmd.com/read/29310833/myeloid-neoplasm-with-eosinophilia-associated-with-isolated-extramedullary-fip1l1-pdgfra-rearrangement
#11
Talal Hilal, Veena Fauble, Rhett P Ketterling, Katalin Kelemen
Myeloid neoplasms with eosinophilia associated with PDGFRA rearrangement are very responsive to tyrosine kinase inhibitors (TKIs). Herein, we report a case of a 53-year-old man with eosinophilia and a well-differentiated extramedullary myeloid tumor with evidence of FIP1L1/PDGFRA rearrangement by fluorescent in situ hybridization in the extramedullary tissue. His bone marrow evaluation revealed a hypercellular marrow with eosinophilia but without evidence of a FIP1L1/PDGFRA rearrangement. The patient was treated with imatinib at a dose of 100 mg daily and responded with normalization of his peripheral eosinophil count...
January 2018: Cancer Genetics
https://www.readbyqxmd.com/read/29296745/copy-number-alterations-detected-as-clonal-hematopoiesis-of-indeterminate-potential
#12
Koichi Takahashi, Feng Wang, Hagop Kantarjian, Xingzhi Song, Keyur Patel, Sattva Neelapu, Curtis Gumbs, Latasha Little, Samantha Tippen, Rebecca Thornton, Courtney D DiNardo, Farhad Ravandi, Carlos Bueso-Ramos, Jianhua Zhang, Xifeng Wu, Guillermo Garcia-Manero, P Andrew Futreal
Recent studies have revealed that clonal hematopoiesis of indeterminate potential (CHIP) is an important risk factor for therapy-related myeloid neoplasms (t-MNs). CHIP is currently defined as a clonal hematopoietic population carrying somatic point mutations in 1 of the leukemia-associated genes. Patients with t-MNs often present with chromosomal abnormalities in addition to somatic point mutations. It remains unclear whether chromosomal abnormalities can cooccur with point mutations as part of CHIP. Here we report that 3 of 14 patients with t-MNs had low amplitude but detectable chromosome arm-level copy number alterations (CNAs) in the peripheral blood samples that were taken at the time of their primary cancer diagnosis and before exposure to therapy...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296743/hmga2-collaborates-with-jak2v617f-in-the-development-of-myeloproliferative-neoplasms
#13
Koki Ueda, Kazuhiko Ikeda, Takayuki Ikezoe, Kayo Harada-Shirado, Kazuei Ogawa, Yuko Hashimoto, Takahiro Sano, Hiroshi Ohkawara, Satoshi Kimura, Akiko Shichishima-Nakamura, Yuichi Nakamura, Yayoi Shikama, Tsutomu Mori, Philip J Mason, Monica Bessler, Soji Morishita, Norio Komatsu, Kotaro Shide, Kazuya Shimoda, Shuhei Koide, Kazumasa Aoyama, Motohiko Oshima, Atsushi Iwama, Yasuchika Takeishi
High-mobility group AT-hook 2 (HMGA2) is crucial for the self-renewal of fetal hematopoietic stem cells (HSCs) but is downregulated in adult HSCs via repression by MIRlet-7 and the polycomb-recessive complex 2 (PRC2) including EZH2. The HMGA2 messenger RNA (mRNA) level is often elevated in patients with myelofibrosis that exhibits an advanced myeloproliferative neoplasm (MPN) subtype, and deletion of Ezh2 promotes the progression of severe myelofibrosis in JAK2V617F mice with upregulation of several oncogenes such as Hmga2...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29291204/embryonal-rhabdomyosarcoma-in-the-maxillary-sinus-with-orbital-involvement-in-a-pediatric-patient-case-report
#14
Ana Carolina Rodrigues de Melo, Tácio Candeia Lyra, Isabella Lima Arrais Ribeiro, Alexandre Rolim da Paz, Paulo Rogério Ferreti Bonan, Ricardo Dias de Castro, Ana Maria Gondim Valença
This report presents a case of embryonal rhabdomyosarcoma (eRMS) located in the left maxillary sinus and invading the orbital cavity in a ten-year-old male patient who was treated at a referral hospital. The images provided from the computed tomography showed a heterogeneous mass with soft-tissue density, occupying part of the left half of the face inside the maxillary sinus, and infiltrating and destroying the bone structure of the maxillary sinus, left orbit, ethmoidal cells, nasal cavity, and sphenoid sinus...
December 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/29290596/management-of-older-adults-with-myelodysplastic-syndromes-mds
#15
REVIEW
Marlise R Luskin, Gregory A Abel
The myelodysplastic syndromes (MDS) are a varied group of hematologic neoplasms that lead to bone marrow failure, and also carry a risk of progression to acute myeloid leukemia. Patients with MDS suffer significant impairments to both their quality of life and survival. Age is the dominant risk factor for the development of MDS, with a median age at diagnosis over 70years. Consequently, patients with MDS frequently have concurrent comorbidities and/or frailty which may be coincident or related to the disease itself...
December 28, 2017: Journal of Geriatric Oncology
https://www.readbyqxmd.com/read/29290072/operations-for-suspected-neoplasms-in-a-resource-limited-setting-experience-and-challenges-in-the-eastern-democratic-of-congo
#16
Luc Malemo Kalisya, Jacques Fadhili Bake, Richard Bigabwa, David H Rothstein, Sarah B Cairo
INTRODUCTION: Surgery is an essential component of a functional health system, with surgical conditions accounting for nearly 11-15% of world disability. While communicable diseases continue to burden low- and low-middle-income countries, non-communicable diseases, such as cancer, are an important cause of morbidity and mortality worldwide. Preliminary data on malignancies in low- and middle-income countries, specifically in Africa, suggest a higher mortality compared to other regions of the world, a difference partially explained by limited availability of screening and early detection systems as well as poorer access to treatment...
December 31, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/29288421/first-line-treatment-with-bendamustine-and-rituximab-in-patients-with-intermediate-high-risk-splenic-marginal-zone-lymphomas
#17
Roberto Castelli, Luigi Bergamaschini, Giorgio Lambertenghi Deliliers
Splenic marginal zone lymphomas (SMZLs) are rare indolent B cell neoplasms that affect the spleen, bone marrow, and blood. Although they have an indolent course in the majority of patients, who have a median survival of 8-10 years, ∼ 30% may experience a worse outcome. The prognostic criteria of progression are lymph node and extra-nodal involvement, high lymphocyte counts, anaemia, and thrombocytopenia. The treatment of SMZLs include a "wait and watch strategy", splenectomy, and alkylating agents ± rituximab...
December 29, 2017: Medical Oncology
https://www.readbyqxmd.com/read/29284372/histologic-grade-does-not-predict-outcome-in-dogs-with-appendicular-osteosarcoma-receiving-the-standard-of-care
#18
Courtney R Schott, Laetitia J Tatiersky, Robert A Foster, Geoffrey A Wood
Canine appendicular osteosarcoma is an aggressive bone neoplasm that imposes a short survival time. There are several published histologic grading systems for canine osteosarcoma but no universally accepted system. Location within the skeleton and therapy received are both correlated with survival time, but these factors were not always considered when the prognostic value of published grading systems was determined. Our objective was to compare 2 published histologic grading systems in a population of dogs with appendicular osteosarcoma treated with the standard of care for curative intent...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/29283939/rabdomyosarcoma-of-the-mandible-an-uncommon-clinical-presentation
#19
Cleverton Roberto de Andrade, Guilherme Dos Santos Trento, Fabiano Jeremias, Elisa Maria Aparecida Giro, Marisa Aparecida Cabrini Gabrielli, Mario Francisco Real Gabrielli, Oslei Paes de Almeida, Valfrido Antonio Pereira-Filho
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents. Most patients present with a mass in the head and neck region, urogenital region, or with distal extremity involvement. The authors describe a challenging case of a 6-year-old male patient presenting with mandibular RMS. The clinical/radiographic/tomographic evaluations classified the tumor as an advanced stage (stage IV), with a mass of 6.0 cm involving the left side of the mandible and parotid region. The biopsy revealed round, spindled, and pleomorphic cells with hyperchromatic nuclei and rare larger rhabdomyoblasts with eosinophilic cytoplasm...
December 27, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29283383/targeted-%C3%AE-therapies-for-the-treatment-of-bone-metastases
#20
REVIEW
Fable Zustovich, Roberto Barsanti
The skeleton is the target tissue for many types of tumors, and, recently, the survival of patients with prostate cancer metastasis has been increased using α-emitting drugs known as targeted α therapies. The use of α-radiopharmaceuticals in medicine was hypothesized at the beginning of the nineteenth century after the observation that α-radionuclides were associated with high cell-killing energy and low tissue penetration in healthy tissues. In the prostate cancer (PC) scenario, current research suggests that this class of radiopharmaceuticals has limited toxicity, and that the mechanism of action does not overlap with pre-existing drugs, allowing us to extend therapeutic armaments and address medical oncology towards personalized and precision medicine...
December 28, 2017: International Journal of Molecular Sciences
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