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https://www.readbyqxmd.com/read/29145678/-thrombocytosis-and-thrombocytopenia-background-and-clinical-relevance
#1
Kai Wille, Parvis Sadjadian, Martin Griesshammer
Due to the central role of platelets in hemostasis, the clinical relevance of quantitative changes in platelet counts (< 150 G/l or > 450 G/l) may be significant. Thrombopoesis (= production of platelets) occurs in the bone marrow, and the hormone thrombopoetin takes control on its regulation.In thrombocytosis, primary causes have to be distinguished from the far more common reactive (= secondary) reasons. The most important form of primary thrombocytosis occurs in myeloproliferative neoplasms especially in essential thrombocythemia (ET)...
November 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29144072/relapse-and-cytogenetic-evolution-in-myeloid-neoplasms
#2
Natalie Ertz-Archambault, Katalin Kelemen
Based on the current WHO Classification of Myeloid Neoplasms, cytogenetic findings play a central role in the diagnostic classification of the myeloid malignancies. Cytogenetic abnormalities detected at primary diagnosis may change over time. Karyotype changes can be characterized as cytogenetic evolution, cytogenetic regression or a combination of both. While the exact mechanism of cytogenetic evolution is not completely understood, the process of cytogenetic evolution is not random, but follows different, and often disease-specific patterns during progression and relapse of myeloid neoplasms...
December 2017: Panminerva Medica
https://www.readbyqxmd.com/read/29142790/an-active-giant-cell-tumor-of-the-patella-a-case-report
#3
Svetoslav A Slavchev, Georgi P Georgiev, Kircho Patrikov
Primary neoplasms of the patella account for less than 1% of all primary bone tumors of the lower extremity, the most frequent of them being the giant cell tumor of bone, the chondroblastoma, and the aneurysmal bone cyst. Herein, we report the case of a 29-year-old woman with an active giant cell tumor of the patella (GCTP) with its clinical and radiological features and a brief review of the literature.
September 2, 2017: Curēus
https://www.readbyqxmd.com/read/29136691/-tumors-of-lymphoid-and-hematopoietic-tissue-of-spleen-a-clinicopathologic-analysis-of-53-cases
#4
D B Chen, D H Shen, H Zhang, Y Wang, Q J Song, S M Yang, X Z Fang
Objective: To study the clinicopathologic features, diagnosis and differential diagnosis of the tumors of lymphoidand hematopoietic tissue of the spleen(TLTS). Methods: Fifty-three cases of TLTS were selected from the pathologic files from Peking University People's Hospital from April 2002 to April 2017. According to WHO classification of tumors of hematopoietic and lymphoid tissues (2008) and its updated classification (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination...
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29129488/dna-hypomethylating-agents-as-epigenetic-therapy-before-and-after-allogeneic-hematopoietic-stem-cell-transplantation-in-myelodysplastic-syndromes-and-juvenile-myelomonocytic-leukemia
#5
REVIEW
Christian Flotho, Sebastian Sommer, Michael Lübbert
Myelodysplastic syndrome (MDS) is a clonal bone marrow disorder, typically of older adults, which is characterized by ineffective hematopoiesis, peripheral blood cytopenias and risk of progression to acute myeloid leukemia. Juvenile myelomonocytic leukemia (JMML) is an aggressive myeloproliferative neoplasm occurring in young children. The common denominator of these malignant myeloid disorders is the limited benefit of conventional chemotherapy and a particular responsiveness to epigenetic therapy with the DNA-hypomethylating agents 5-azacytidine (azacitidine) or decitabine...
November 9, 2017: Seminars in Cancer Biology
https://www.readbyqxmd.com/read/29129340/synchronous-colorectal-cancer-and-multiple-myeloma-with-chest-wall-involvement-is-this-a-coincidence
#6
Qiu-Li Li, Jin-An Ma, Hai-Peng Li, Ren-Bing Huang, Chun-Hong Hu, Xian-Ling Liu, Ya-Wen Gao, Gang-Hua Feng, Fang Wu
Multiple primary malignant neoplasms (MPMNs) are rare malignant neoplasms that simultaneously or successively occur in the same patient as 2 or more primary malignancies. Currently, an increasing number of cases are being reported. In general, MPMNs more commonly occur as 2 solid tumors or 2 hematological malignancies. Cases of MPMN that involve a solid tumor and a hematological malignancy are rare. Here, we report a case of synchronous colorectal cancer (CRC) and multiple myeloma (MM) with chest wall involvement...
September 25, 2017: Current Problems in Cancer
https://www.readbyqxmd.com/read/29128070/flow-cytometric-assessment-of-chronic-myeloid-neoplasms
#7
REVIEW
Min Shi, Phuong Nguyen, Dragan Jevremovic
Flow cytometry immunophenotyping of the hematopoietic cells from the bone marrow can help with diagnosis, prognosis, and therapy of chronic myeloid neoplasms. Unlike with B-cell neoplasms, there is no simple phenotypic test to substitute for clonality. Therefore, antigen panels to evaluate myeloid neoplasms are larger, and the gating strategies more complex than for lymphoid neoplasms. The number of phenotypic abnormalities in hematopoietic cells correlates with disease severity and cytogenetic complexity, and can be integrated into a scoring system for diagnostic and prognostic purposes...
December 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/29125038/melanotic-neuroectodermal-tumour-of-infancy-a-case-report-and-differential-diagnosis
#8
A P Tan, Thomas S Jacques, Kshitij Mankad, Gregory James, Owase Jeelani, Olga Slater, Felice D'Arco
Melanotic neuroectodermal tumour of infancy is an uncommon pigmented neoplasm of neural crest origin. It was first described in 1918 by Krompecher, known as congenital melanocarcinoma at that time. Although it is generally agreed upon that it is a benign entity, it is locally aggressive and has a significant recurrent risk, reported to be between 10-15%. There have also been prior reports of malignant behaviour in these tumours, although extremely rare. The majority of cases of this tumour (about 70%) arise from the maxilla and its occurrence in the cranial vault represents approximately 15...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/29119232/histopathological-markers-of-treatment-response-and-recurrence-risk-in-ovarian-cancers-and-borderline-tumors
#9
REVIEW
S Avril
Histopathology plays an important role in defining response to treatment for different tumor types. Histopathologic response criteria are currently used as reference standard in various types of cancer, including breast cancer, gastroesophageal cancer, and bone tumors. Since there were no generally accepted response criteria established for ovarian cancer, a systematic analysis of various features of tumor regression was performed. Patient survival served as the reference standard to validate the histopathologic features of tumor regression...
November 8, 2017: Der Pathologe
https://www.readbyqxmd.com/read/29110879/a-diagnostic-approach-to-bone-tumours
#10
REVIEW
Adrienne M Flanagan, Daniel Lindsay
In this review we discuss an approach to diagnosing primary bone tumours, namely the cartilaginous, bone-forming, giant cell-rich, fibro-osseous and round cell neoplasms. Less common lesions including chordoma are also discussed. The value of integrating clinical, histopathological and relevant radiological features is emphasised with a view to providing the general histopathologist with a methodical approach to reaching an accurate diagnosis.
October 27, 2017: Pathology
https://www.readbyqxmd.com/read/29109062/dural-penetration-of-cavernous-hemangioma-on-skull-an-uncommon-clinical-presentation
#11
Hyun-Seung Ryu, Kyung-Sub Moon, Kyung-Hwa Lee, In-Young Kim, Shin Jung
Cavernous hemangioma (CH) of bone is a rare, benign neoplasm and usually found in the vertebral body. This tumor rarely develops in the calvaria, with predilection to occur in the frontal and temporo-parietal bones. A 56-year-old man with a right frontal palpable mass was admitted to our hospital. Magnetic resonance imaging (MRI) revealed a large extra-axial tumor in right frontal bone. Intraoperatively, the bony mass involved the multilayers composed of extracranium-skull-dura-intradura-cortex. Pathological examination revealed a typical calvarial CH penetrating dura mater...
November 3, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29107667/fgfr1-translocation-with-concurrent-myeloproliferative-neoplasm-systemic-mastocytosis-and-lymphoblastic-lymphoma-a-case-report
#12
Koping Chang, Jia-Hau Liu, Shan-Chi Yu, Chung-Wu Lin
FGFR1 translocation may cause myeloid or lymphoid neoplasm but rarely systemic mastocytosis (SM). Conversely, SM is associated with myeloproliferative neoplasm (MPN), but rarely lymphoblastic lymphoma (LBL) or FGFR1 translocation. We report the first case of FGFR1 translocation in a patient with concurrent LBL, MPN, and SM. A 21-year-old male patient presented with diffuse lymphadenopathies and leukocytosis. TdT(+)/cytoCD3(+)/CD79a(weakly+) LBL was identified in the lymph node. Bone marrow had MPN, SM, and TdT(+)/CD79a(+)/cytoCD3(weakly+) LBL...
October 28, 2017: Human Pathology
https://www.readbyqxmd.com/read/29107666/jak2-mutated-langerhans-cell-histiocytosis-associated-with-primary-myelofibrosis-treated-with-ruxolitinib
#13
Arturo Bonometti, Filippo Bagnoli, Daniele Fanoni, Luigia Venegoni, Laura Corti, Paola Bianchi, Elena Maria Elli, Giuseppe Isimbaldi, Vincenzo L'Imperio, Gianluca Nazzaro, Emanuela Passoni, Emilio Berti
The pathogenesis and cellular origin of Langerhans cell histiocytosis (LCH) are debated. Recently, mutations on MAPK and PI3K pathways have been linked to disrupted cell proliferation in LCH. Janus Kinase 2 (JAK2) mutations play the same role in Philadelphia-negative chronic myeloproliferative neoplasms. We describe the case of a patient affected by JAK2-positive Primary Myelofibrosis (PMF) who developed a clonally related LCH while in treatment with Ruxolitinib. JAK-inhibitors are well known to affect function and differentiation of different hematological lineages, including mononuclear phagocytes precursors...
October 28, 2017: Human Pathology
https://www.readbyqxmd.com/read/29104110/pseudomyogenic-hemangioendothelioma-of-skin-bone-and-soft-tissue-a-clinicopathological-immunohistochemical-and-fluorescence-in-situ-hybridization-study
#14
Dinesh Pradhan, Karen Schoedel, Richard L McGough, Sarangarajan Ranganathan, Uma N M Rao
Pseudomyogenic hemangioendothelioma (PHE) is an uncommon neoplasm with propensity for local recurrence. The tumor mimics epithelioid hemangioendothelioma and epithelioid sarcoma, representing a possible diagnostic pitfall. We investigated the clinicopathological, immunohistochemical and fluorescence in situ hybridization features of PHEs. Eight cases of PHE were retrieved from our pathology archives. The clinical and outcome information was available in six patients. In six cases the tumors were located in the lower limb while the upper limb was involved in two cases...
November 2, 2017: Human Pathology
https://www.readbyqxmd.com/read/29097382/gene-expression-and-risk-of-leukemic-transformation-in-myelodysplasia
#15
Yusuke Shiozawa, Luca Malcovati, Anna Gallì, Andrea Pellagatti, Mohsen Karimi, Aiko Sato-Otsubo, Yusuke Sato, Hiromichi Suzuki, Tetsuichi Yoshizato, Kenichi Yoshida, Yuichi Shiraishi, Kenichi Chiba, Hideki Makishima, Jacqueline Boultwood, Eva Hellström-Lindberg, Satoru Miyano, Mario Cazzola, Seishi Ogawa
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic disorders with a highly variable prognosis. To identify a gene expression-based classification of myelodysplasia with biological and clinical relevance, we performed a comprehensive transcriptomic analysis of myeloid neoplasms with dysplasia using transcriptome sequencing. Unsupervised clustering of gene expression data of bone marrow CD34+ cells from 100 patients identified two subgroups. The first subtype was characterized by increased expression of genes related to erythroid/megakaryocytic (EMK) lineages, whereas the second subtype showed up-regulation of genes related to immature progenitor (IMP) cells...
November 2, 2017: Blood
https://www.readbyqxmd.com/read/29096349/extramedullary-hematopoiesis-mimicking-mediastinal-tumor-in-a-patient-with-hereditary-spherocytosis-case-report
#16
Jae Bum Park, Song Am Lee, Yo Han Kim, Woo Surng Lee, Jae Joon Hwang
INTRODUCTION: Extramedullary hematopoiesis (EMH) is a rare disorder, defined as the appearance of hematopoietic elements outside the bone marrow or peripheral blood. The exact mechanism of this development is still unknown. We herein report a case of intrathoracic EMH, manifesting as a posterior mediastinal tumor in a patient with hereditary spherocytosis. PRESENTATION OF CASE: A 45-year-old man who presented with anemia, jaundice and abdominal pain was diagnosed with hereditary spherocytosis...
October 27, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29076133/effects-of-vascular-endothelial-growth-factors-and-their-receptors-on-megakaryocytes-and-platelets-and-related-diseases
#17
REVIEW
Jin-Gang Yang, Li-Li Wang, Dong-Chu Ma
It is well known that vascular endothelial growth factors (VEGFs) and their receptors (vascular endothelial growth factor receptors, VEGFRs) are expressed in different tissues, and VEGF-VEGFR loops regulate a wide range of responses, including metabolic homeostasis, cell proliferation, migration and tubuleogenesis. As ligands, VEGFs act on three structurally related VEGFRs (VEGFR1, VEGFR2 and VEGFR3 [also termed FLT1, KDR and FLT4, respectively]) that deliver downstream signals. Haematopoietic stem cells (HSCs), megakaryocytic cell lines, cultured megakaryocytes (MKs), primary MKs and abnormal MKs express and secrete VEGFs...
October 26, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29075489/intravascular-large-b-cell-lymphoma-associated-with-myelofibrosis-a-case-report
#18
Jong Gwon Choi, Hwan Hwi Cho, Sang Rok Kang, Se Min Jang, Eun Hyung Yoo, Hyun Jung Cho, Sun Moon Kim, Do Yeun Cho
Myelofibrosis (MF) is often accompanied by chronic myeloid leukemia, hairy cell leukemia, or certain primary myeloproliferative neoplasms, but is rarely associated with lymphoid neoplasms. We herein describe a case of intravascular large B-cell lymphoma (IVLBCL) with MF. IVLBCL is a rare, aggressive type of extranodal B-cell lymphoma, defined by proliferation of lymphomatous cells within small-to medium-sized vessels. A 60-year-old woman was admitted to the hospital with anemia, thrombocytopenia and fever. Bone marrow biopsy findings included trilineage hematopoiesis, increased numbers of immature cells, markedly abnormal and enlarged megakaryocytes, and diffuse fibrosis in multiple focal areas throughout the entire bone marrow space...
November 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29075488/mucin-poor-and-aggressive-mucinous-tubular-and-spindle-cell-carcinoma-of-the-kidney-two-case-reports
#19
Shiro Uchida, Koyu Suzuki, Mieko Uno, Fumi Nozaki, Chih-Ping Li, Eriko Abe, Teruo Yamauchi, Saya Horiuchi, Minobu Kamo, Kazunori Hattori, Yoji Nagashima
Mucinous tubular and spindle cell carcinoma (MTSCC) is a relatively rare renal epithelial neoplasm. Although MTSCC is considered to be a low-grade and indolent neoplasm, aggressive cases have been recently reported. The present study discussed two additional cases of high-grade MTSCC causing multiple distant metastases with a fatal course. In case 1, a 71-year-old patient presented with hematuria and pyuria. Computed tomography (CT) scan of the right kidney revealed a mass lesion, for which partial nephrectomy was performed...
November 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29066743/cytoplasmic-cyclin-d1-controls-the-migration-and-invasiveness-of-mantle-lymphoma-cells
#20
Simon Body, Anna Esteve-Arenys, Hadjer Miloudi, Clara Recasens-Zorzo, Guergana Tchakarska, Alexandra Moros, Sophie Bustany, Anna Vidal-Crespo, Vanina Rodriguez, Régis Lavigne, Emmanuelle Com, Isolda Casanova, Ramón Mangues, Oliver Weigert, Alejandra Sanjuan-Pla, Pablo Menéndez, Bénédicte Marcq, Jean-Michel Picquenot, Patricia Pérez-Galán, Fabrice Jardin, Gaël Roué, Brigitte Sola
Mantle cell lymphoma (MCL) is a hematologic neoplasm characterised by the t(11;14)(q13;q32) translocation leading to aberrant cyclin D1 expression. The cell functions of cyclin D1 depend on its partners and/or subcellular distribution, resulting in different oncogenic properties. We observed the accumulation of cyclin D1 in the cytoplasm of a subset of MCL cell lines and primary cells. In primary cells, this cytoplasmic distribution was correlated with a more frequent blastoid phenotype. We performed immunoprecipitation assays and mass spectrometry on enriched cytosolic fractions from two cell lines...
October 24, 2017: Scientific Reports
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