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https://www.readbyqxmd.com/read/28817404/primary-renal-sarcomas-with-bcor-ccnb3-gene-fusion-a-report-of-2-cases-showing-histologic-overlap-with-clear-cell-sarcoma-of-kidney-suggesting-further-link-between-bcor-related-sarcomas-of-the-kidney-and-soft-tissues
#1
Pedram Argani, Yu-Chien Kao, Lei Zhang, Carlos Bacchi, Andres Matoso, Rita Alaggio, Jonathan I Epstein, Cristina R Antonescu
We report 2 primary renal sarcomas demonstrating BCOR-CCNB3 gene fusions that have recently been identified in undifferentiated round cell sarcomas of bone and soft tissue. These neoplasms occurred in male children aged 11 and 12 years, and both were cystic as a result of entrapment and dilatation of native renal tubules. Both cases were composed of variably cellular bland spindle cells with fine chromatin set in myxoid stroma and separated by a branching capillary vasculature. Both neoplasms demonstrated immunoreactivity for BCOR, cyclin D1, TLE1, and SATB2 in the spindle neoplastic cells and negativity in the prominent capillary vasculature...
August 16, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28806822/-multiple-myeloma-current-status-in-diagnostic-testing-and-therapy
#2
Michael Kehrer, Sebastian Koob, Andreas Strauss, Dieter Christian Wirtz, Jan Schmolders
Background Multiple myeloma is a haematological blood cancer of the bone marrow and is classified by the World Health Organisation (WHO) as a plasma cell neoplasm. In multiple myeloma, normal plasma cells transform into malignant myeloma cells and produce large quantities of an abnormal immunoglobulin called monoclonal protein or M protein. This ultimately causes multiple myeloma symptoms such as bone damage or kidney problems. The annual worldwide incidence of multiple myeloma is estimated to be 6 - 7/100,000 and accounts for 1% of all cancer...
August 14, 2017: Zeitschrift Für Orthopädie und Unfallchirurgie
https://www.readbyqxmd.com/read/28802501/myeloid-neoplasms
#3
REVIEW
Antonio Subtil
The classification of myeloid neoplasms has undergone major changes and currently relies heavily on genetic abnormalities. Cutaneous manifestations of myeloid neoplasms may be the presenting sign of underlying bone marrow disease. Dermal infiltration by neoplastic cells may occur in otherwise normal skin or in sites of cutaneous inflammation. Leukemia cutis occasionally precedes evidence of blood and/or bone marrow involvement (aleukemic leukemia cutis).
September 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/28798246/extramedullary-haematopoiesis-presenting-with-cardiac-tamponade-in-a-patient-with-polycythaemia-vera
#4
Faysal Haroun, Viktoria Elkis, Anne Chen, Elsie Lee
A 71-year-old man with a history of polycythaemia vera, diagnosed 4 years ago, presented to the emergency room with shortness of breath. A bedside echocardiogram revealed a large pericardial effusion with features concerning for pericardial tamponade. A left anterior thoracotomy and a pericardial window were emergently performed in the operating room and relieved the patient's symptoms. Histology evaluation of the pericardial fragments and pericardial fluid revealed the presence of trilineage haematopoietic elements without any increase in the blasts...
August 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28797996/detection-mutations-of-jak2-exon-12-in-patients-with-jak2-v617f-negative-myeloproliferative-disorders
#5
S Z Makani, N Parsamanesh, S Mirzaahmadi, M Hashemi, F Shaveisi-Zadeh, N Mansouri, M Ghazi, A Movafagh
BACKGROUND: Mutations in exon 12 of JAK2 gene are detected as clonal markers in hematopoietic lineages in myeloproliferative disorders (MPNs). Our aim was, to study the relation between N542-E543del mutation of JAK2 gene and myeloproliferative neoplasms in V617Fnegative patients. PATIENTS AND METHODS: DNA specimen from 34 patients and 44 healthy controls were genotyped using ARMs- PCR method. We analyzed exon 12 JAK2 aberration in 34 myeloproliferative cases to be readily detected by both ARMS-PCR and DNA analysis regardless of whether peripheral blood or bone marrow cells was manipulated as the origin of RNA...
May 2017: Gulf Journal of Oncology
https://www.readbyqxmd.com/read/28797511/conditions-simulating-primary-bone-neoplasms
#6
REVIEW
Jodi M Carter, Benjamin Matthew Howe, Carrie Y Inwards
A number of nonneoplastic conditions can mimic tumors of bone. Some of the more common mimics of primary bone tumors include infectious, inflammatory, periosteal, and degenerative joint disease-associated lesions that produce tumorlike bone surface-based or intraosseous lesions. This article considers a spectrum of reactive and nonreactive processes including stress fracture, subchondral cysts, osteonecrosis, heterotopic ossification, osteomyelitis, sarcoidosis, and amyloidoma that can present in such a way that they are mistaken for a tumor arising primary in bone...
September 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28797508/hematopoietic-tumors-primarily-presenting-in-bone
#7
REVIEW
Arjen H G Cleven, Pancras C W Hogendoorn
Hematologic neoplasms that primarily present in bone are rare; this article describes the most common examples of hematologic tumors primarily presenting in bone, including plasma cell myeloma, solitary plasmacytoma of bone, primary non-Hodgkin lymphoma of bone, acute lymphoblastic leukemia/lymphoma, and Langerhans cell histiocytosis. The macroscopic and microscopic features, differential diagnosis, diagnostic workup, and prognosis of all these different entities are discussed, with special emphasis on common differential diagnosis...
September 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28796790/mesenchymal-stromal-cells-msc-from-jak2-myeloproliferative-neoplasms-differ-from-normal-msc-and-contribute-to-the-maintenance-of-neoplastic-hematopoiesis
#8
Teresa L Ramos, Luis Ignacio Sánchez-Abarca, Beatriz Rosón-Burgo, Alba Redondo, Ana Rico, Silvia Preciado, Rebeca Ortega, Concepción Rodríguez, Sandra Muntión, Ángel Hernández-Hernández, Javier De Las Rivas, Marcos González, José Ramón González Porras, Consuelo Del Cañizo, Fermín Sánchez-Guijo
There is evidence of continuous bidirectional cross-talk between malignant cells and bone marrow-derived mesenchymal stromal cells (BM-MSC), which favors the emergence and progression of myeloproliferative neoplastic (MPN) diseases. In the current work we have compared the function and gene expression profile of BM-MSC from healthy donors (HD-MSC) and patients with MPN (JAK2V617F), showing no differences in the morphology, proliferation and differentiation capacity between both groups. However, BM-MSC from MPN expressed higher mean fluorescence intensity (MIF) of CD73, CD44 and CD90, whereas CD105 was lower when compared to controls...
2017: PloS One
https://www.readbyqxmd.com/read/28793931/strategy-for-the-treatment-and-follow-up-of-sinonasal-solitary-extramedullary-plasmacytoma-a-case-series
#9
Elena Cantone, Antonella Miriam Di Lullo, Luana Marano, Elia Guadagno, Gelsomina Mansueto, Pasquale Capriglione, Lucio Catalano, Maurizio Iengo
BACKGROUND: Extramedullary plasmacytoma is a rare neoplasm characterized by monoclonal proliferation of plasma cells outside bone marrow. It accounts for 4% of all non-epithelial sinonasal tumors. According to the literature, radiotherapy is the standard therapy for extramedullary plasmacytoma. However, the conversion rate of extramedullary plasmacytoma to multiple myeloma is reported to be between 11 and 33% over 10 years. The highest risk of conversion is reported during the first 2 years after diagnosis, but conversion has been noted up to 15 years after diagnosis...
August 10, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28792693/serum-hepcidin-levels-in-multiple-myeloma
#10
Manolov Victor, Hadjiev Evgeniy, Tzvetkova Gergana, Petrova Julia, Vasilev Vasil, Marinov Borislav, Bogov Ivo, Gramatikova Zlatina, Tzatchev Kamen
BACKGROUND: Multiple myeloma (MM) is a malignant disease with a 10% frequency among all haematology neoplasms. It is characterized by clone proliferation of plasmatic cells in bone marrow, monoclonal gammopathy, and anemia, hypercalcemia, and kidney failure and bone lesions. IL-6 is an inflammatory cytokine, potential growth factor for myeloma cells, as elevated serum levels are connected with poor disease prognosis. IL-6 modulates many gene transcriptions, encoding synthesis of acute phase proteins, including C-reactive protein (CRP) and hepcidin...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28791654/pharmacotherapy-of-myelofibrosis
#11
REVIEW
Douglas Tremblay, Bridget Marcellino, John Mascarenhas
Myelofibrosis (MF) is a myeloproliferative neoplasm that is pathologically characterized by bone marrow myeloproliferation, reticulin and collagen fibrosis, and extramedullary hematopoiesis. Constitutive activation of the Janus associated kinase (JAK)-signal transducers and activators of transcription signaling pathway with resultant elevation in pro-inflammatory cytokine levels is the pathogenic hallmark of MF. JAK inhibitors, namely ruxolitinib, have been successful in alleviating symptoms and reducing splenomegaly, but therapy-related myelosuppression has led to the further development of highly selective JAK2 inhibitors...
August 8, 2017: Drugs
https://www.readbyqxmd.com/read/28780248/invasive-follicular-variant-of-papillary-thyroid-cancer-harboring-the-nras-mutation-q61k-and-presenting-with-bone-metastasis-a-case-report
#12
Raman Mehrzad, Michiya Nishino, Carmelo Nucera, Dora Dias-Santagata, James V Hennessey, Per-Olof Hasselgren
INTRODUCTION: The follicular variant of papillary thyroid cancer (FVPTC) can be noninvasive or invasive. The invasive form of FVPTC commonly harbors BRAF mutations whereas RAS mutations are more often associated with noninvasive FVPTC and a favorable clinical outcome. CASE REPORT: A 47-year-old man presented with a metastasis to his right iliac bone as the initial manifestation of a 1.6cm invasive FVPTC. After total thyroidectomy, the patient underwent additional treatment, including thyroid hormone suppressive treatment to non-detectable TSH levels, repeated courses of radioiodine treatment, external beam radiation, and treatment with the tyrosine kinase inhibitor sorafenib...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28777856/-clinical-and-laboratory-features-of-13-cases-of-myeloid-neoplasms-with-double-del-20q
#13
Shuxiao Bai, Chunxiao Wu, Jun Zhang, Suning Chen, Jinlan Pan
OBJECTIVE: To report on clinical and laboratory features of myeloid neoplasms with double del(20q). METHODS: Cytogenetic examination of bone marrow was performed on 13 cases of myeloid neophasms with double del(20q) after 24 hours of cell culture. R-banding was used to analyze the karyotypes. Interphase fluorescence in situ hybridization (FISH) was performed using dual-color probes for 20q11/20q12. RESULTS: Double del(20q) was found to be the sole abnormality in 9 cases, double del(20q) and trisomy 9 was found in 1 case, trisomy del(20q) was found in 1 case, and sole del(20q) clone and double del(20q) clone were found to coexist in 2 cases...
August 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28775205/therapy-related-hematological-malignancies-after-peptide-receptor-radionuclide-therapy-with-177-lu-dota-octreotate-incidence-course-predicting-factors-in-patients-with-gep-nets
#14
Hendrik Bergsma, Kirsten van Lom, Mark Konijnenberg, Boen Kam, Jaap Teunissen, Wouter de Herder, Eric Krenning, Dik Kwekkeboom
Peptide Receptor Radionuclide Therapy (PRRT) may induce long-term toxicity to the bone marrow (BM). The aim of this study was to analyze persistent dysfunction of the hematopoietic system after PRRT with (177)Lu-DOTATATE in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Methods: The incidence and course of persistent hematological dysfunction (PHD) was analyzed in 274 (=GEP-NET) out of 367 patients with somatostatin receptor-positive tumors. PHD was defined as diagnosis of Myelodysplastic Syndrome (MDS), Acute Myeloid Leukemia (AML), Myeloproliferative Neoplasms (MPN), Myelodysplastic/Myeloproliferative neoplasms (MDS/MPN) or otherwise unexplained cytopenia (for more than 6 months)...
August 3, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28770949/treatment-of-patients-with-primary-myelofibrosis-using-dasatinib
#15
Q-L Song, B Zhang, Y Xu, R-X Xia, X-H Lu, Z-X Pei, Q-W Xu, W-Y Li, Z-D Li
OBJECTIVE: Primary myelofibrosis (PMF) is a chronic clonal myeloproliferative neoplasm. It is associated with a poor prognosis, with a median survival time of approximately five years. Thus far, there are no specific targeted drugs for PMF. In this study, we evaluated the efficacy and safety of dasatinib, a second-generation tyrosine kinase inhibitor, in six PMF patients. PATIENTS AND METHODS: From June 1, 2015 to February 29, 2016, six patients with PMF in our department were enrolled into this trial...
July 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28763336/metastatic-cutaneous-paraganglioma-a-case-report-and-review-of-the-literature
#16
Dianne de Leon, Kara Walton, Olayemi Sokumbi
Paragangliomas are rare neoplasms that arise from chromaffin cells of the sympathetic and parasympathetic nervous system. These tumors are often cured by surgical resection but the risk for metastatic disease exists, particularly for extra-adrenal paragangliomas. The behavior of these tumors is unpredictable, and clinical and histopathological features associated with malignancy have not been determined. The most common sites of metastases include local and distant lymph nodes, bone, liver, and lung. Cutaneous metastases are exceedingly rare with only 2 reported cases, both of which presented on the scalp...
September 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28760557/testicular-metastasis-from-prostatic-adenocarcinoma-presenting-as-recurrent-epididymo-orchitis
#17
Kyle Waisanen, Tijani Osumah, Sneha S Vaish
OBJECTIVE: To present a rare case of testicular metastasis from prostatic adenocarcinoma origin and to provide a meaningful discussion of currently available supporting literature. MATERIALS AND METHODS: Pertinent health information was obtained by retrospective analysis of hospital electronic medical records. RESULTS: A 93-year-old male with history of castration resistant prostatic adenocarcinoma presenting with recurrent episodes of epididymo-orchitis with Initial symptoms responsive to oral antibiotics...
July 28, 2017: Urology
https://www.readbyqxmd.com/read/28760297/progress-in-myelodysplastic-syndromes-clinicopathologic-correlations-and-immune%C3%A2-checkpoints
#18
Juliana E Hidalgo-López, Rashmi Kanagal-Shamanna, Andrés E Quesada, Beenu Thakral, Zhihong Hu, Takayuki Mitsuhashi, Mariko Yabe, Guillermo Garcia-Manero, Carlos E Bueso-Ramos
BACKGROUND: Myelodysplastic syndromes (MDS) are a group of clonal neoplasms characterized by ineffective hematopoiesis. Hypomethylating agent (HMA) therapy is one of the mainstays of MDS therapy. Failure of HMA therapy is related to poor outcome; hence, new therapeutic approaches are warranted in these patients. In MDS, the immune system has a pivotal role in modulation of hematopoiesis and clonal expansion. In neoplastic conditions, immune checkpoint (PD-1 and CTLA4 molecules) hide tumor cells from immune surveillance...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28750389/principles-in-the-examination-and-management-of-bone-neoplasms-normal-elements-and-the-cytologic-features-of-reactive-changes-in-the-bone
#19
(no author information available yet)
No abstract text is available yet for this article.
2017: Monographs in Clinical Cytology
https://www.readbyqxmd.com/read/28747182/recurrent-primary-mediastinal-giant-cell-tumor-of-soft-tissue-with-radiological-findings-a-rare-case-report-and-literature-review
#20
Qiongjie Hu, Jian Peng, Liming Xia
BACKGROUND: Giant cell tumor of soft tissue (GCT-ST), which histologically resembles GCT of bone, is a rare tumor. Usually, it is located in the lower extremities and trunk. GCT-ST, occurring in mediastinum, is extremely rare. CASE PRESENTATION: We encountered an 18-year-old Chinese woman who had mild dull pain on the left side of back. The following chest computed tomography (CT) showed a heterogeneous mass deeply situated in the posterior mediastinum with compression of the lung and invasion of the adjacent rib...
July 26, 2017: World Journal of Surgical Oncology
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