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Sample records for negative myeloproliferative neoplasms

  1. Philadelphia-negative classical myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Barbui, T.; Barosi, G.; Birgegard, G.

    2011-01-01

    We present a review of critical concepts and produce recommendations on the management of Philadelphia-negative classical myeloproliferative neoplasms, including monitoring, response definition, first- and second-line therapy, and therapy for special issues. Key questions were selected according...... for splenectomy include symptomatic portal hypertension, drug-refractory painful splenomegaly, and frequent RBC transfusions. The risk of allogeneic stem-cell transplantation-related complications is justified in transplantation-eligible patients whose median survival time is expected to be less than 5 years....

  2. Philadelphia-negative chronic myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Rosane Isabel Bittencourt

    2012-01-01

    Full Text Available Chronic myeloproliferative diseases without the Philadelphia chromosome marker (Ph-, although first described 60 years ago, only became the subject of interest after the turn of the millennium. In 2001, the World Health Organization (WHO defined the classification of this group of diseases and in 2008 they were renamed myeloproliferative neoplasms based on morphological, cytogenetic and molecular features. In 2005, the identification of a recurrent molecular abnormality characterized by a gain of function with a mutation in the gene encoding Janus kinase 2 (JAK2 paved the way for greater knowledge of the pathophysiology of myeloproliferative neoplasms. The JAK2 mutation is found in 90-98% of polycythemia vera and in about 50% essential thrombocytosis and primary myelofibrosis. In addition to the JAK2 mutation, other mutations involving TET2 (ten-eleven translocation, LNK (a membrane-bound adaptor protein; IDH1/2 (isocitrate dehydrogenase 1/2 enzyme; ASXL1 (additional sex combs-like 1 genes were found in myeloproliferative neoplasms thus showing the importance of identifying molecular genetic alterations to confirm diagnosis, guide treatment and improve our understanding of the biology of these diseases. Currently, polycythemia vera, essential thrombocytosis, myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia and mastocytosis are included in this group of myeloproliferative neoplasms, but are considered different situations with individualized diagnostic methods and treatment. This review updates pathogenic aspects, molecular genetic alterations, the fundamental criteria for diagnosis and the best approach for each of these entities.

  3. Myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Roaldsnes, Christina; Holst, René; Frederiksen, Henrik

    2017-01-01

    BACKGROUND: Polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) are clonal disorders collectively named as myeloproliferative neoplasms (MPN). Published data on epidemiology of MPN after the discovery of the JAK2 mutation and the 2008 WHO classifications are scarce. We...

  4. Interferon-alpha in the treatment of Philadelphia-negative chronic myeloproliferative neoplasms. Status and perspectives

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    Hasselbalch, Hans Carl; Larsen, Thomas Stauffer; Riley, Caroline Hasselbalch

    2011-01-01

    The Philadelphia-negative chronic myeloproliferative neoplasms encompass essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF). A major break-through in the understanding of the pathogenesis of these neoplasms occurred in 2005 by the discovery of the JAK2 V617F...

  5. Second malignancies in hydroxyurea and interferon-treated Philadelphia-negative myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Hansen, Iben Onsberg; Sørensen, Anders Lindholm; Hasselbalch, Hans Carl

    2017-01-01

    OBJECTIVE: In an era of controversy in regard to 'hydroxyurea-leukaemogenicity' and when interferon-alfa2 (IFN) is being revived in the treatment of Philadelphia-negative myeloproliferative neoplasms (MPNs), we aim in this single-centre observational study to describe the frequencies of second...

  6. Interferon-alpha in the treatment of Philadelphia-negative chronic myeloproliferative neoplasms. Status and perspectives

    DEFF Research Database (Denmark)

    Hasselbalch, H.C.; Larsen, T.S.; Riley, C.H.

    2011-01-01

    The Philadelphia-negative chronic myeloproliferative neoplasms encompass essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF). A major break-through in the understanding of the pathogenesis of these neoplasms occurred in 2005 by the discovery of the JAK2 V617F mu...... and potentially cure after long-term immune therapy with IFN-alpha2 is discussed and foreseen as an achievable goal in the future....

  7. Bone morbidity in chronic myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Farmer, Sarah; Ocias, Lukas Frans; Vestergaard, Hanne

    2015-01-01

    Patients with the classical Philadelphia chromosome-negative chronic myeloproliferative neoplasms including essential thrombocythemia, polycythemia vera and primary myelofibrosis often suffer from comorbidities, in particular, cardiovascular diseases and thrombotic events. Apparently, there is al...... mastocytosis (SM) where pathogenic mechanisms for bone manifestations probably involve effects of mast cell mediators on bone metabolism, the mechanisms responsible for increased fracture risk in other chronic myeloproliferative neoplasms are not known........ Chronic inflammation has been suggested to explain the initiation of clonal development and progression in chronic myeloproliferative neoplasms. Decreased bone mineral density and enhanced fracture risk are well-known manifestations of many chronic systemic inflammatory diseases. As opposed to systemic...

  8. Chasing down the triple-negative myeloproliferative neoplasms: Implications for molecular diagnostics.

    Science.gov (United States)

    Langabeer, Stephen E

    2016-01-01

    The majority of patients with classical myeloproliferative neoplasms (MPN) of polycythemia vera, essential thrombocythemia, and primary myelofibrosis harbor distinct disease-driving mutations within the JAK2 , CALR , or MPL genes. The term triple-negative has been recently applied to those MPN without evidence of these consistent mutations, prompting whole or targeted exome sequencing approaches to determine the driver mutational status of this subgroup. These strategies have identified numerous novel mutations that occur in alternative exons of both JAK2 and MPL , the majority of which result in functional activation. Current molecular diagnostic approaches may possess insufficient coverage to detect these alternative mutations, prompting further consideration of targeted exon sequencing into routine diagnostic practice. How to incorporate these illuminating findings into the expanding molecular diagnostic algorithm for MPN requires continual attention.

  9. Myeloproliferative neoplasm stem cells.

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    Mead, Adam J; Mullally, Ann

    2017-03-23

    Myeloproliferative neoplasms (MPNs) arise in the hematopoietic stem cell (HSC) compartment as a result of the acquisition of somatic mutations in a single HSC that provides a selective advantage to mutant HSC over normal HSC and promotes myeloid differentiation to engender a myeloproliferative phenotype. This population of somatically mutated HSC, which initiates and sustains MPNs, is termed MPN stem cells. In >95% of cases, mutations that drive the development of an MPN phenotype occur in a mutually exclusive manner in 1 of 3 genes: JAK2 , CALR , or MPL The thrombopoietin receptor, MPL, is the key cytokine receptor in MPN development, and these mutations all activate MPL-JAK-STAT signaling in MPN stem cells. Despite common biological features, MPNs display diverse disease phenotypes as a result of both constitutional and acquired factors that influence MPN stem cells, and likely also as a result of heterogeneity in the HSC in which MPN-initiating mutations arise. As the MPN clone expands, it exerts cell-extrinsic effects on components of the bone marrow niche that can favor the survival and expansion of MPN stem cells over normal HSC, further sustaining and driving malignant hematopoiesis. Although developed as targeted therapies for MPNs, current JAK2 inhibitors do not preferentially target MPN stem cells, and as a result, rarely induce molecular remissions in MPN patients. As the understanding of the molecular mechanisms underlying the clonal dominance of MPN stem cells advances, this will help facilitate the development of therapies that preferentially target MPN stem cells over normal HSC. © 2017 by The American Society of Hematology.

  10. General Information about Chronic Myeloproliferative Neoplasms

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    ... and diagnose chronic myeloproliferative neoplasms. Myeloproliferative neoplasms are a group of diseases in which the bone marrow makes ... the patient’s bloodstream. Transfusion therapy Transfusion therapy ( blood transfusion ) is a method of giving red blood cells, white blood ...

  11. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet.

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    Barbui, Tiziano; Tefferi, Ayalew; Vannucchi, Alessandro M; Passamonti, Francesco; Silver, Richard T; Hoffman, Ronald; Verstovsek, Srdan; Mesa, Ruben; Kiladjian, Jean-Jacques; Hehlmann, Rȕdiger; Reiter, Andreas; Cervantes, Francisco; Harrison, Claire; Mc Mullin, Mary Frances; Hasselbalch, Hans Carl; Koschmieder, Steffen; Marchetti, Monia; Bacigalupo, Andrea; Finazzi, Guido; Kroeger, Nicolaus; Griesshammer, Martin; Birgegard, Gunnar; Barosi, Giovanni

    2018-02-27

    This document updates the recommendations on the management of Philadelphia chromosome-negative myeloproliferative neoplasms (Ph-neg MPNs) published in 2011 by the European LeukemiaNet (ELN) consortium. Recommendations were produced by multiple-step formalized procedures of group discussion. A critical appraisal of evidence by using Grades of Recommendation, Assessment, Development and Evaluation (GRADE) methodology was performed in the areas where at least one randomized clinical trial was published. Seven randomized controlled trials provided the evidence base; earlier phase trials also informed recommendation development. Key differences from the 2011 diagnostic recommendations included: lower threshold values for hemoglobin and hematocrit and bone marrow examination for diagnosis of polycythemia vera (PV), according to the revised WHO criteria; the search for complementary clonal markers, such as ASXL1, EZH2, IDH1/IDH2, and SRSF2 for the diagnosis of myelofibrosis (MF) in patients who test negative for JAK2V617, CALR or MPL driver mutations. Regarding key differences of therapy recommendations, both recombinant interferon alpha and the JAK1/JAK2 inhibitor ruxolitinib are recommended as second-line therapies for PV patients who are intolerant or have inadequate response to hydroxyurea. Ruxolitinib is recommended as first-line approach for MF-associated splenomegaly in patients with intermediate-2 or high-risk disease; in case of intermediate-1 disease, ruxolitinib is recommended in highly symptomatic splenomegaly. Allogeneic stem cell transplantation is recommended for transplant-eligible MF patients with high or intermediate-2 risk score. Allogeneic stem cell transplantation is also recommended for transplant-eligible MF patients with intermediate-1 risk score who present with either refractory, transfusion-dependent anemia, blasts in peripheral blood > 2%, adverse cytogenetics, or high-risk mutations. In these situations, the transplant procedure should be

  12. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet.

    Science.gov (United States)

    Barbui, Tiziano; Barosi, Giovanni; Birgegard, Gunnar; Cervantes, Francisco; Finazzi, Guido; Griesshammer, Martin; Harrison, Claire; Hasselbalch, Hans Carl; Hehlmann, Rudiger; Hoffman, Ronald; Kiladjian, Jean-Jacques; Kröger, Nicolaus; Mesa, Ruben; McMullin, Mary F; Pardanani, Animesh; Passamonti, Francesco; Vannucchi, Alessandro M; Reiter, Andreas; Silver, Richard T; Verstovsek, Srdan; Tefferi, Ayalew

    2011-02-20

    We present a review of critical concepts and produce recommendations on the management of Philadelphia-negative classical myeloproliferative neoplasms, including monitoring, response definition, first- and second-line therapy, and therapy for special issues. Key questions were selected according the criterion of clinical relevance. Statements were produced using a Delphi process, and two consensus conferences involving a panel of 21 experts appointed by the European LeukemiaNet (ELN) were convened. Patients with polycythemia vera (PV) and essential thrombocythemia (ET) should be defined as high risk if age is greater than 60 years or there is a history of previous thrombosis. Risk stratification in primary myelofibrosis (PMF) should start with the International Prognostic Scoring System (IPSS) for newly diagnosed patients and dynamic IPSS for patients being seen during their disease course, with the addition of cytogenetics evaluation and transfusion status. High-risk patients with PV should be managed with phlebotomy, low-dose aspirin, and cytoreduction, with either hydroxyurea or interferon at any age. High-risk patients with ET should be managed with cytoreduction, using hydroxyurea at any age. Monitoring response in PV and ET should use the ELN clinicohematologic criteria. Corticosteroids, androgens, erythropoiesis-stimulating agents, and immunomodulators are recommended to treat anemia of PMF, whereas hydroxyurea is the first-line treatment of PMF-associated splenomegaly. Indications for splenectomy include symptomatic portal hypertension, drug-refractory painful splenomegaly, and frequent RBC transfusions. The risk of allogeneic stem-cell transplantation-related complications is justified in transplantation-eligible patients whose median survival time is expected to be less than 5 years.

  13. Drugs Approved for Myeloproliferative Neoplasms

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    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for myeloproliferative neoplasms. The list includes generic names, brand names, and common drug combinations, which are shown in capital letters. The drug names link to NCI's Cancer Drug Information summaries.

  14. Antecedent cardiovascular disease and autoimmunity in Philadelphia-negative chronic myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Sørensen, Anders Lindholm; Hasselbalch, Hans K

    2016-01-01

    Introduction: A single institution case-control study was conducted to evaluate the risk of developing chronic myeloproliferative neoplasms (MPNs) associated with prior autoimmune disease and cardiovascular disease (CVD). Method: Cases were 323 MPN patients and controls were 333 chronic lymphocytic...... leukemia (CLL) patients. Odds ratios (ORs) and p-values using Fischer's exact tests were calculated. The data was adjusted for confounding effects by logistic regression. Results: A significantly increased risk of MPNs compared to CLL was observed in subjects with a prior history of any autoimmune disease...... (OR = 1.86, 95%CI 1.16-2.98). A positive association between JAK2-V617F positive MPN and any autoimmune disease was observed at follow-up (OR = 2.62, 95% CI 1.21-5.67). A significantly increased risk of MPN compared to CLL was also observed in subjects with prior thromboembolic events (TE-events) (OR...

  15. Chronic myeloproliferative neoplasms and subsequent cancer risk

    DEFF Research Database (Denmark)

    Frederiksen, H.; Farkas, Dora Kormendine; Christiansen, C.F.

    2011-01-01

    Patients with chronic myeloproliferative neoplasms, including essential thrombocythemia (ET), polycythemia vera (PV), and chronic myeloid leukemia (CML), are at increased risk of new hematologic malignancies, but their risk of nonhematologic malignancies remains unknown. In the present study, we...... diagnosed with a chronic myeloproliferative neoplasm during 1977-2008. We compared the incidence of subsequent cancer in this cohort with that expected on the basis of cancer incidence in the general population (standardized incidence ratio). Overall, ET, PV, and CML patients were at increased risk...... conclude that patients with chronic myeloproliferative neoplasms are at increased risk of developing a new malignant disease....

  16. Neurological Findings in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Semra Paydas

    2013-04-01

    Full Text Available Myeloproliferative neoplasms (MPN arise from genetic deficiencies at the level of pluripotent stem cells. Each of these neoplasms is a clonal stem cell disorder with specific phenotypic, genetic and clinical properties. Age is one of the most important factors in the development of symptoms and complications associated with MPNs.High white blood cell counts in chronic myelocytic leukemia also known as leukocytosis may lead to central nervous system findings. Tumors developing outside the bone marrow named as extramedullary myeloid tumors (EMMT could be detected at the initial diagnosis or during the prognosis of the disease, which may cause neurological symptoms due to pressure of leukemic cell mass on various tissues along with spinal cord. Central nervous system involvement and thrombocytopenic hemorrhage may lead to diverse neurological symptoms and findings.Transient ischemic attack and thrombotic stroke are the most common symptoms in polycythemia vera. Besides thrombosis and hemorrage, transformation to acute leukemia can cause neurological symptoms and findings. Transient ischemic attack, thrombotic stroke and specifically hemorrage can give rise to neurological symptoms similar to MPN in essential thrombocytosis.Extramedullary hematopoiesis refers to hematopoietic centers arise in organ/tissues other than bone marrow in myelofibrosis. Extramedullar hematopoietic centers may cause intracranial involvement, spinal cord compression, seizures and hydrocephalia. Though rare, extramedullary hematopoiesis can be detected in cranial/spinal meninges, paraspinal tissue and intracerebral regions. Extramedullary hematopoiesis has been reported in peripheral neurons, choroid plexus, pituitary, orbits, orbital and lacrimal fossa and in sphenoidal sinuses. [Cukurova Med J 2013; 38(2.000: 157-169

  17. Molecular diagnostics of myeloproliferative neoplasms.

    Science.gov (United States)

    Langabeer, Stephen E; Andrikovics, Hajnalka; Asp, Julia; Bellosillo, Beatriz; Carillo, Serge; Haslam, Karl; Kjaer, Lasse; Lippert, Eric; Mansier, Olivier; Oppliger Leibundgut, Elisabeth; Percy, Melanie J; Porret, Naomi; Palmqvist, Lars; Schwarz, Jiri; McMullin, Mary F; Schnittger, Susanne; Pallisgaard, Niels; Hermouet, Sylvie

    2015-10-01

    Since the discovery of the JAK2 V617F mutation in the majority of the myeloproliferative neoplasms (MPN) of polycythemia vera, essential thrombocythemia and primary myelofibrosis ten years ago, further MPN-specific mutational events, notably in JAK2 exon 12, MPL exon 10 and CALR exon 9 have been identified. These discoveries have been rapidly incorporated into evolving molecular diagnostic algorithms. Whilst many of these mutations appear to have prognostic implications, establishing MPN diagnosis is of immediate clinical importance with selection, implementation and the continual evaluation of the appropriate laboratory methodology to achieve this diagnosis similarly vital. The advantages and limitations of these approaches in identifying and quantitating the common MPN-associated mutations are considered herein with particular regard to their clinical utility. The evolution of molecular diagnostic applications and platforms has occurred in parallel with the discovery of MPN-associated mutations, and it therefore appears likely that emerging technologies such as next-generation sequencing and digital PCR will in the future play an increasing role in the molecular diagnosis of MPN. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. Prevalence and prognostic impact of allelic imbalances associated with leukemic transformation of Philadelphia chromosome–negative myeloproliferative neoplasms

    Science.gov (United States)

    Krug, Utz O.; Lee, Dhong Hyun Tony; Kawamata, Norihiko; Iwanski, Gabriela B.; Lasho, Terra; Weiss, Tamara; Nowak, Daniel; Koren-Michowitz, Maya; Kato, Motohiro; Sanada, Masashi; Shih, Lee-Yung; Nagler, Arnon; Raynaud, Sophie D.; Müller-Tidow, Carsten; Mesa, Ruben; Haferlach, Torsten; Gilliland, D. Gary; Tefferi, Ayalew; Ogawa, Seishi; Koeffler, H. Phillip

    2010-01-01

    Philadelphia chromosome–negative myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia, and primary myelofibrosis show an inherent tendency for transformation into leukemia (MPN-blast phase), which is hypothesized to be accompanied by acquisition of additional genomic lesions. We, therefore, examined chromosomal abnormalities by high-resolution single nucleotide polymorphism (SNP) array in 88 MPN patients, as well as 71 cases with MPN-blast phase, and correlated these findings with their clinical parameters. Frequent genomic alterations were found in MPN after leukemic transformation with up to 3-fold more genomic changes per sample compared with samples in chronic phase (P disease progression including not only established targets (ETV6, TP53, and RUNX1) but also new candidate genes on 7q, 16q, 19p, and 21q. Moreover, trisomy 8 or amplification of 8q24 (MYC) was almost exclusively detected in JAK2V617F− cases with MPN-blast phase. Remarkably, copy number–neutral loss of heterozygosity (CNN-LOH) on either 7q or 9p including homozygous JAK2V617F was related to decreased survival after leukemic transformation (P = .01 and P = .016, respectively). Our high-density SNP-array analysis of MPN genomes in the chronic compared with leukemic stage identified novel target genes and provided prognostic insights associated with the evolution to leukemia. PMID:20068225

  19. Methylation profiling of SOCS1, SOCS2, SOCS3, CISH and SHP1 in Philadelphia-negative myeloproliferative neoplasm.

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    Zhang, Min Yue; Fung, Tsz Kin; Chen, Fang Yuan; Chim, Chor Sang

    2013-10-01

    Janus kinase-signal transducer and activator of transcription (JAK/STAT) signalling, pivotal in Philadelphia-negative (Ph-ve) myeloproliferative neoplasm (MPN), is negatively regulated by molecules including SOCSs, CISH and SHP1. SOCS1, SOCS2 and SOCS3 methylation have been studied in MPN with discordant results. Herein, we studied the methylation status of SOCS1, SOCS2 and SOCS3, CISH and SHP1 by methylation-specific polymerase chain reaction (MSP) in cell lines and 45 diagnostic marrow samples of Ph-ve MPN. Moreover, we attempted to explain the discordance of methylation frequency by mapping the studied MSP primers to the respective genes. Methylation was detected in normal controls using SOCS2 MSP primers in the 3'translated exonic sequence, but not primers around the transcription start site in the 5' untranslated regions (5'UTR). SOCS1, SOCS2, SOCS3 and CISH were completely unmethylated in primary MPN samples and cell lines. In contrast, methylation of SHP1 was detected in 8.9% primary marrow samples. Moreover, SHP1 was completely methylated in K562 cell line, leading to reversible SHP1 silencing. A review of methylation studies of SOCS1 and SOCS3 showed that spuriously high rates of SOCS methylation had been reported using MSP primers targeting CpG sites in the 3'translated exonic sequence, which is also methylated in normal controls. However, using MSP primers localized to the 5'UTR, methylation of SOCS1, SOCS2 and SOCS3 is infrequent across all studies. In summary, methylation of SOCS1, SOCS2, SOCS3 and CISH is infrequent in Ph-ve MPN. Appropriate MSP primers are important for accurate estimation of the methylation frequency. The role of SHP1 methylation in the pathogenesis of MPN warrants further investigation. © 2013 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  20. Myeloproliferative neoplasms in five multiple sclerosis patients

    DEFF Research Database (Denmark)

    Thorsteinsdottir, Sigrun; Bjerrum, Ole Weis

    2013-01-01

    The concurrence of myeloproliferative neoplasms (MPNs) and multiple sclerosis (MS) is unusual. We report five patients from a localized geographic area in Denmark with both MS and MPN; all the patients were diagnosed with MPNs in the years 2007-2012. We describe the patients' history and treatment...

  1. Clinical end points for drug treatment trials in BCR-ABL1-negative classic myeloproliferative neoplasms: consensus statements from European LeukemiaNET (ELN) and Internation Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT).

    Science.gov (United States)

    Barosi, G; Tefferi, A; Besses, C; Birgegard, G; Cervantes, F; Finazzi, G; Gisslinger, H; Griesshammer, M; Harrison, C; Hehlmann, R; Hermouet, S; Kiladjian, J-J; Kröger, N; Mesa, R; Mc Mullin, M F; Pardanani, A; Passamonti, F; Samuelsson, J; Vannucchi, A M; Reiter, A; Silver, R T; Verstovsek, S; Tognoni, G; Barbui, T

    2015-01-01

    The discovery of somatic mutations, primarily JAK2V617F and CALR, in classic BCR-ABL1-negative myeloproliferative neoplasms (MPNs) has generated interest in the development of molecularly targeted therapies, whose accurate assessment requires a standardized framework. A working group, comprised of members from European LeukemiaNet (ELN) and International Working Group for MPN Research and Treatment (IWG-MRT), prepared consensus-based recommendations regarding trial design, patient selection and definition of relevant end points. Accordingly, a response able to capture the long-term effect of the drug should be selected as the end point of phase II trials aimed at developing new drugs for MPNs. A time-to-event, such as overall survival, or progression-free survival or both, as co-primary end points, should measure efficacy in phase III studies. New drugs should be tested for preventing disease progression in myelofibrosis patients with early disease in randomized studies, and a time to event, such as progression-free or event-free survival should be the primary end point. Phase III trials aimed at preventing vascular events in polycythemia vera and essential thrombocythemia should be based on a selection of the target population based on new prognostic factors, including JAK2 mutation. In conclusion, we recommended a format for clinical trials in MPNs that facilitates communication between academic investigators, regulatory agencies and drug companies.

  2. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2

    NARCIS (Netherlands)

    Nangalia, J.; Massie, C.E.; Baxter, E.J.; Nice, F.L.; Gundem, G.; Wedge, D.C.; Avezov, E.; Li, J.; Kollmann, K.; Kent, D.G.; Aziz, A.; Godfrey, A.L.; Hinton, J.; Martincorena, I.; Loo, P. Van; Jones, A.V.; Guglielmelli, P.; Tarpey, P.; Harding, H.P.; Fitzpatrick, J.D.; Goudie, C.T.; Ortmann, C.A.; Loughran, S.J.; Raine, K.; Jones, D.R.; Butler, A.P.; Teague, J.W.; O'Meara, S.; McLaren, S.; Bianchi, M.; Silber, Y.; Dimitropoulou, D.; Bloxham, D.; Mudie, L.; Maddison, M.; Robinson, B.; Keohane, C.; Maclean, C.; Hill, K.; Orchard, K.; Tauro, S.; Du, M.Q.; Greaves, M.; Bowen, D.; Huntly, B.J.; Harrison, C.N.; Cross, N.C.; Ron, D.; Vannucchi, A.M.; Papaemmanuil, E.; Campbell, P.J.; Green, A.R.

    2013-01-01

    BACKGROUND: Somatic mutations in the Janus kinase 2 gene (JAK2) occur in many myeloproliferative neoplasms, but the molecular pathogenesis of myeloproliferative neoplasms with nonmutated JAK2 is obscure, and the diagnosis of these neoplasms remains a challenge. METHODS: We performed exome sequencing

  3. Comparative study of different methodologies to detect the JAK2 V617F mutation in chronic BCR-ABL1 negative myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Alline Didone

    2016-04-01

    Full Text Available Objectives: A mutation in the JAK2 gene, V617F, has been identified in several BCR-ABL1 negative myeloproliferative neoplasms (MPN: polycythemia vera (PV, essential thrombocythemia (ET, and primary myelofibrosis (PMF. Defining the presence or absence of this mutation is an essential part of clinical diagnostic algorithms and patient management. Here, we aimed to evaluate the performance of three PCR-based assays: Amplification Refractory Mutation System (ARMS, High-Resolution Melting analysis (HRM, and Sanger direct sequencing, and compare their results with those obtained by a PCR restriction fragment polymorphism assay (PCR-RFLP. Design and methods: We used blood samples from 136 patients (PV=20; PMF=20; ET=28, and other MPN suspected cases=68. Results: Comparable results were observed among the four assays in patients with PV, PMF, and MPN suspected cases. In patients with a diagnosis of ET, the JAK2 V617F mutation was detected in 67.8% of them by the PCR-ARMS and PCR-HRM assay and in 64% of them by the conventional Sanger sequence approach. The PCR-ARMS and PCR-HRM assays were 100% concordant. With these tests, only one of the 20 patients with ET and one of the three patients with clinically suspected MPN gave different results compared with those obtained by the PCR-RFLP. Conclusions: Our results have demonstrated that the PCR-ARMS and PCR-HRM assays could detect the JAK2 V617F mutation effectively in MPN patients, but PCR-HRM assays are rapid and the most cost-effective procedures. Keywords: Myeloproliferative, JAK2 V617F, Mutation, Wild type, Screening

  4. Inheritance of the chronic myeloproliferative neoplasms. A systematic review

    DEFF Research Database (Denmark)

    Ranjan, Ajenthen; Penninga, E; Jelsig, Am

    2012-01-01

    This systematic review investigated the inheritance of the classical chronic myeloproliferative neoplasms (MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and chronic myelogenous leukemia (CML). Sixty-one articles were included and provided 135...

  5. Safety and efficacy of combination therapy of interferon-alpha2 + JAK1-2 Inhibitor in the philadelphia-negative chronic myeloproliferative neoplasms. Preliminary results from the danish combi-trial-an open label, single arm, non-randomized multicenter phase II study

    DEFF Research Database (Denmark)

    Mikkelsen, S. U.; Kjaer, L.; Skov, V.

    2015-01-01

    Background: The Philadelphia-negative, chronic myeloproliferative neoplasms (MPN) include essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (MF) (PMF). Chronic inflammation and a deregulated immune system are considered important for clonal evolution and disease pro...

  6. A new era for IFN-α in the treatment of Philadelphia-negative chronic myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Hasselbalch, H.C.

    2011-01-01

    in treating patients with these neoplasms favors the upfront use of pegylated interferons, the goal being to influence the development of the disease at the molecular level and revert patients to a stage of 'minimal residual disease/operational cure' instead of progressive clonal evolution, genomic...

  7. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2.

    Science.gov (United States)

    Nangalia, J; Massie, C E; Baxter, E J; Nice, F L; Gundem, G; Wedge, D C; Avezov, E; Li, J; Kollmann, K; Kent, D G; Aziz, A; Godfrey, A L; Hinton, J; Martincorena, I; Van Loo, P; Jones, A V; Guglielmelli, P; Tarpey, P; Harding, H P; Fitzpatrick, J D; Goudie, C T; Ortmann, C A; Loughran, S J; Raine, K; Jones, D R; Butler, A P; Teague, J W; O'Meara, S; McLaren, S; Bianchi, M; Silber, Y; Dimitropoulou, D; Bloxham, D; Mudie, L; Maddison, M; Robinson, B; Keohane, C; Maclean, C; Hill, K; Orchard, K; Tauro, S; Du, M-Q; Greaves, M; Bowen, D; Huntly, B J P; Harrison, C N; Cross, N C P; Ron, D; Vannucchi, A M; Papaemmanuil, E; Campbell, P J; Green, A R

    2013-12-19

    Somatic mutations in the Janus kinase 2 gene (JAK2) occur in many myeloproliferative neoplasms, but the molecular pathogenesis of myeloproliferative neoplasms with nonmutated JAK2 is obscure, and the diagnosis of these neoplasms remains a challenge. We performed exome sequencing of samples obtained from 151 patients with myeloproliferative neoplasms. The mutation status of the gene encoding calreticulin (CALR) was assessed in an additional 1345 hematologic cancers, 1517 other cancers, and 550 controls. We established phylogenetic trees using hematopoietic colonies. We assessed calreticulin subcellular localization using immunofluorescence and flow cytometry. Exome sequencing identified 1498 mutations in 151 patients, with medians of 6.5, 6.5, and 13.0 mutations per patient in samples of polycythemia vera, essential thrombocythemia, and myelofibrosis, respectively. Somatic CALR mutations were found in 70 to 84% of samples of myeloproliferative neoplasms with nonmutated JAK2, in 8% of myelodysplasia samples, in occasional samples of other myeloid cancers, and in none of the other cancers. A total of 148 CALR mutations were identified with 19 distinct variants. Mutations were located in exon 9 and generated a +1 base-pair frameshift, which would result in a mutant protein with a novel C-terminal. Mutant calreticulin was observed in the endoplasmic reticulum without increased cell-surface or Golgi accumulation. Patients with myeloproliferative neoplasms carrying CALR mutations presented with higher platelet counts and lower hemoglobin levels than patients with mutated JAK2. Mutation of CALR was detected in hematopoietic stem and progenitor cells. Clonal analyses showed CALR mutations in the earliest phylogenetic node, a finding consistent with its role as an initiating mutation in some patients. Somatic mutations in the endoplasmic reticulum chaperone CALR were found in a majority of patients with myeloproliferative neoplasms with nonmutated JAK2. (Funded by the Kay

  8. The clinical significance of JAK2V617F mutation for Philadelphia-negative chronic myeloproliferative neoplasms in patients with splanchnic vein thrombosis.

    Science.gov (United States)

    Yonal, Ipek; Pinarbası, Binnur; Hindilerden, Fehmi; Hancer, Veysel Sabri; Nalcaci, Meliha; Kaymakoglu, Sabahattin; Diz-Kucukkaya, Reyhan

    2012-10-01

    Polycythemia vera (PV), essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF), collectively known as Philadelphia-negative (Ph-negative) chronic myeloproliferative neoplasms (MPNs), MPNs represent the most common causes of splanchnic vein thrombosis (SVT), including Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT). The JAK2V617F mutation has been demonstrated in most of the Ph-negative chronic MPNs. The study objective was to assess the diagnostic value of JAK2V617F mutation in patients with SVT in a group of 68 patients with SVT (42 PVT,19 BCS, 7 combined PVT and BCS). By DNA-melting curve analysis, the JAK2V617F mutation was detected in 42.1 % of BCS, 38.1 % of PVT and 71.4 % of combined PVT and BCS groups. Thirteen of 15 (86.6 %) SVT patients with overt MPN and 16 of 53 (30.1 %) SVT patients without overt MPN (patients with either normal blood counts or cytopenias), including 6 of 16 with BCS (37.5 %), 7 of 33 with PVT (21.2 %) and 3 of 4 with combined BCS and PVT (75 %) possessed JAK2V617F mutation. A substantial proportion of patients with SVT were recognized as carriers of the JAK2V617F mutation despite the absence of overt signs of MPN. Receiver Operating Characteristic (ROC) curve analysis determined a platelet count of 190,000 mm(3) (area under the curve; AUC = 0.724, p = 0.002) and a white blood cell (WBC) count of 8,150 mm(3) (AUC = 0.76, p = 0.001) as the best cut-off values for the highest sensitivity and specificity ratios of the JAK2V617F mutation in patients with SVT. A significant positive correlation existed between the JAK2V617F mutational status of SVT patients and the WBC and platelet counts. Our results imply that JAK2V617F mutation screening should be an initial test for MPN in patients with SVT.

  9. [Approach to diagnosis and management of myeloproliferative neoplasm variants].

    Science.gov (United States)

    Mitsumori, Toru; Kirito, Keita

    2015-08-01

    Myeloproliferative neoplasm (MPN) variants are defined as relatively uncommon myeloid neoplasms which do not meet the criteria for either classical MPN or myelodysplastic syndrome. Due to the lack of specific markers, it has been challenging to accurately diagnose these malignant diseases. Recent studies have revealed new genetic abnormalities in MPN variants. These research advances are anticipated to open new approaches to not only achieving accurate diagnosis but also novel therapeutic options for these diseases.

  10. Chronic Myeloid Leukemia 2011: Successes, challenges, and strategies – Proceedings of the 5th Annual BCR-ABL1 positive and BCR-ABL1 negative myeloproliferative neoplasms workshop

    Science.gov (United States)

    Mughal, Tariq I; Radich, Jerald P; Van Etten, Richard A.; Quintás-Cardama, Alfonso; Skorski, Tomasz; Ravandi, Farhad; DeAngelo, Daniel J.; Gambacorti-Passerini, Carlo; Martinelli, Giovanni; Tefferi, Ayalew

    2012-01-01

    This report is based on the presentations and discussions at the 5th annual BCR-ABL1 positive and BCR-ABL1 negative myeloproliferative neoplasms (MPN) workshop, which took place immediately following the 52nd American Society of Hematology (ASH) meeting in Orlando, Florida on December 7th-8th, 2011. Relevant data which was presented at the ASH meeting as well as all other recent publications were presented and discussed at the workshop. This report covers front-line therapies of BCR-ABL1-positive leukemias, in addition to addressing some topical biological, pre-clinical and clinical issues, such as new insights into genomic instability and resistance to tyrosine kinase inhibitors (TKIs), risk stratification and optimizing molecular monitoring. A report pertaining to the new therapies and other pertinent preclinical and clinical issues in the BCR-ABL1 negative MPNs is published separately. PMID:21850662

  11. Allo-SCT for  Philadelphia-negative myeloproliferative neoplasms in blast phase: a study from the Societe Française de Greffe de Moelle et de Therapie Cellulaire (SFGM-TC).

    Science.gov (United States)

    Cahu, X; Chevallier, P; Clavert, A; Suarez, F; Michallet, M; Vincent, L; Vigouroux, S; Blaise, D; Mariette, C; Bilger, K; Robin, M; Yakoub-Agha, I; Peffault de Latour, R; Mohty, M

    2014-06-01

    Progression of Philadelphia-negative myeloproliferative (MPN) or myelodysplastic/myeloproliferative neoplasms (MDS/MPN) to acute myeloid leukemia (AML) is an adverse event in the course of the disease. Although allogeneic hematopoietic SCT (allo-SCT) is considered as the only curative therapy, few data exist on the outcome of patients with Philadelphia-negative MPN or MDS/MPN in blast phase who received an allo-SCT. Sixty patients were included in this retrospective study. AML was secondary to an MPN in 43 cases, whereas AML evolved from an MDS/MPN in 17 cases. Patients received allo-SCT in CR or advanced disease in 26 cases and 34 cases, respectively. With a median follow-up of 31 months (range, 25-44), OS and leukemia-free survival (LFS) were, respectively, 18% and 9% at 3 years. CR at transplant was associated with an improved LFS in univariate and multivariate analysis. The 3-year LFS was 18% for patients undergoing allo-SCT in CR versus 3% in advanced disease (P=0.008). Absence of thrombosis and an intermediate or favorable AML karyotype were associated with an improved outcome for patients who received allo-SCT in CR. New strategies are needed to improve the outcome of patients with MPN-MDS/MPN in blast phase.

  12. Myelodysplastic and myeloproliferative neoplasms: updates on the overlap syndromes.

    Science.gov (United States)

    Thota, Swapna; Gerds, Aaron T

    2018-04-01

    Myelodysplastic and myeloproliferative neoplasms (MDS/MPN) is a rare and distinct group of myeloid neoplasms with overlapping MDS and MPN features. Next generation sequencing studies have led to an improved understanding of MDS/MPN disease biology by identifying recurrent somatic mutations. Combining the molecular findings to patho-morphologic features has improved the precision of diagnosis and prognostic models in MDS/MPN. We discuss and highlight these updates in MDS/MPN nomenclature and diagnostic criteria per revised 2016 WHO classification of myeloid neoplasms in this article. There is an ongoing effort for data integration allowing for comprehensive genomic characterization, development of improved prognostic tools, and investigation for novel therapies using an international front specific for MDS/MPN. In this article, we discuss updates in prognostic models and current state of treatment for MDS/MPN.

  13. SETBP1 mutations as a biomarker for myelodysplasia /myeloproliferative neoplasm overlap syndrome

    OpenAIRE

    Linder, Katherine; Iragavarapu, Chaitanya; Liu, Delong

    2017-01-01

    Myelodysplasia (MDS) /myeloproliferative neoplasm (MPN) overlap syndrome has been described since the 2001 WHO classification as disorders that have both proliferative and dysplastic changes simultaneously. Specific disorders include chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), BCR-ABL negative atypical chronic myeloid leukemia (aCML) and unclassifiable MDS/MPN (MPN/MDS-U). Recurrent gene mutations in these conditions have been described. Among them, SETBP1...

  14. Hypothetical atopic dermatitis-myeloproliferative neoplasm (AD-MPN syndrome

    Directory of Open Access Journals (Sweden)

    Toshiaki eKawakami

    2015-08-01

    Full Text Available Atopic dermatitis (AD is a chronic inflammatory skin disease. Myeloproliferative neoplasms (MPNs are hematopoietic malignancies caused by uncontrolled proliferation of hematopoietic stem/progenitor cells. Recent studies have described several mutant mice exhibiting both AD-like skin inflammation and MPN. Common pathways for skin inflammation encompass overexpression of thymic stromal lymphopoietin and reduced signaling of epidermal growth factor receptor in the epidermis, while overproduction of granulocyte-colony stimulating factor by keratinocytes and constitutive activation of Stat5 in hematopoietic stem cells are important for the development of MPN. The murine studies suggest the existence of a similar human disease tentatively termed the AD-MPN syndrome.

  15. Molecular mechanisms associated with leukemic transformation of MPL-mutant myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Beer, Philip A; Ortmann, Christina A; Stegelmann, Frank

    2010-01-01

    , was not detected in 172 patients with a myeloproliferative neoplasm. In patients with a prior MPL W515L-mutant myeloproliferative neoplasm, leukemic transformation was accompanied by MPL-mutant leukemic blasts, was seen in the absence of prior cytoreductive therapy and often involved loss of wild-type MPL...

  16. Obesity and related risk of myeloproliferative neoplasms among israeli adolescents.

    Science.gov (United States)

    Leiba, Adi; Duek, Adrian; Afek, Arnon; Derazne, Estela; Leiba, Merav

    2017-07-01

    Obesity has been associated with various malignancies, but a clear association between overweight and myeloproliferative neoplasms (MPN) has not been established. This study assessed the association between adolescent obesity and future risk for MPN. Data on 2,516,256 Israeli adolescents, who underwent a compulsory general health examination at ages 16 to 19, between 1967 and 2011, were linked to the National Cancer Registry in this nationwide, population-based cohort study. Cox proportional hazards models were used to estimate the hazard ratio (HR) for MPN associated with BMI measured at adolescence. The mean follow-up of 19.86 ± 12.15 years reflected 49,977,521 person years, during which 433 examinees developed MPN, primarily chronic myelogenous leukemia, polycythemia vera, and essential thrombocythemia. Obesity (BMI ≥ 95th percentile) in adolescence significantly predicted increased risk of MPN with HR (adjusted for sex) of 1.81 (95% confidence interval 1.13-2.92, P = 0.014). Adolescent obesity might be related to an increased incidence of myeloproliferative neoplasms. © 2017 The Obesity Society.

  17. Platelet indices in Philadelphia-negative chronic myeloproliferative neoplasms / Indicii plachetari de volum în neoplasmele mieloproliferative cronice Philadelphia-negative

    Directory of Open Access Journals (Sweden)

    Olteanu Ariela-Ligia

    2015-06-01

    Full Text Available Introducere: Neoplasmele mieloproliferative cronice cromozom Philadelphia negative (Ph-MPN -policitemia vera (PV, trombocitemia esenţială (TE şi mielofibroza primară (MP - sunt caracterizate de o rată mare de complicaţii trombotice, parţial date de activarea trombocitară. Trombocitele mari sunt mai active, cu o funcţie procoagulantă crescută şi au rol patogenic demonstrat în tromboza arterială şi venoasă. În studiul nostru am încercat să stabilim dacă indicii trombocitari (MPV, PDW, P-LCR, furnizaţi de analizoarele de hematologie în hemoleucograma completă, sunt modificaţi la pacienţii diagnosticaţi cu Ph-MPN comparativ cu subiecţii sănătoşi. Material şi metodă: Hemoleucograma, ce a inclus şi indicii trombocitari, a fost determinată la 102 pacienţi Ph- MPN şi la 102 subiecţi sănătoşi cu ajutorul analizorului de hematologie Sysmex XS 1000i (metoda impedanţei, iar testele biochimice (glucoza şi parametrii metabolismului lipidic, pe analizorul Architect c8000. Prezenţa mutaţiei JAK2 V617F, la pacienţii cu Ph-MPN, a fost evidenţiată prin tehnica ARMS-PCR. Rezultate: Indicii de volum plachetari (IVP măsuraţi prin metoda impedanţei, nu au fost semnificativ modificaţi la pacienţii cu Ph-MPN comparativ cu cei ai subiecţilor sănătoşi. Totuşi, am observat o moderată corelaţie între valoarea acestor indici şi prezenţa mutaţiei JAK2V617F, fără însă a se atinge o diferenţă semnificativă din punct de vedere statistic. IVP au fost crescuţi la micul subgrup de pacienţi trataţi cu anagrelide şi scăzuţi la cei trataţi cu simvastatin, comparativ cu pacienţii care nu au primit aceste terapii. Concluzii: Folosind metoda impedanţei în determinarea indicilor trombocitari, în studiul nostru nu am demonstrat o diferenţă semnificativă între valorile indicilor trombocitari la pacienţii cu Ph- MPN comparativ cu subiecţii sănătoşi. Sunt necesare studii ulterioare pentru a demonstra o

  18. Mouse models of myeloproliferative neoplasms: JAK of all grades

    Directory of Open Access Journals (Sweden)

    Juan Li

    2011-05-01

    Full Text Available In 2005, several groups identified a single gain-of-function point mutation in the JAK2 kinase that was present in the majority of patients with myeloproliferative neoplasms (MPNs. Since this discovery, much effort has been dedicated to understanding the molecular consequences of the JAK2V617F mutation in the haematopoietic system. Three waves of mouse models have been produced recently (bone marrow transplantation, transgenic and targeted knock-in, which have facilitated the understanding of the molecular pathogenesis of JAK2V617F-positive MPNs, providing potential platforms for designing and validating novel therapies in humans. This Commentary briefly summarises the first two types of mouse models and then focuses on the more recently generated knock-in models.

  19. Cytokine Regulation of Microenvironmental Cells in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Gregor Hoermann

    2015-01-01

    Full Text Available The term myeloproliferative neoplasms (MPN refers to a heterogeneous group of diseases including not only polycythemia vera (PV, essential thrombocythemia (ET, and primary myelofibrosis (PMF, but also chronic myeloid leukemia (CML, and systemic mastocytosis (SM. Despite the clinical and biological differences between these diseases, common pathophysiological mechanisms have been identified in MPN. First, aberrant tyrosine kinase signaling due to somatic mutations in certain driver genes is common to these MPN. Second, alterations of the bone marrow microenvironment are found in all MPN types and have been implicated in the pathogenesis of the diseases. Finally, elevated levels of proinflammatory and microenvironment-regulating cytokines are commonly found in all MPN-variants. In this paper, we review the effects of MPN-related oncogenes on cytokine expression and release and describe common as well as distinct pathogenetic mechanisms underlying microenvironmental changes in various MPN. Furthermore, targeting of the microenvironment in MPN is discussed. Such novel therapies may enhance the efficacy and may overcome resistance to established tyrosine kinase inhibitor treatment in these patients. Nevertheless, additional basic studies on the complex interplay of neoplastic and stromal cells are required in order to optimize targeting strategies and to translate these concepts into clinical application.

  20. Pathogenesis of Myeloproliferative Neoplasms: Role and Mechanisms of Chronic Inflammation

    Science.gov (United States)

    Bigot-Corbel, Edith; Gardie, Betty

    2015-01-01

    Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal diseases characterized by the excessive and chronic production of mature cells from one or several of the myeloid lineages. Recent advances in the biology of MPNs have greatly facilitated their molecular diagnosis since most patients present with mutation(s) in the JAK2, MPL, or CALR genes. Yet the roles played by these mutations in the pathogenesis and main complications of the different subtypes of MPNs are not fully elucidated. Importantly, chronic inflammation has long been associated with MPN disease and some of the symptoms and complications can be linked to inflammation. Moreover, the JAK inhibitor clinical trials showed that the reduction of symptoms linked to inflammation was beneficial to patients even in the absence of significant decrease in the JAK2-V617F mutant load. These observations suggested that part of the inflammation observed in patients with JAK2-mutated MPNs may not be the consequence of JAK2 mutation. The aim of this paper is to review the different aspects of inflammation in MPNs, the molecular mechanisms involved, the role of specific genetic defects, and the evidence that increased production of certain cytokines depends or not on MPN-associated mutations, and to discuss possible nongenetic causes of inflammation. PMID:26538820

  1. Methylation of miR-34a, miR-34b/c, miR-124-1 and miR-203 in Ph-negative myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Chim Chor Sang

    2011-11-01

    Full Text Available Abstract Background MicroRNA (miR miR-34a, -34b/c, -124-1 and -203 are tumor suppressor miRs implicated in carcinogenesis. Methods We studied DNA methylation of these miRs in Philadelphia-negative (Ph-ve myeloproliferative neoplasms (MPNs. Methylation-specific PCR (MSP, verified by direct sequencing of the methylated MSP products, was performed in cell lines, normal controls and diagnostic marrow samples of patients with MPNs. Results Methylation of these miRs was absent in the normal controls. miR-34b/c were homozygously methylated in HEL cells but heterozygously in MEG-01. In HEL cells, homozygous miR-34b/c methylation was associated with miR silencing, and 5-aza-2'-deoxycytidine treatment led to re-expression of both miR-34b and miR-34c, consistent with that both miRs are under the regulation of the same promoter CpG island. miR-34a was heterozygously methylated in MEG-01 and K-562. miR-203 was completely unmethylated in K-562 and SET-2 but no MSP amplification was found in both HEL and MEG-01, suggestive of miR deletion. In primary samples, four each had miR-34b/c and -203 methylation, in which two had concomitant methylation of miR-34b/c and -203. miR-34a was methylated in one patient and none had methylation of miR-124-1. Seven patients (15.6% had methylation of at least one of the four miRs. miR methylation did not correlate with clinical parameters, disease complications or JAK2 V617F mutation. Conclusion This is the first report of miR hypermethylation in MPNs. miR-203 hypermethylation is not specific to Ph+ve leukemias but also present in Ph-ve MPNs. miR-34b/c methylation was associated with reversible miR silencing. There was no correlation of miR methylation with clinical demographic data or outcome.

  2. Differential Dynamics of CALR Mutant Allele Burden in Myeloproliferative Neoplasms during Interferon Alfa Treatment

    DEFF Research Database (Denmark)

    Kjær, Lasse; Cordua, Sabrina; Holmström, Morten O

    2016-01-01

    Discovery of somatic mutations in the calreticulin gene (CALR) has identified a subgroup of Philadelphia-negative chronic myeloproliferative neoplasms (MPN) with separate haematological characteristics and prognosis. CALR mutations serve as novel markers both of diagnostic value and as targets...... for monitoring molecular responses during therapy. Interferon-α (IFN) selectively targets the malignant clone in a subset of MPN patients and can induce both haematological and molecular remissions in CALR mutated essential thrombocythemia (ET) patients. We investigated the response to IFN in a cohort of 21 CALR...... mutated MPN patients including ET, prefibrotic primary myelofibrosis (pre-PMF), and primary myelofibrosis (PMF) with a median follow-up of 31 months. For evaluation of a molecular response, we developed highly sensitive quantitative PCR (qPCR) assays for monitoring the mutant allele burden of the two most...

  3. Neutrophilic Dermatosis of the Dorsal Hands Associated with a “Myeloproliferative Neoplasm, Unclassifiable“ and a Simultaneous Cancer of Colon

    Directory of Open Access Journals (Sweden)

    Francisco José Fernández-Fernández

    2010-01-01

    Full Text Available Neutrophilic dermatosis of the dorsal hands is a variant of Sweet's syndrome. We herein describe an 83-year old woman with a neutrophilic dermatosis of the dorsal hands associated with a "myeloproliferative neoplasm, unclassifiable" and a simultaneous cancer of colon. To our knowledge, and after a search in PubMed, the association of Sweet's syndrome with a "myeloproliferative neoplasm, unclassifiable" and a simultaneous cancer of colon has not previously been reported.

  4. The Importance of Identification of M-BCRABL Oncogene and JAK2V617F Mutation in Myeloproliferative Neoplasms

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    Szántó Annamária

    2014-04-01

    Full Text Available Background: The elucidation of the genetic background of the myeloproliferative neoplasms completely changed the management of these disorders: the presence of the Philadelphia chromosome and/or the BCR-ABL oncogene is pathognomonic for chronic myeloid leukemia and identification of JAK2 gene mutations are useful in polycytemia vera (PV, essential thrombocytemia (ET and myelofibrosis (PMF. The aim of this study was to investigate the role of molecular biology tests in the management of myeloproliferative neoplasms.

  5. Myeloproliferative neoplasms and inflammation: whether to target the malignant clone or the inflammatory process or both.

    Science.gov (United States)

    Koschmieder, S; Mughal, T I; Hasselbalch, H C; Barosi, G; Valent, P; Kiladjian, J-J; Jeryczynski, G; Gisslinger, H; Jutzi, J S; Pahl, H L; Hehlmann, R; Maria Vannucchi, A; Cervantes, F; Silver, R T; Barbui, T

    2016-05-01

    The Philadelphia-negative myeloproliferative neoplasms (MPNs) are clonal disorders involving hematopoietic stem and progenitor cells and are associated with myeloproliferation, splenomegaly and constitutional symptoms. Similar signs and symptoms can also be found in patients with chronic inflammatory diseases, and inflammatory processes have been found to play an important role in the pathogenesis and progression of MPNs. Signal transduction pathways involving JAK1, JAK2, STAT3 and STAT5 are causally involved in driving both the malignant cells and the inflammatory process. Moreover, anti-inflammatory and immune-modulating drugs have been used successfully in the treatment of MPNs. However, to date, many unresoved issues remain. These include the role of somatic mutations that are present in addition to JAK2V617F, CALR and MPL W515 mutations, the interdependency of malignant and nonmalignant cells and the means to eradicate MPN-initiating and -maintaining cells. It is imperative for successful therapeutic approaches to define whether the malignant clone or the inflammatory cells or both should be targeted. The present review will cover three aspects of the role of inflammation in MPNs: inflammatory states as important differential diagnoses in cases of suspected MPN (that is, in the absence of a clonal marker), the role of inflammation in MPN pathogenesis and progression and the use of anti-inflammatory drugs for MPNs. The findings emphasize the need to separate the inflammatory processes from the malignancy in order to improve our understanding of the pathogenesis, diagnosis and treatment of patients with Philadelphia-negative MPNs.

  6. Bleeding, thrombosis, and anticoagulation in myeloproliferative neoplasms (MPN: analysis from the German SAL-MPN-registry

    Directory of Open Access Journals (Sweden)

    A. Kaifie

    2016-03-01

    Full Text Available Abstract Background Patients with Ph-negative myeloproliferative neoplasms (MPN, such as polycythemia vera (PV, essential thrombocythemia (ET, and primary myelofibrosis (PMF, are at increased risk for thrombosis/thromboembolism and major bleeding. Due to the morbidity and mortality of these events, antiplatelet and/or anticoagulant agents are commonly employed as primary and/or secondary prophylaxis. On the other hand, disease-related bleeding complications (i.e., from esophageal varices are common in patients with MPN. This analysis was performed to define the frequency of such events, identify risk factors, and assess antiplatelet/anticoagulant therapy in a cohort of patients with MPN. Methods The MPN registry of the Study Alliance Leukemia is a non-interventional prospective study including adult patients with an MPN according to WHO criteria (2008. For statistical analysis, descriptive methods and tests for significant differences as well as contingency tables were used to identify the odds of potential risk factors for vascular events. Results MPN subgroups significantly differed in sex distribution, age at diagnosis, blood counts, LDH levels, JAK2V617F positivity, and spleen size (length. While most thromboembolic events occurred around the time of MPN diagnosis, one third of these events occurred after that date. Splanchnic vein thrombosis was most frequent in post-PV-MF and MPN-U patients. The chance of developing a thromboembolic event was significantly elevated if patients suffered from post-PV-MF (OR 3.43; 95 % CI = 1.39–8.48 and splenomegaly (OR 1.76; 95 % CI = 1.15–2.71. Significant odds for major bleeding were previous thromboembolic events (OR = 2.71; 95 % CI = 1.36–5.40, splenomegaly (OR = 2.22; 95 % CI 1.01–4.89, and the administration of heparin (OR = 5.64; 95 % CI = 1.84–17.34. Major bleeding episodes were significantly less frequent in ET patients compared to other MPN subgroups

  7. Splanchnic vein thrombosis in myeloproliferative neoplasms: pathophysiology and molecular mechanisms of disease

    Science.gov (United States)

    How, Joan; Zhou, Amy; Oh, Stephen T.

    2016-01-01

    Myeloproliferative neoplasms (MPNs) are the most common underlying prothrombotic disorder found in patients with splanchnic vein thrombosis (SVT). Clinical risk factors for MPN-associated SVTs include younger age, female sex, concomitant hypercoagulable disorders, and the JAK2 V617F mutation. These risk factors are distinct from those associated with arterial or deep venous thrombosis (DVT) in MPN patients, suggesting disparate disease mechanisms. The pathophysiology of SVT is thought to derive from local interactions between activated blood cells and the unique splanchnic endothelial environment. Other mutations commonly found in MPNs, including CALR and MPL, are rare in MPN-associated SVT. The purpose of this article is to review the clinical and molecular risk factors for MPN-associated SVT, with particular focus on the possible mechanisms of SVT formation in MPN patients. PMID:28246554

  8. Are chronic myeloproliferative neoplasms associated with age-related macular degeneration?

    DEFF Research Database (Denmark)

    Bak, M.; Sorensen, T. L.; Flachs, E. M.

    2015-01-01

    population-based matched cohort study using Danish registries. We included all patients age 18+ or older with a first listed diagnosis of MPN in the Danish National Patient Registry between 1994 and 2013. Patients with Essential Thrombocythemia (ET), Polycythemia Vera (PV), Myelofibrosis (MF), Unclassifiable......Background. Patients with Chronic Myeloproliferative Neoplasms (MPNs) already have increased comorbidity at time of diagnosis, and studies show association with ophthalmic manifestations. Retinal vascular symptoms including vascular occlusions and hemorrhages are present, but other manifestations...... of the eye have not been thoroughly investigated in these patients. Previously reported studies show signs of systemic inflammation in patients with MPN as well as in patients with Age-Related Macular Degeneration (AMD). Our hypothesis is that the presence of MPN predisposes some individuals to develop AMD...

  9. Non-invasive imaging of retinal blood flow in myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Willerslev, Anne; Hansen, Mathias M; Klefter, Oliver Niels

    2017-01-01

    . The retinopathy had resolved at follow-up in all patients. CONCLUSION: With non-invasive retinal imaging, we were able to demonstrate increased retinal venous blood velocity, increased retinal arterial blood oxygenation and normalization of intravascular reflectivity patterns after successful treatment......PURPOSE: To study the circulation in the retinal vessels in patients with blood dyscrasia due to myeloproliferative neoplasms using non-invasive retinal imaging. METHODS: Prospective consecutive case series of seven treatment-naïve patients with chronic myeloid leukaemia (n = 2), polycythemia vera...... (n = 4), essential thrombocytosis (n = 1) examined before and after cytoreductive treatment. We investigated retinal circulation with motion-contrast imaging, retinal oximetry and spectral-domain optical coherence tomography. RESULTS: Retinal venous blood velocity increased by 8.14% (CI95 3.67% to 12...

  10. An international consortium proposal of uniform response criteria for myelodysplastic/myeloproliferative neoplasms (MDS/MPN) in adults.

    Science.gov (United States)

    Savona, Michael R; Malcovati, Luca; Komrokji, Rami; Tiu, Ramon V; Mughal, Tariq I; Orazi, Attilio; Kiladjian, Jean-Jacques; Padron, Eric; Solary, Eric; Tibes, Raoul; Itzykson, Raphael; Cazzola, Mario; Mesa, Ruben; Maciejewski, Jaroslaw; Fenaux, Pierre; Garcia-Manero, Guillermo; Gerds, Aaron; Sanz, Guillermo; Niemeyer, Charlotte M; Cervantes, Francisco; Germing, Ulrich; Cross, Nicholas C P; List, Alan F

    2015-03-19

    Myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN) are hematologically diverse stem cell malignancies sharing phenotypic features of both myelodysplastic syndromes and myeloproliferative neoplasms. There are currently no standard treatment recommendations for most adult patients with MDS/MPN. To optimize efforts to improve the management and disease outcomes, it is essential to identify meaningful clinical and biologic end points and standardized response criteria for clinical trials. The dual dysplastic and proliferative features in these stem cell malignancies define their uniqueness and challenges. We propose response assessment guidelines to harmonize future clinical trials with the principal objective of establishing suitable treatment algorithms. An international panel comprising laboratory and clinical experts in MDS/MPN was established involving 3 independent academic MDS/MPN workshops (March 2013, December 2013, and June 2014). These recommendations are the result of this collaborative project sponsored by the MDS Foundation. © 2015 by The American Society of Hematology.

  11. TERT rs2736100 genotypes are associated with differential risk of myeloproliferative neoplasms in Swedish and Chinese male patient populations

    OpenAIRE

    Dahlstr?m, Jenny; Liu, Tiantian; Yuan, Xiaotian; Saft, Leonie; Ghaderi, Mehran; Wei, Ya Bin; Lavebratt, Catharina; Li, Ping; Zheng, Chengyun; Bj?rkholm, Magnus; Xu, Dawei

    2016-01-01

    The telomerase reverse transcriptase (TERT) gene rs2736100_C allele has recently been shown to be?associated with an increased risk for myeloproliferative neoplasms (MPNs) among?Caucasians. However, it is unknown if this association is present in other ethnical populations and whether rs2736100 allele frequencies mirror the incidence of MPNs in a population. Here we genotyped TERT rs2736100 variants in 126 Swedish and 101 Chinese MPN patients and their age-, sex-, and ethnically-matched healt...

  12. Critical appraisal of the role of ruxolitinib in myeloproliferative neoplasm-associated myelofibrosis

    Directory of Open Access Journals (Sweden)

    Barosi G

    2015-05-01

    Full Text Available Giovanni Barosi,1 Vittorio Rosti,1 Robert Peter Gale2 1Center for the Study of Myelofibrosis, IRCCS Policlinico S Matteo Foundation, Pavia, Italy; 2Haematology Research Centre, Division of Experimental Medicine, Department of Medicine, Imperial College London, London, UK Abstract: The recent approval of molecular-targeted therapies for myeloproliferative neoplasm-associated myelofibrosis (MPN-MF has dramatically changed its therapeutic landscape. Ruxolitinib, a JAK1/JAK2 tyrosine kinase inhibitor, is now widely used for first- and second-line therapy in persons with MPN-MF, especially those with disease-related splenomegaly, intermediate- or high-risk disease, and constitutional symptoms. The goal of this work is to critically analyze data supporting use of ruxolitinib in the clinical settings approved by the US Food and Drug Administration (FDA and European Medicines Agency (EMA. We systematically reviewed the literature and analyzed the risk of biases in the two randomized studies (COMFORT I and COMFORT II on which FDA and EMA approval was based. Our strategy was to apply the Grading of Recommendation, Assessment, Development and Evaluation (GRADE approach by evaluating five dimensions of evidence: (1 overall risk of bias, (2 imprecision, (3 inconsistency, (4 indirectness, and (5 publication bias. Based on these criteria, we downgraded the evidence from the COMFORT I and COMFORT II trials for performance, attrition, and publication bias. In the disease-associated splenomegaly sphere, we upgraded the quality of evidence because of large effect size but downgraded it because of comparator choice and outcome indirectness (quality of evidence, low. In the sphere of treating persons with intermediate- or high-risk disease, we downgraded the evidence because of imprecision in effect size measurement and population indirectness. In the sphere of disease-associated symptoms, we upgraded the evidence because of the large effect size, but downgraded it

  13. JAK2/IDH-mutant–driven myeloproliferative neoplasm is sensitive to combined targeted inhibition

    Science.gov (United States)

    McKenney, Anna Sophia; Somasundara, Amritha Varshini Hanasoge; Spitzer, Barbara; Intlekofer, Andrew M.; Ahn, Jihae; Shank, Kaitlyn; Rapaport, Franck T.; Patel, Minal A.; Papalexi, Efthymia; Shih, Alan H.; Chiu, April; Freinkman, Elizaveta; Akbay, Esra A.; Steadman, Mya; Nagaraja, Raj; Yen, Katharine; Teruya-Feldstein, Julie; Wong, Kwok-Kin; Rampal, Raajit; Thompson, Craig B.

    2018-01-01

    Patients with myeloproliferative neoplasms (MPNs) frequently progress to bone marrow failure or acute myeloid leukemia (AML), and mutations in epigenetic regulators such as the metabolic enzyme isocitrate dehydrogenase (IDH) are associated with poor outcomes. Here, we showed that combined expression of Jak2V617F and mutant IDH1R132H or Idh2R140Q induces MPN progression, alters stem/progenitor cell function, and impairs differentiation in mice. Jak2V617F Idh2R140Q–mutant MPNs were sensitive to small-molecule inhibition of IDH. Combined inhibition of JAK2 and IDH2 normalized the stem and progenitor cell compartments in the murine model and reduced disease burden to a greater extent than was seen with JAK inhibition alone. In addition, combined JAK2 and IDH2 inhibitor treatment also reversed aberrant gene expression in MPN stem cells and reversed the metabolite perturbations induced by concurrent JAK2 and IDH2 mutations. Combined JAK2 and IDH2 inhibitor therapy also showed cooperative efficacy in cells from MPN patients with both JAK2mut and IDH2mut mutations. Taken together, these data suggest that combined JAK and IDH inhibition may offer a therapeutic advantage in this high-risk MPN subtype. PMID:29355841

  14. Physical Activity as a Nonpharmacological Symptom Management Approach in Myeloproliferative Neoplasms: Recommendations for Future Research.

    Science.gov (United States)

    Eckert, Ryan; Huberty, Jennifer; Gowin, Krisstina; Mesa, Ruben; Marks, Lisa

    2017-12-01

    Essential thrombocythemia, polycythemia vera, and myelofibrosis are rare chronic hematological malignancies known as myeloproliferative neoplasms (MPNs) and are characterized by deregulated myeloid lineage cell production, splenomegaly, and heterogeneous symptom profiles. MPN patients suffer from a significant symptom burden (eg, fatigue, depressive symptoms, early satiety) and an impaired overall quality of life (QoL). Current treatments typically include pharmacological approaches, which may come with additional side effects and may be limited by treatment-associated toxicities (ie, cytopenias). Nonpharmacological approaches such as physical activity may be beneficial for reducing symptom burden and improving QoL. To date, no studies have examined physical activity as a nonpharmacological approach in MPN patients despite preliminary evidence supporting its benefit in other hematological cancers. The purpose of this article is to (1) review the literature related to physical activity and specific hematological cancer subtypes and to (2) make suggestions for future research involving physical activity in MPN patients as a symptom management strategy. A brief review of studies examining physical activity in leukemias, lymphomas, and myelomas (excluding stem-cell transplant patients) was conducted. There is preliminary evidence to suggest that physical activity may be an effective approach to improve patient-reported outcomes (fatigue, depression, anxiety, sleep), physical fitness (cardiovascular fitness, balance, body composition), and overall QoL in other hematological cancers. Based on encouraging findings in other hematological cancers, future research should examine the feasibility and effectiveness of physical activity in MPN patients.

  15. Splenectomy in patients with Myeloproliferative Neoplasms: efficacy, complications and impact on survival and transformation

    Science.gov (United States)

    Santos, Fabio P S; Tam, Constantine S; Kantarjian, Hagop; Cortes, Jorge; Thomas, Deborah; Pollock, Raphael; Verstovsek, Srdan

    2013-01-01

    Background Splenectomy may be an effective therapeutic option for treating massive splenomegaly in patients with myeloproliferative neoplasms (MPNs). There is still limited data on its short- and long-term benefits and risks. Methods Efficacy and short-term complications were analyzed in 94 patients with different MPNs who underwent splenectomy at MD Anderson. The long-term impact of splenectomy on overall survival (OS) and transformation free survival (TFS) was evaluated in 461 patients with myelofibrosis (MF) seen at MD Anderson including 50 who underwent splenectomy during disease evolution. Results Splenectomy improved anemia and thrombocytopenia in 47% and 66% of patients, respectively. Most common complications were leukocytosis (76%), thrombocytosis (43%), and venous thromboembolism (16%). Post-operative mortality was 5%. Among patients with MF, splenectomy during disease evolution was associated with decreased OS (Hazard Ratio [HR] =2.17, pSplenectomy is a possible therapeutic option for patients with MF and other MPNs, and its greatest benefits are related to improvement in spleen pain and discomfort, anemia and thrombocytopenia. However, in patients with MF it appears to be associated with increased mortality. PMID:23573823

  16. Myeloproliferative neoplasms (MPNs) have a significant impact on patients' overall health and productivity: the MPN Landmark survey.

    Science.gov (United States)

    Mesa, Ruben; Miller, Carole B; Thyne, Maureen; Mangan, James; Goldberger, Sara; Fazal, Salman; Ma, Xiaomei; Wilson, Wendy; Paranagama, Dilan C; Dubinski, David G; Boyle, John; Mascarenhas, John O

    2016-02-27

    The Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) negatively affect patient quality of life (QoL) and are associated with increased risk of mortality. The MPN Landmark survey was conducted from May to July 2014 in patients with MF, PV, or ET under active management in the United States. The survey assessed respondent perceptions of disease burden and treatment management and included questions on overall disease burden, QoL, activities of daily living, and work productivity. Outcomes were further analyzed by calculated (ie, not respondent-reported) prognostic risk score and symptom severity quartile. The survey was completed by 813 respondents (MF, n = 207; PV, n = 380; ET, n = 226). The median respondent age in each of the 3 MPN subtypes ranged from 62 to 66 years; median disease duration was 4 to 7 years. Many respondents reported that they had experienced MPN-related symptoms ≥1 year before diagnosis (MF, 49 %; PV, 61 %; ET, 58 %). Respondents also reported that MPN-related symptoms reduced their QoL, including respondents with low prognostic risk scores (MF, 67 %; PV, 62 %; ET, 57 %) and low symptom severity (MF, 51 %; PV, 33 %; ET, 15 %). Many respondents, including those with a low prognostic risk score, reported that their MPN had caused them to cancel planned activities or call in sick to work at least once in the preceding 30 days (cancel planned activities: MF, 56 %; PV, 35 %; ET, 35 %; call in sick: MF, 40 %; PV, 21 %; ET, 23 %). These findings of the MPN Landmark survey support previous research about the symptom burden experienced by patients with MPNs and are the first to detail the challenges that patients with MPNs experience related to reductions in activities of daily living and work productivity.

  17. Myeloproliferative neoplasms (MPNs) have a significant impact on patients’ overall health and productivity: the MPN Landmark survey

    International Nuclear Information System (INIS)

    Mesa, Ruben; Miller, Carole B.; Thyne, Maureen; Mangan, James; Goldberger, Sara; Fazal, Salman; Ma, Xiaomei; Wilson, Wendy; Paranagama, Dilan C.; Dubinski, David G.; Boyle, John; Mascarenhas, John O.

    2016-01-01

    The Philadelphia chromosome−negative myeloproliferative neoplasms (MPN) myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) negatively affect patient quality of life (QoL) and are associated with increased risk of mortality. The MPN Landmark survey was conducted from May to July 2014 in patients with MF, PV, or ET under active management in the United States. The survey assessed respondent perceptions of disease burden and treatment management and included questions on overall disease burden, QoL, activities of daily living, and work productivity. Outcomes were further analyzed by calculated (ie, not respondent-reported) prognostic risk score and symptom severity quartile. The survey was completed by 813 respondents (MF, n = 207; PV, n = 380; ET, n = 226). The median respondent age in each of the 3 MPN subtypes ranged from 62 to 66 years; median disease duration was 4 to 7 years. Many respondents reported that they had experienced MPN-related symptoms ≥1 year before diagnosis (MF, 49 %; PV, 61 %; ET, 58 %). Respondents also reported that MPN-related symptoms reduced their QoL, including respondents with low prognostic risk scores (MF, 67 %; PV, 62 %; ET, 57 %) and low symptom severity (MF, 51 %; PV, 33 %; ET, 15 %). Many respondents, including those with a low prognostic risk score, reported that their MPN had caused them to cancel planned activities or call in sick to work at least once in the preceding 30 days (cancel planned activities: MF, 56 %; PV, 35 %; ET, 35 %; call in sick: MF, 40 %; PV, 21 %; ET, 23 %). These findings of the MPN Landmark survey support previous research about the symptom burden experienced by patients with MPNs and are the first to detail the challenges that patients with MPNs experience related to reductions in activities of daily living and work productivity. The online version of this article (doi:10.1186/s12885-016-2208-2) contains supplementary material, which is available to authorized users

  18. Determining the role of inflammation in the selection of JAK2 mutant cells in myeloproliferative neoplasms.

    Science.gov (United States)

    Zhang, Jie; Fleischman, Angela G; Wodarz, Dominik; Komarova, Natalia L

    2017-07-21

    Myeloproliferative neoplasm (MPN) is a hematologic malignancy characterized by the clonal outgrowth of hematopoietic cells with a somatically acquired mutation most commonly in JAK2 (JAK2 V617F ). This mutation endows upon myeloid progenitors cytokine independent growth and consequently leads to excessive production of myeloid lineage cells. It has been previously suggested that inflammation may play a role in the clonal evolution of JAK2 V617F mutants. In particular, it is possible that one or more cellular kinetic parameters of hematopoietic stem cells (HSCs) are affected by inflammation, such as division or death rates of cells, and the probability of HSC differentiation. This suggests a mechanism that can steer the outcome of the cellular competition in favor of the mutants, initiating the disease. In this paper we create a number of mathematical evolutionary models, from very abstract to more concrete, that describe cellular competition in the context of inflammation. It is possible to build a model axiomatically, where only very general assumptions are imposed on the modeling components and no arbitrary (and generally unknown) functional forms are used, and still generate a set of testable predictions. In particular, we show that, if HSC death is negligible, the evolutionary advantage of mutant cells can only be conferred by an increase in differentiation probability of HSCs in the presence of inflammation, and if death plays a significant role in the dynamics, an additional mechanism may be an increase of HSC's division-to-death ratio in the presence of inflammation. Further, we show that in the presence of inflammation, the wild type cell population is predicted to shrink under inflammation (even in the absence of mutants). Finally, it turns out that if only the differentiation probability is affected by the inflammation, then the resulting steady state population of wild type cells will contain a relatively smaller percentage of HSCs under inflammation. If

  19. JAK inhibitors for the treatment of myeloproliferative neoplasms and other disorders [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    William Vainchenker

    2018-01-01

    Full Text Available JAK inhibitors have been developed following the discovery of the JAK2V617F in 2005 as the driver mutation of the majority of non-BCR-ABL1 myeloproliferative neoplasms (MPNs. Subsequently, the search for JAK2 inhibitors continued with the discovery that the other driver mutations (CALR and MPL also exhibited persistent JAK2 activation. Several type I ATP-competitive JAK inhibitors with different specificities were assessed in clinical trials and exhibited minimal hematologic toxicity. Interestingly, these JAK inhibitors display potent anti-inflammatory activity. Thus, JAK inhibitors targeting preferentially JAK1 and JAK3 have been developed to treat inflammation, autoimmune diseases, and graft-versus-host disease. Ten years after the beginning of clinical trials, only two drugs have been approved by the US Food and Drug Administration: one JAK2/JAK1 inhibitor (ruxolitinib in intermediate-2 and high-risk myelofibrosis and hydroxyurea-resistant or -intolerant polycythemia vera and one JAK1/JAK3 inhibitor (tofacitinib in methotrexate-resistant rheumatoid arthritis. The non-approved compounds exhibited many off-target effects leading to neurological and gastrointestinal toxicities, as seen in clinical trials for MPNs. Ruxolitinib is a well-tolerated drug with mostly anti-inflammatory properties. Despite a weak effect on the cause of the disease itself in MPNs, it improves the clinical state of patients and increases survival in myelofibrosis. This limited effect is related to the fact that ruxolitinib, like the other type I JAK2 inhibitors, inhibits equally mutated and wild-type JAK2 (JAK2WT and also the JAK2 oncogenic activation. Thus, other approaches need to be developed and could be based on either (1 the development of new inhibitors specifically targeting JAK2V617F or (2 the combination of the actual JAK2 inhibitors with other therapies, in particular with molecules targeting pathways downstream of JAK2 activation or the stability of JAK2

  20. Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: Prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs

    NARCIS (Netherlands)

    R.M. Emanuel (Robyn); A.C. Dueck (Amylou); H.L. Geyer (Holly); J.J. Kiladjian; S. Slot (Stefanie); S. Zweegman (Sonja); P.A.W. te Boekhorst (Peter); S. Commandeur (Suzan); H. Schouten (Harry); F. Sackmann (Federico); A.K. Fuentes (Ana Kerguelen); D. Hernández-Maraver (Dolores); C. Pahl (Clemens); M. Griesshammer (Martin); F. Stegelmann (Frank); K. Doehner (Konstanze); T. Lehmann (Thomas); K. Bonatz (Karin); A. Reiter (Alfred); F. Boyer (Francoise); J. Etienne (Jerome); J.-C. Ianotto (Jean-Christophe); D. Ranta (Dana); L. Roy (Lydia); J.-Y. Cahn (Jean-Yves); C.N. Harrison (Claire); D. Radia (Deepti); P. Muxi (Pablo); N. Maldonado (Norman); C. Besses (Carlos); F. Cervantes (Francisco); P.L. Johansson (Peter); T. Barbui (Tiziano); G. Barosi (Giovanni); A.M. Vannucchi (Alessandro); F. Passamonti (Francesco); B. Andreasson (Bjorn); M.L. Ferarri (Maria); A. Rambaldi (Alessandro); J. Samuelsson (Jan); G. Birgegard (Gunnar); A. Tefferi (Ayalew); A.A. Mesa

    2012-01-01

    textabstractPurpose: Myeloproliferative neoplasm (MPN) symptoms are troublesome to patients, and alleviation of this burden represents a paramount treatment objective in the development of MPN-directed therapies. We aimed to assess the utility of an abbreviated symptom score for the most pertinent

  1. Kinetics of del(7 q) driven leukemogenesis in a patient with JAK2 V617F and TET2 mutated chronic myeloproliferative neoplasm

    DEFF Research Database (Denmark)

    Herborg, Laura Laine; Nederby, Line; Kjeldsen, Eigil

    2013-01-01

    Chronic myeloid neoplasms have susceptibility to transform into acute myeloid leukemia due to attainment of additional molecular lesions. We here describe the kinetics of a del(7q) driven leukemogenesis in a patient with multiple TET2 mutations and JAK2 V617F mutated chronic myeloproliferative...

  2. A nationwide population-based cross-sectional survey of health-related quality of life in patients with myeloproliferative neoplasms in Denmark (MPNhealthSurvey)

    DEFF Research Database (Denmark)

    Brochmann Mortensen, Nana; Flachs, Esben Meulengracht; Christensen, Anne Illemann

    2017-01-01

    of patients with myeloproliferative neoplasms (MPNs). In Denmark, all MPN patients are treated in public hospitals and treatments received are free of charge for these patients. Therefore, MPN patients receive the best available treatment to the extent of its suitability for them and if they wish to receive...

  3. Social Media and Myeloproliferative Neoplasms (MPN): Analysis of Advanced Metrics From the First Year of a New Twitter Community: #MPNSM.

    Science.gov (United States)

    Pemmaraju, Naveen; Utengen, Audun; Gupta, Vikas; Kiladjian, Jean-Jacques; Mesa, Ruben; Thompson, Michael A

    2016-12-01

    The social media platform Twitter has provided the hematology/oncology community with unprecedented, novel methods of interpersonal communication and increased ability for the dissemination of important updates in a rapidly moving field. The advent, and subsequent success, of disease-specific Twitter communities have further enabled interested healthcare stakeholders to become quickly organized around a unique set of rare medical conditions, such as hematologic malignancies, that, historically, generally lack large amounts of reliable online information. One example is the Twitter community #MPNSM (myeloproliferative neoplasms on social media), which was started approximately one and half years ago and has served as a recognized venue for discussion among many members of the MPN community, including patients, researchers, providers, and advocacy organizations. This article will focus on understanding the impact of the founding of this community via the analysis of advanced Twitter metrics of user experience, from the first year of use for this novel healthcare hashtag.

  4. Mutation Analysis of JAK2V617F, FLT3-ITD, NPM1, and DNMT3A in Chinese Patients with Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Min Wang

    2014-01-01

    Full Text Available Since the discovery of JAK2V617F tyrosine kinase-activating mutation, several genes have been found mutated in myeloproliferative neoplasms (MPNs. FLT3-ITD, NPM1, and DNMT3A mutations frequently occurred in AML patients and have been found conferred with myeloproliferative neoplasms in mouse model. Therefore, we sought to search for mutations in JAK2V617F, FLT3-ITD, NPM1, and DNMT3A in 129 cases including 120 classic MPN cases and 9 MDS/MPN cases. JAK2V617F mutation was found in 60% of the 120 classic MPNs. However, none of the patients displayed FLT3-ITD and NPM1 mutations; only 2 patients harbored DNMT3A R882 mutation. Further studies including whole-genome sequence will be conducted to investigate the possible involvement of these genes in MPN.

  5. High frequency of endothelial colony forming cells marks a non-active myeloproliferative neoplasm with high risk of splanchnic vein thrombosis.

    Directory of Open Access Journals (Sweden)

    Vittorio Rosti

    Full Text Available Increased mobilization of circulating endothelial progenitor cells may represent a new biological hallmark of myeloproliferative neoplasms. We measured circulating endothelial colony forming cells (ECFCs in 106 patients with primary myelofibrosis, fibrotic stage, 49 with prefibrotic myelofibrosis, 59 with essential thrombocythemia or polycythemia vera, and 43 normal controls. Levels of ECFC frequency for patient's characteristics were estimated by using logistic regression in univariate and multivariate setting. The sensitivity, specificity, likelihood ratios, and positive predictive value of increased ECFC frequency were calculated for the significantly associated characteristics. Increased frequency of ECFCs resulted independently associated with history of splanchnic vein thrombosis (adjusted odds ratio = 6.61, 95% CI = 2.54-17.16, and a summary measure of non-active disease, i.e. hemoglobin of 13.8 g/dL or lower, white blood cells count of 7.8×10(9/L or lower, and platelet count of 400×10(9/L or lower (adjusted odds ratio = 4.43, 95% CI = 1.45-13.49 Thirteen patients with splanchnic vein thrombosis non associated with myeloproliferative neoplasms were recruited as controls. We excluded a causal role of splanchnic vein thrombosis in ECFCs increase, since no control had elevated ECFCs. We concluded that increased frequency of ECFCs represents the biological hallmark of a non-active myeloproliferative neoplasm with high risk of splanchnic vein thrombosis. The recognition of this disease category copes with the phenotypic mimicry of myeloproliferative neoplasms. Due to inherent performance limitations of ECFCs assay, there is an urgent need to arrive to an acceptable standardization of ECFC assessment.

  6. Mutations with epigenetic effects in myeloproliferative neoplasms and recent progress in treatment: Proceedings from the 5th International Post-ASH Symposium

    International Nuclear Information System (INIS)

    Tefferi, A; Abdel-Wahab, O; Cervantes, F; Crispino, J D; Finazzi, G; Girodon, F; Gisslinger, H; Gotlib, J; Kiladjian, J-J; Levine, R L; Licht, J D; Mullally, A; Odenike, O; Pardanani, A; Silver, R T; Solary, E; Mughal, T

    2011-01-01

    Immediately following the 2010 annual American Society of Hematology (ASH) meeting, the 5th International Post-ASH Symposium on Chronic Myelogenous Leukemia and BCR-ABL1-Negative Myeloproliferative Neoplasms (MPNs) took place on 7–8 December 2010 in Orlando, Florida, USA. During this meeting, the most recent advances in laboratory research and clinical practice, including those that were presented at the 2010 ASH meeting, were discussed among recognized authorities in the field. The current paper summarizes the proceedings of this meeting in BCR-ABL1-negative MPN. We provide a detailed overview of new mutations with putative epigenetic effects (TET oncogene family member 2 (TET2), additional sex comb-like 1 (ASXL1), isocitrate dehydrogenase (IDH) and enhancer of zeste homolog 2 (EZH2)) and an update on treatment with Janus kinase (JAK) inhibitors, pomalidomide, everolimus, interferon-α, midostaurin and cladribine. In addition, the new ‘Dynamic International Prognostic Scoring System (DIPSS)-plus' prognostic model for primary myelofibrosis (PMF) and the clinical relevance of distinguishing essential thrombocythemia from prefibrotic PMF are discussed

  7. The Hepatocyte Growth Factor (HGF)/Met Axis: A Neglected Target in the Treatment of Chronic Myeloproliferative Neoplasms?

    Energy Technology Data Exchange (ETDEWEB)

    Boissinot, Marjorie [Translational Neuro-Oncology Group, Leeds Institute of Cancer and Pathology, University of Leeds, Level 5 Wellcome Trust Brenner Building, St James’s Hospital, Leeds LS9 7TF (United Kingdom); Vilaine, Mathias [Institute of Research on Cancer and Aging (IRCAN), CNRS-Inserm-UNS UMR 7284, U 1081, Centre A. Lacassagne, 33 Avenue Valombrose, Nice 06189 (France); Hermouet, Sylvie, E-mail: sylvie.hermouet@univ-nantes.fr [Centre Hospitalier Universitaire (CHU), Place Alexis Ricordeau, Nantes 44093 (France); Inserm UMR892, Centre de Recherche en Cancérologie Nantes-Angers, Institut de Recherche en Santé, Université de Nantes, 8 quai Moncousu, Nantes cedex 44007 (France)

    2014-08-12

    Met is the receptor of hepatocyte growth factor (HGF), a cytoprotective cytokine. Disturbing the equilibrium between Met and its ligand may lead to inappropriate cell survival, accumulation of genetic abnormalities and eventually, malignancy. Abnormal activation of the HGF/Met axis is established in solid tumours and in chronic haematological malignancies, including myeloma, acute myeloid leukaemia, chronic myelogenous leukaemia (CML), and myeloproliferative neoplasms (MPNs). The molecular mechanisms potentially responsible for the abnormal activation of HGF/Met pathways are described and discussed. Importantly, inCML and in MPNs, the production of HGF is independent of Bcr-Abl and JAK2V617F, the main molecular markers of these diseases. In vitro studies showed that blocking HGF/Met function with neutralizing antibodies or Met inhibitors significantly impairs the growth of JAK2V617F-mutated cells. With personalised medicine and curative treatment in view, blocking activation of HGF/Met could be a useful addition in the treatment of CML and MPNs for those patients with high HGF/MET expression not controlled by current treatments (Bcr-Abl inhibitors in CML; phlebotomy, hydroxurea, JAK inhibitors in MPNs)

  8. The Hepatocyte Growth Factor (HGF/Met Axis: A Neglected Target in the Treatment of Chronic Myeloproliferative Neoplasms?

    Directory of Open Access Journals (Sweden)

    Marjorie Boissinot

    2014-08-01

    Full Text Available Met is the receptor of hepatocyte growth factor (HGF, a cytoprotective cytokine. Disturbing the equilibrium between Met and its ligand may lead to inappropriate cell survival, accumulation of genetic abnormalities and eventually, malignancy. Abnormal activation of the HGF/Met axis is established in solid tumours and in chronic haematological malignancies, including myeloma, acute myeloid leukaemia, chronic myelogenous leukaemia (CML, and myeloproliferative neoplasms (MPNs. The molecular mechanisms potentially responsible for the abnormal activation of HGF/Met pathways are described and discussed. Importantly, inCML and in MPNs, the production of HGF is independent of Bcr-Abl and JAK2V617F, the main molecular markers of these diseases. In vitro studies showed that blocking HGF/Met function with neutralizing antibodies or Met inhibitors significantly impairs the growth of JAK2V617F-mutated cells. With personalised medicine and curative treatment in view, blocking activation of HGF/Met could be a useful addition in the treatment of CML and MPNs for those patients with high HGF/MET expression not controlled by current treatments (Bcr-Abl inhibitors in CML; phlebotomy, hydroxurea, JAK inhibitors in MPNs.

  9. NUP98-HOXA9-transgenic zebrafish develop a myeloproliferative neoplasm and provide new insight into mechanisms of myeloid leukaemogenesis.

    Science.gov (United States)

    Forrester, A Michael; Grabher, Clemens; McBride, Eileen R; Boyd, Ellen R; Vigerstad, Märta H; Edgar, Alexander; Kai, Fui-Boon; Da'as, Sahar I; Payne, Elspeth; Look, A Thomas; Berman, Jason N

    2011-10-01

    NUP98-HOXA9 [t(7;11) (p15;p15)] is associated with inferior prognosis in de novo and treatment-related acute myeloid leukaemia (AML) and contributes to blast crisis in chronic myeloid leukaemia (CML). We have engineered an inducible transgenic zebrafish harbouring human NUP98-HOXA9 under the zebrafish spi1(pu.1) promoter. NUP98-HOXA9 perturbed zebrafish embryonic haematopoiesis, with upregulated spi1 expression at the expense of gata1a. Markers associated with more differentiated myeloid cells, lcp1, lyz, and mpx were also elevated, but to a lesser extent than spi1, suggesting differentiation of early myeloid progenitors may be impaired by NUP98-HOXA9. Following irradiation, NUP98-HOXA9-expressing embryos showed increased numbers of cells in G2-M transition compared to controls and absence of a normal apoptotic response, which may result from an upregulation of bcl2. These data suggest NUP98-HOXA9-induced oncogenesis may result from a combination of defects in haematopoiesis and an aberrant response to DNA damage. Importantly, 23% of adult NUP98-HOXA9-transgenic fish developed a myeloproliferative neoplasm (MPN) at 19-23 months of age. In summary, we have identified an embryonic haematopoietic phenotype in a transgenic zebrafish line that subsequently develops MPN. This tool provides a unique opportunity for high-throughput in vivo chemical modifier screens to identify novel therapeutic agents in high risk AML. © 2011 Blackwell Publishing Ltd.

  10. Allogeneic hematopoietic stem cell transplant in adult patients with myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) overlap syndromes.

    Science.gov (United States)

    Sharma, Prashant; Shinde, Shivani S; Damlaj, Moussab; Hefazi Rorghabeh, Mehrdad; Hashmi, Shahrukh K; Litzow, Mark R; Hogan, William J; Gangat, Naseema; Elliott, Michelle A; Al-Kali, Aref; Tefferi, Ayalew; Patnaik, Mrinal M

    2017-04-01

    MDS/MPN (myelodysplastic syndrome/myeloproliferative neoplasm) overlap syndromes are myeloid malignancies for which allogeneic hematopoietic stem cell transplant (allo-HSCT) is potentially curative. We describe transplant outcomes of 43 patients - 35 with chronic myelomonocytic leukemia, CMML (of which 17 had blast transformation, BT) and eight with MDS/MPN-unclassifiable (MDS/MPN,U). At median follow-up of 21 months, overall survival (OS), cumulative incidence of relapse (CIR) and non-relapse mortality (NRM) were 55%, 29%, and 25% respectively in CMML without BT and 47%, 40%, and 34% respectively in CMML with BT. Higher HSCT-comorbidity index (HSCT-CI >3 versus ≤3; p = 0.015) and splenomegaly (p = 0.006) predicted worse OS in CMML without BT. In CMML with BT, engraftment failure (p = 0.006) and higher HSCT-CI (p = 0.03) were associated with inferior OS, while HSCT within 1-year of diagnosis was associated with improved OS (p = 0.045). In MDS/MPN,U, at median follow-up of 15 months, OS, CIR, and NRM were 62%, 30%, and 14%, respectively.

  11. The role of thrombocytapheresis in the contemporary management of hyperthrombocytosis in myeloproliferative neoplasms: A case-based review.

    Science.gov (United States)

    Boddu, Prajwal; Falchi, Lorenzo; Hosing, Chitra; Newberry, Kate; Bose, Prithviraj; Verstovsek, Srdan

    2017-07-01

    Extreme thrombocytosis induces an acquired thrombotic-hemorrhagic diathesis, and left uncontrolled is a harbinger of potentially fatal vascular complications. Currently, cytoreduction with medical therapy remains the mainstay of hyperthrombocytosis management. However, it offers a less-than-ideal option in situations where a rapid reduction in platelets is urgently needed, as in the presence of vital end-organ ischemia or to ameliorate of life-threatening hemorrhage. The role of thrombocytapheresis, or plateletpheresis, in hyperthrombocytosis has become increasingly obsolete given the proactive titration of cytoreductive therapies and early identification and correction of reversible causes of reactive thrombocytosis. Despite its narrowed indications, plateletpheresis continues to offer a valuable temporizing measure in platelet count reduction before cytoreductive agents exert their maximal effect. In this context, it is important for the treating physician to be aware of the symptoms and risks associated with hyperthrombocytosis to inform best clinical practices. In this review, we discuss the role of plateletpheresis in the modern-day management of hyperthrombocytosis in patients with myeloproliferative neoplasms through a case based review of the literature. It becomes apparent throughout the discussion that the decision to perform plateletpheresis should be individualized based upon the clinical scenario, degree of thrombocytosis, available infrastructure and every patient's risk profile. Copyright © 2017 Elsevier Ltd. All rights reserved.

  12. The mevalonate pathway as a therapeutic target in the Ph-negative chronic myeloproliferative disorders

    DEFF Research Database (Denmark)

    Hasselbalch, Hans Carl; Riley, Caroline H

    2007-01-01

    The Ph-negative chronic myeloproliferative disorders (CMPDs) polycythaemia vera, essential thrombocytosis and idiopathic myelofibrosis are acquired stem cell disorders, which pathophysiologically are featured by clonal myeloproliferation and accumulation of myeloid cells, the latter being...... being partly explained by a proteolytic bone marrow mileu owing to excessive release of various proteases with ensuing extracellular matrix degradation and constitutive mobilisation of CD34+ cells into the peripheral blood. Thrombohaemorrhagic complications are major clinical problems contributing...

  13. Identification of myeloproliferative neoplasm drug agents via predictive simulation modeling: assessing responsiveness with micro-environment derived cytokines.

    Science.gov (United States)

    Kobayashi, Susumu S; Vali, Shireen; Kumar, Ansu; Singh, Neeraj; Abbasi, Taher; Sayeski, Peter P

    2016-06-14

    Previous studies have shown that the bone marrow micro-environment supports the myeloproliferative neoplasms (MPN) phenotype including via the production of cytokines that can induce resistance to frontline MPN therapies. However, the mechanisms by which this occurs are poorly understood. Moreover, the ability to rapidly identify drug agents that can act as adjuvants to existing MPN frontline therapies is virtually non-existent. Here, using a novel predictive simulation approach, we sought to determine the effect of various drug agents on MPN cell lines, both with and without the micro-environment derived inflammatory cytokines. We first created individual simulation models for two representative MPN cell lines; HEL and SET-2, based on their genomic mutation and copy number variation (CNV) data. Running computational simulations on these virtual cell line models, we identified a synergistic effect of two drug agents on cell proliferation and viability; namely, the Jak2 kinase inhibitor, G6, and the Bcl-2 inhibitor, ABT737. IL-6 did not show any impact on the cells due to the predicted lack of IL-6 signaling within these cells. Interestingly, TNFα increased the sensitivity of the single drug agents and their use in combination while IFNγ decreased the sensitivity. In summary, this study predictively identified two drug agents that reduce MPN cell viability via independent mechanisms that was prospectively validated. Moreover, their efficacy is either potentiated or inhibited, by some of the micro-environment derived cytokines. Lastly, this study has validated the use of this simulation based technology to prospectively determine such responses.

  14. A randomized study of pomalidomide vs placebo in persons with myeloproliferative neoplasm-associated myelofibrosis and RBC-transfusion dependence.

    Science.gov (United States)

    Tefferi, A; Al-Ali, H K; Barosi, G; Devos, T; Gisslinger, H; Jiang, Q; Kiladjian, J-J; Mesa, R; Passamonti, F; Ribrag, V; Schiller, G; Vannucchi, A M; Zhou, D; Reiser, D; Zhong, J; Gale, R P

    2017-04-01

    RBC-transfusion dependence is common in persons with myeloproliferative neoplasm (MPN)-associated myelofibrosis. The objective of this study was to determine the rates of RBC-transfusion independence after therapy with pomalidomide vs placebo in persons with MPN-associated myelofibrosis and RBC-transfusion dependence. Two hundred and fifty-two subjects (intent-to-treat (ITT) population) including 229 subjects confirmed by central review (modified ITT population) were randomly assigned (2:1) to pomalidomide or placebo. Trialists and subjects were blinded to treatment allocation. Primary end point was proportion of subjects achieving RBC-transfusion independence within 6 months. One hundred and fifty-two subjects received pomalidomide and 77 placebo. Response rates were 16% (95% confidence interval (CI), 11, 23%) vs 16% (8, 26%; P=0.87). Response in the pomalidomide cohort was associated with ⩽4 U RBC/28 days (odds ratio (OR)=3.1; 0.9, 11.1), age ⩽65 (OR=2.3; 0.9, 5.5) and type of MPN-associated myelofibrosis (OR=2.6; 0.7, 9.5). Responses in the placebo cohort were associated with ⩽4 U RBC/28 days (OR=8.6; 0.9, 82.3), white blood cell at randomization >25 × 10 9 /l (OR=4.9; 0.8, 28.9) and interval from diagnosis to randomization >2 years (OR=4.9; 1.1, 21.9). Pomalidomide was associated with increased rates of oedema and neutropenia but these adverse effects were manageable. Pomalidomide and placebo had similar RBC-transfusion-independence response rates in persons with MPN-associated RBC-transfusion dependence.

  15. The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis.

    Science.gov (United States)

    Pizzi, Marco; Gergis, Usama; Chaviano, Felicia; Orazi, Attilio

    2016-09-01

    Hematopoietic stem cell transplant (HSCT) is the only curative treatment for myeloproliferative neoplasm-associated myelofibrosis (MPN-MF). The main clinical manifestation of MPN-MF is splenomegaly secondary to extramedullary hematopoiesis (EMH). The effects of HSCT on splenic EMH and associated vascular and stromal changes are unknown. This study compares the findings seen in spleens following HSCT with those of nontransplanted patients, normal controls, and matched bone marrow (BM) samples. This study included three transplanted MPN-MF spleens, three nontransplanted MPN-MF spleens, and three normal controls. Spleens were assessed for: (a) presence/extent of EMH; (b) presence of Gamna-Gandy bodies; (c) splenic fibrosis; (d) CD34-positive microvessel density; (e) CD8-positive sinusoids; (f) frequency of smooth muscle actin-positive myoid cells; and (g) nerve growth factor receptor-positive adventitial reticulum cells. In two cases, matched BM samples were assessed for cellularity, presence of atypical megakaryocytes, and fibrosis. Compared with normal controls, all MPN-MF spleens were larger in size, had EMH, red pulp fibrosis, higher CD34-positive microvessel density, and decreased CD8-positive sinusoids. Compared with nontransplanted cases, post-HSCT spleens showed disappearance or reduction of EMH. Gamna-Gandy bodies were increased; no differences in the remaining parameters were found. A reduction of splenic EMH was associated with normalization of BM cellularity and megakaryopoiesis. HSCT reduces/abrogates splenic EMH and is associated with an increased number of Gamna-Gandy bodies, which may suggest vascular damage. The lack of stromal changes in spleens removed shortly after transplant is in line with similar observations in the BM, where a longer interval is often necessary for resolution of fibrosis. Copyright © 2016 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. All rights reserved.

  16. TERT rs2736100 genotypes are associated with differential risk of myeloproliferative neoplasms in Swedish and Chinese male patient populations.

    Science.gov (United States)

    Dahlström, Jenny; Liu, Tiantian; Yuan, Xiaotian; Saft, Leonie; Ghaderi, Mehran; Wei, Ya Bin; Lavebratt, Catharina; Li, Ping; Zheng, Chengyun; Björkholm, Magnus; Xu, Dawei

    2016-10-01

    The telomerase reverse transcriptase (TERT) gene rs2736100_C allele has recently been shown to be associated with an increased risk for myeloproliferative neoplasms (MPNs) among Caucasians. However, it is unknown if this association is present in other ethnical populations and whether rs2736100 allele frequencies mirror the incidence of MPNs in a population. Here we genotyped TERT rs2736100 variants in 126 Swedish and 101 Chinese MPN patients and their age-, sex-, and ethnically-matched healthy controls. Healthy Chinese adults had a higher frequency of the A allele and lower frequencies of the C allele compared to Swedish counterparts (57.4 vs 47.0 % for A, 42.6 vs 53.0 % for C, P = 0.006). Both Swedish and Chinese patients harbored significantly higher C allele frequency than their controls (62.7 vs 53.0 % and 57.4 vs 42.6 % for Swedish and Chinese, respectively, P = 0.004). Swedes and Chinese bearing the CC genotype had a significantly increased risk of MPN compared to AA carriers (OR = 2.47; 95 % CI: 1.33-4.57, P = 0.003, for Swedes, and OR = 3.45; 95 % CI: 1.52-7.85, P = 0.005, for Chinese). Further analyses showed that rs2736100_CC was associated with robustly enhanced risk in males only (CC vs AA, OR = 5.11; 95 % CI: 2.19-11.92, P frequency.

  17. A new internet-based tool for reporting and analysing patient-reported outcomes and the feasibility of repeated data collection from patients with myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Brochmann, Nana; Zwisler, Ann-Dorthe; Kjerholt, Mette

    2016-01-01

    University Hospital, Denmark. Validated questionnaires that were used were European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30, Myeloproliferative Neoplasm Symptom Assessment Form, Brief Fatigue Inventory and Short Form 36 Health Survey. Questionnaires were filled...... out ≥6 months online or on paper according to participant preference. Regularity of questionnaire submission was investigated, and participant acceptance was evaluated by focus-group interviews. RESULTS: Of 135 invited patients, 118 (87 %) accepted participation. One hundred and seven participants (91...

  18. A nationwide population-based cross-sectional survey of health-related quality of life in patients with myeloproliferative neoplasms in Denmark (MPNhealthSurvey)

    DEFF Research Database (Denmark)

    Brochmann, Nana; Flachs, Esben Meulengracht; Christensen, Anne Illemann

    2017-01-01

    of patients with myeloproliferative neoplasms (MPNs). In Denmark, all MPN patients are treated in public hospitals and treatments received are free of charge for these patients. Therefore, MPN patients receive the best available treatment to the extent of its suitability for them and if they wish to receive...... the treatment. The aims of this article are to describe the survey design and the characteristics of respondents and nonrespondents. MATERIAL AND METHODS: Individuals with MPN diagnoses registered in the Danish National Patient Register (NPR) were invited to participate. The registers of the Danish Civil...

  19. Improved diagnosis of the transition to JAK2 (V⁶¹⁷F homozygosity: the key feature for predicting the evolution of myeloproliferative neoplasms.

    Directory of Open Access Journals (Sweden)

    Mariana Selena Gonzalez

    Full Text Available Most cases of BCR-ABL1-negative myeloproliferative neoplasms (MPNs, essential thrombocythemia, polycythemia vera and primary myelofibrosis are associated with JAK2 (V617F mutations. The outcomes of these cases are critically influenced by the transition from JAK2 (V617F heterozygosity to homozygosity. Therefore, a technique providing an unbiased assessment of the critical allele burden, 50% JAK2 (V617F, is highly desirable. In this study, we present an approach to assess the JAK2 (V617F burden from genomic DNA (gDNA and complementary DNA (cDNA using one-plus-one template references for allele-specific quantitative-real-time-PCR (qPCR. Plasmidic gDNA and cDNA constructs encompassing one PCR template for JAK2 (V617F spaced from one template for JAK2(Wild Type were constructed by multiple fusion PCR amplifications. Repeated assessments of the 50% JAK2(V617F burden within the dynamic range of serial dilutions of gDNA and cDNA constructs resulted in 52.53 ± 4.2% and 51.46 ± 4.21%, respectively. The mutation-positive cutoff was estimated to be 3.65% (mean +2 standard deviation using 20 samples from a healthy population. This qPCR approach was compared with the qualitative ARMS-PCR technique and with two standard methods based on qPCR, and highly significant correlations were obtained in all cases. qPCR assays were performed on paired gDNA/cDNA samples from 20 MPN patients, and the JAK2 (V617F expression showed a significant correlation with the allele burden. Our data demonstrate that the qPCR method using one-plus-one template references provides an improved assessment of the clinically relevant transition of JAK2 (V617F from heterozygosity to homozygosity.

  20. The role of sexuality symptoms in myeloproliferative neoplasm symptom burden and quality of life: An analysis by the MPN QOL International Study Group.

    Science.gov (United States)

    Geyer, Holly L; Andreasson, Bjorn; Kosiorek, Heidi E; Dueck, Amylou C; Scherber, Robyn M; Martin, Kari A; Butler, Kristina A; Harrison, Claire N; Radia, Deepti H; Cervantes, Francisco; Kiladjian, Jean-Jacques; Reiter, Andreas; Birgegard, Gunnar; Passamonti, Francesco; Senyak, Zhenya; Vannucchi, Alessandro M; Paoli, Chiara; Xiao, Zhijian; Samuelsson, Jan; Mesa, Ruben A

    2016-06-15

    Patients with myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia, and myelofibrosis, are faced with oppressive symptom profiles that compromise daily functioning and quality of life. Among these symptoms, sexuality-related symptoms have emerged as particularly prominent and largely unaddressed. In the current study, the authors evaluated how sexuality symptoms from MPN relate to other patient characteristics, disease features, treatments, and symptoms. A total of 1971 patients with MPN (827 with essential thrombocythemia, 682 with polycythemia vera, 456 with myelofibrosis, and 6 classified as other) were prospectively evaluated and patient responses to the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) and the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC-QLQ C30) were collected, along with information regarding individual disease characteristics and laboratory data. Sexuality scores were compared with an age-matched, healthy control population. Overall, patients with MPN were found to have greater sexual dysfunction compared with the healthy population (MPN-SAF score of 3.6 vs 2.0; P65 years and in those with cytopenias and transfusion requirements, and those receiving certain therapies such as immunomodulators or steroids. The results of the current study identify the topic of sexuality as a prominent issue for the MPN population, and this area would appear to benefit from additional investigation and management. Cancer 2016;122:1888-96. © 2016 American Cancer Society. © 2016 American Cancer Society.

  1. Chronic Myeloproliferative Neoplasms Treatment

    Science.gov (United States)

    ... Types Bladder Cancer Breast Cancer Colorectal Cancer Kidney (Renal Cell) Cancer Leukemia Liver Cancer Lung Cancer Lymphoma Pancreatic Cancer Prostate Cancer Skin Cancer Thyroid Cancer Uterine Cancer All Cancer Types A to ...

  2. Myelodysplastic/ Myeloproliferative Neoplasms Treatment

    Science.gov (United States)

    ... symptoms of chronic myelomonocytic leukemia include fever, weight loss, and feeling very tired. Certain factors affect prognosis (chance of recovery) and treatment options. Chronic myelomonocytic leukemia ...

  3. Association of Oesophageal Varices and Splanchnic Vein Thromboses in Patients with JAK2-Positive Myeloproliferative Neoplasms: Presentation of Two Cases and Data from a Retrospective Analysis

    Directory of Open Access Journals (Sweden)

    Cornelia S. Link

    2013-06-01

    Full Text Available Background: Oesophageal varices and gastrointestinal bleeding are common complications of liver cirrhosis. More rarely, oesophageal varices occur in patients with non-cirrhotic portal hypertension that results from thromboses of portal or splanchnic veins. Case Report: We describe 2 young men who initially presented with varices as a result of portal vein thromboses. In the clinical follow-up, both were tested positive for a JAK2 mutation and consequently diagnosed with myeloproliferative neoplasms (MPNs. In an attempt to characterise the frequency of gastrointestinal complications in patients with JAK2-positive MPNs, we retrospectively analysed all known affected patients from our clinic for the diagnosis of portal vein thromboses and oesophageal varices. Strikingly, 48% of those who had received an oesophagogastroduodenoscopy had detectable oesophageal or gastric varices, and 82% of those suffered from portal or splanchnic vein thromboses. Conclusion: While the association between JAK2, myeloproliferative disease and thrombotic events is well established, patients with idiopathic oesophageal varices are not regularly tested for JAK2 mutations. However, the occurrence of oesophageal varices may be the first presenting symptom of a MPN with a JAK2 mutation, and affected patients may profit from a close haematological monitoring to assure the early detection of developing MPN.

  4. Gene expression profiling of loss of TET2 and/or JAK2V617F mutant hematopoietic stem cells from mouse models of myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Takuro Kameda

    2015-06-01

    Full Text Available Myeloproliferative neoplasms (MPNs are clinically characterized by the chronic overproduction of differentiated peripheral blood cells and the gradual expansion of malignant intramedullary/extramedullary hematopoiesis. In MPNs mutations in JAK2 MPL or CALR are detected mutually exclusive in more than 90% of cases [1,2]. Mutations in them lead to the abnormal activation of JAK/STAT signaling and the autonomous growth of differentiated cells therefore they are considered as “driver” gene mutations. In addition to the above driver gene mutations mutations in epigenetic regulators such as TET2 DNMT3A ASXL1 EZH2 or IDH1/2 are detected in about 5%–30% of cases respectively [3]. Mutations in TET2 DNMT3A EZH2 or IDH1/2 commonly confer the increased self-renewal capacity on normal hematopoietic stem cells (HSCs but they do not lead to the autonomous growth of differentiated cells and only exhibit subtle clinical phenotypes [4,6–8,5]. It was unclear how mutations in such epigenetic regulators influenced abnormal HSCs with driver gene mutations how they influenced the disease phenotype or whether a single driver gene mutation was sufficient for the initiation of human MPNs. Therefore we focused on JAK2V617F and loss of TET2—the former as a representative of driver gene mutations and the latter as a representative of mutations in epigenetic regulators—and examined the influence of single or double mutations on HSCs (Lineage−Sca-1+c-Kit+ cells (LSKs by functional analyses and microarray whole-genome expression analyses [9]. Gene expression profiling showed that the HSC fingerprint genes [10] was statistically equally enriched in TET2-knockdown-LSKs but negatively enriched in JAK2V617F–LSKs compared to that in wild-type-LSKs. Double-mutant-LSKs showed the same tendency as JAK2V617F–LSKs in terms of their HSC fingerprint genes but the expression of individual genes differed between the two groups. Among 245 HSC fingerprint genes 100 were more

  5. Myeloproliferative Neoplasm (MPN) Symptom Assessment Form Total Symptom Score: Prospective International Assessment of an Abbreviated Symptom Burden Scoring System Among Patients With MPNs

    Science.gov (United States)

    Emanuel, Robyn M.; Dueck, Amylou C.; Geyer, Holly L.; Kiladjian, Jean-Jacques; Slot, Stefanie; Zweegman, Sonja; te Boekhorst, Peter A.W.; Commandeur, Suzan; Schouten, Harry C.; Sackmann, Federico; Kerguelen Fuentes, Ana; Hernández-Maraver, Dolores; Pahl, Heike L.; Griesshammer, Martin; Stegelmann, Frank; Doehner, Konstanze; Lehmann, Thomas; Bonatz, Karin; Reiter, Andreas; Boyer, Francoise; Etienne, Gabriel; Ianotto, Jean-Christophe; Ranta, Dana; Roy, Lydia; Cahn, Jean-Yves; Harrison, Claire N.; Radia, Deepti; Muxi, Pablo; Maldonado, Norman; Besses, Carlos; Cervantes, Francisco; Johansson, Peter L.; Barbui, Tiziano; Barosi, Giovanni; Vannucchi, Alessandro M.; Passamonti, Francesco; Andreasson, Bjorn; Ferarri, Maria L.; Rambaldi, Alessandro; Samuelsson, Jan; Birgegard, Gunnar; Tefferi, Ayalew; Mesa, Ruben A.

    2012-01-01

    Purpose Myeloproliferative neoplasm (MPN) symptoms are troublesome to patients, and alleviation of this burden represents a paramount treatment objective in the development of MPN-directed therapies. We aimed to assess the utility of an abbreviated symptom score for the most pertinent and representative MPN symptoms for subsequent serial use in assessing response to therapy. Patients and Methods The Myeloproliferative Neoplasm Symptom Assessment Form total symptom score (MPN-SAF TSS) was calculated as the mean score for 10 items from two previously validated scoring systems. Questions focus on fatigue, concentration, early satiety, inactivity, night sweats, itching, bone pain, abdominal discomfort, weight loss, and fevers. Results MPN-SAF TSS was calculable for 1,408 of 1,433 patients with MPNs who had a mean score of 21.2 (standard deviation [SD], 16.3). MPN-SAF TSS results significantly differed among MPN disease subtypes (P < .001), with a mean of 18.7 (SD, 15.3), 21.8 (SD, 16.3), and 25.3 (SD, 17.2) for patients with essential thrombocythemia, polycythemia vera, and myelofibrosis, respectively. The MPN-SAF TSS strongly correlated with overall quality of life (QOL; r = 0.59; P < .001) and European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire C30 (EORTC QLQ-C30) functional scales (all P < .001 and absolute r ≥ 0.50 except social functioning r = 0.48). No significant trends were present when comparing therapy subgroups. The MPN-SAF TSS had excellent internal consistency (Cronbach's α = .83). Factor analysis identified a single underlying construct, indicating that the MPN-SAF TSS is an appropriate, unified scoring method. Conclusion The MPN-SAF TSS is a concise, valid, and accurate assessment of MPN symptom burden with demonstrated clinical utility in the largest prospective MPN symptom study to date. This new prospective scoring method may be used to assess MPN symptom burden in both clinical practice and trial settings. PMID

  6. Efficacy of NS-018, a potent and selective JAK2/Src inhibitor, in primary cells and mouse models of myeloproliferative neoplasms

    International Nuclear Information System (INIS)

    Nakaya, Y; Shide, K; Niwa, T; Homan, J; Sugahara, S; Horio, T; Kuramoto, K; Kotera, T; Shibayama, H; Hori, K; Naito, H; Shimoda, K

    2011-01-01

    Aberrant activation of Janus kinase 2 (JAK2) caused by somatic mutation of JAK2 (JAK2V617F) or the thrombopoietin receptor (MPLW515L) plays an essential role in the pathogenesis of myeloproliferative neoplasms (MPNs), suggesting that inhibition of aberrant JAK2 activation would have a therapeutic benefit. Our novel JAK2 inhibitor, NS-018, was highly active against JAK2 with a 50% inhibition (IC 50 ) of <1 n, and had 30–50-fold greater selectivity for JAK2 over other JAK-family kinases, such as JAK1, JAK3 and tyrosine kinase 2. In addition to JAK2, NS-018 inhibited Src-family kinases. NS-018 showed potent antiproliferative activity against cell lines expressing a constitutively activated JAK2 (the JAK2V617F or MPLW515L mutations or the TEL–JAK2 fusion gene; IC 50 =11–120 n), but showed only minimal cytotoxicity against most other hematopoietic cell lines without a constitutively activated JAK2. Furthermore, NS-018 preferentially suppressed in vitro erythropoietin-independent endogenous colony formation from polycythemia vera patients. NS-018 also markedly reduced splenomegaly and prolonged the survival of mice inoculated with Ba/F3 cells harboring JAK2V617F. In addition, NS-018 significantly reduced leukocytosis, hepatosplenomegaly and extramedullary hematopoiesis, improved nutritional status, and prolonged survival in JAK2V617F transgenic mice. These results suggest that NS-018 will be a promising candidate for the treatment of MPNs

  7. MECOM, HBS1L-MYB, THRB-RARB, JAK2, and TERT polymorphisms defining the genetic predisposition to myeloproliferative neoplasms: A study on 939 patients.

    Science.gov (United States)

    Trifa, Adrian P; Bănescu, Claudia; Bojan, Anca S; Voina, Cristian M; Popa, Ștefana; Vișan, Simona; Ciubean, Alina D; Tripon, Florin; Dima, Delia; Popov, Viola M; Vesa, Ștefan C; Andreescu, Mihaela; Török-Vistai, Tünde; Mihăilă, Romeo G; Berbec, Nicoleta; Macarie, Ioan; Coliţă, Andrei; Iordache, Maria; Cătană, Alina C; Farcaș, Marius F; Tomuleasa, Ciprian; Vasile, Kinga; Truică, Cristina; Todincă, Adriana; Pop-Muntean, Lavinia; Manolache, Raluca; Bumbea, Horia; Vlădăreanu, Ana-Maria; Gaman, Mihaela; Ciufu, Cristina M; Popp, Radu A

    2018-01-01

    Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are classical myeloproliferative neoplasms (MPN), characterized by specific somatic mutations in JAK2, CALR or MPL genes. JAK2 46/1 and TERT rs2736100 polymorphisms are known to significantly predispose to MPN. This study aimed to establish the additional contribution of the recently described MECOM rs2201862, HBS1L-MYB rs9376092 and THRB-RARB rs4858647 polymorphisms to the occurrence of MPN. These three polymorphisms, along with JAK2 46/1 and TERT rs2736100 were genotyped in 939 MPN patients (454 with ET, 337 with PV and 148 with PMF) and 483 controls. MECOM rs2201862 associated significantly with each MPN entity, except for ET, and with all major molecular sub-types, especially those CALR-mutated (OR = 1.4; 95% CI = 1.1-1.8; P-value = .005). HBS1L-MYB rs9376092 associated only with JAK2 V617F-mutated ET (OR = 1.4; 95% CI = 1.1-1.7; P-value = .003). THRB-RARB rs4858647 had a weak association with PMF only (OR = 1.5; 95% CI = 1-2.1; P-value = .04). Surprisingly, JAK2 46/1 haplotype was associated significantly not only with JAK2 V617F-mutated MPN, but also with CALR-mutated MPN (OR = 1.4; 95% CI = 1.1-1.8; P-value = .01). TERT rs2736100 was associated equally strong with all MPN, regardless of phenotype or molecular sub-type. In conclusion, JAK2 46/1, TERT rs2736100 and MECOM rs2201862 are the chief predisposing polymorphisms to MPN. © 2017 Wiley Periodicals, Inc.

  8. Rare Cancers and Social Media: Analysis of Twitter Metrics in the First 2 Years of a Rare-Disease Community for Myeloproliferative Neoplasms on Social Media-#MPNSM.

    Science.gov (United States)

    Pemmaraju, Naveen; Utengen, Audun; Gupta, Vikas; Kiladjian, Jean-Jacques; Mesa, Ruben; Thompson, Michael A

    2017-12-01

    The use of social media has now become a standard means of communication for many individuals worldwide. The use of one specific form of social media, Twitter, has increased among healthcare providers, both as a means of information gathering and as a conduit for original content creation. Recently, major efforts by users have been put forward to help streamline the unprecedented amount of information that can be found on Twitter. These efforts have led to the creation of diseasespecific hashtag (#) medical communities and have greatly enhanced the ability to understand and better categorize the available data on Twitter. Specifically, for those involved in rare cancer fields, adhering to organically designed and consistently used hashtags has led to the rapid, reliable dissemination of information and the ability to efficiently discuss and debate topics of interest in the field. For the field of myeloproliferative neoplasms (MPNs), the creation of #MPNSM (myeloproliferative neoplasms on social media) in 2015 has facilitated interactions among healthcare stakeholders from all over the world in the MPN field. In order to better understand the trends and topics of interest to Twitter users of this novel medical community, we conducted the present analysis which focuses on Twitter analytics from the first two years of #MPNSM. In this analysis, we observed a sustained increase in the number of Twitter users, number of tweets, number of impressions, and number of retweets over time, demonstrating the feasibility of creating and maintaining a disease-specific hashtag for a rare cancer over time.

  9. Prophylaxis and management of venous thromboembolism in patients with myeloproliferative neoplasms: consensus statement of the Haemostasis Working Party of the German Society of Hematology and Oncology (DGHO), the Austrian Society of Hematology and Oncology (ÖGHO) and Society of Thrombosis and Haemostasis Research (GTH e.V.).

    Science.gov (United States)

    Kreher, Stephan; Ochsenreither, Sebastian; Trappe, Ralf U; Pabinger, Ingrid; Bergmann, Frauke; Petrides, Petro E; Koschmieder, Steffen; Matzdorff, Axel; Tiede, Andreas; Griesshammer, Martin; Riess, Hanno

    2014-12-01

    Patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) like polycythemia vera and essential thrombocythemia are at increased risk of arterial and venous thrombosis. Strategies of prevention may consist of platelet aggregation inhibitors and/or cytoreductive agents depending on the underlying disease and the individual risk. Clinical evidence for management of acute venous thromboembolic events in MPN patients is limited. Modality and duration of therapeutic anticoagulation after venous thrombosis has to be evaluated critically with special regard to the increased risk for spontaneous bleeding events associated with the underlying diseases. Both for therapy of the acute event and for secondary prophylaxis, low-molecular-weight heparins should preferentially be used. A prolongation of the therapeutic anticoagulation beyond the usual 3 to 6 months can only be recommended in high-risk settings and after careful evaluation of potential risks and benefits for the individual patient. New direct oral anticoagulants (NOAC) should not preferentially be used due to lack of clinical experience in patients with MPN and potential drug interactions (e.g. with JAK inhibitors). Consequent treatment of the underlying myeloproliferative disease and periodical evaluation of the response to therapy is crucial for optimal secondary prophylaxis of thromboembolic events in those patients.

  10. An International MDS/MPN Working Group’s perspective and recommendations on molecular pathogenesis, diagnosis and clinical characterization of myelodysplastic/myeloproliferative neoplasms

    Science.gov (United States)

    Mughal, Tariq I.; Cross, Nicholas C.P.; Padron, Eric; Tiu, Ramon V.; Savona, Michael; Malcovati, Luca; Tibes, Raoul; Komrokji, Rami S.; Kiladjian, Jean-Jacques; Garcia-Manero, Guillermo; Orazi, Attilio; Mesa, Ruben; Maciejewski, Jaroslaw P.; Fenaux, Pierre; Itzykson, Raphael; Mufti, Ghulam; Solary, Eric; List, Alan F.

    2015-01-01

    In the 2008 WHO classification, chronic myeloid malignancies that share both myelodysplastic and myeloproliferative features define the myelodysplastic/myeloproliferative group, which includes chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, atypical chronic myeloid leukemia, refractory anemia with ring sideroblasts and thrombocytosis, and myelodysplastic/myeloproliferative unclassified. With the notable exception of refractory anemia with ring sideroblasts and thrombocytosis, there is much overlap among the various subtypes at the molecular and clinical levels, and a better definition of these entities, an understanding of their biology and an identification of subtype-specific molecular or cellular markers are needed. To address some of these challenges, a panel comprised of laboratory and clinical experts in myelodysplastic/myeloproliferative was established, and four independent academic MDS/MPN workshops were held on: 9th March 2013, in Miami, Florida, USA; 6th December 2013, in New Orleans, Louisiana, USA; 13th June 2014 in Milan, Italy; and 5th December 2014 in San Francisco, USA. During these meetings, the current understanding of these malignancies and matters of biology, diagnosis and management were discussed. This perspective and the recommendations on molecular pathogenesis, diagnosis and clinical characterization for adult onset myelodysplastic/myeloproliferative is the result of a collaborative project endorsed and supported by the MDS Foundation. PMID:26341525

  11. Recent advances in the bcr-abl negative chronic myeloproliferative diseases

    Directory of Open Access Journals (Sweden)

    Stroncek David F

    2006-10-01

    Full Text Available Abstract The chronic myeloproliferative disorders are clonal hematopoietic stem cell disorders of unknown etiology. In one of these (chronic myeloid leukemia, there is an associated pathognomonic chromosomal abnormality known as the Philadelphia chromosome. This leads to constitutive tyrosine kinase activity which is responsible for the disease and is used as a target for effective therapy. This review concentrates on the search in the other conditions (polycythemia vera, essential thrombocythemia and idiopathic mylofibrosis for a similar biological marker with therapeutic potential. There is no obvious chromosomal marker in these conditions and yet evidence of clonality can be obtained in females by the use of X-inactivation patterns. PRV-1mRNA over expression, raised vitamin B12 levels and raised neutrophil alkaline phosphatase scores are evidence that cells in these conditions have received excessive signals for proliferation, maturation and reduced apoptosis. The ability of erythroid colonies to grow spontaneously without added external erythropoietin in some cases, provided a useful marker and a clue to this abnormal signaling. In the past year several important discoveries have been made which go a long way in elucidating the involved pathways. The recently discovered JAK2 V617F mutation which occurs in the majority of cases of polycythemia vera and in about half of the cases with the two other conditions, enables constitutive tyrosine kinase activity without the need for ligand binding to hematopoietic receptors. This mutation has become the biological marker for these conditions and has spurred the development of a specific therapy to neutralize its effects. The realization that inherited mutations in the thrombopoietin receptor (c-Mpl can cause a phenotype of thrombocytosis such as in Mpl Baltimore (K39N and in a Japanese family with S505A, has prompted the search for acquired mutations in this receptor in chronic myeloproliferative

  12. Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable

    Science.gov (United States)

    ... symptoms of chronic myelomonocytic leukemia include fever, weight loss, and feeling very tired. Certain factors affect prognosis (chance of recovery) and treatment options. Chronic myelomonocytic leukemia ...

  13. Molecular diagnostics of myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Langabeer, S. E.; Andrikovics, H.; Asp, J.

    2015-01-01

    identified. These discoveries have been rapidly incorporated into evolving molecular diagnostic algorithms. Whilst many of these mutations appear to have prognostic implications, establishing MPN diagnosis is of immediate clinical importance with selection, implementation and the continual evaluation...... of the appropriate laboratory methodology to achieve this diagnosis similarly vital. The advantages and limitations of these approaches in identifying and quantitating the common MPN-associated mutations are considered herein with particular regard to their clinical utility. The evolution of molecular diagnostic...... applications and platforms has occurred in parallel with the discovery of MPN-associated mutations, and it therefore appears likely that emerging technologies such as next-generation sequencing and digital PCR will in the future play an increasing role in the molecular diagnosis of MPN....

  14. Clinical Characteristics of Cerebrovascular Pathology with Patients Suffering from Ph-Negative Myeloproliferative Disease

    Directory of Open Access Journals (Sweden)

    Marine M. Tanashyan

    2016-09-01

    Full Text Available Background: Disturbances of microcirculation play a significant role in the development and progression of both acute and chronic cerebrovascular diseases (CVD and may be associated with different hemogram abnormalities. One of the reasons of the prothrombogenic state of the endothelium is the increase in the number of blood corpuscles leading to (non-Ph myeloproliferative disorders (MPD including essential thrombocythemia (ET, polycythemia vera (PV, and primary myelofibrosis (PM. Materials and Methods: The study included 167 patients: 102 patients with Ph-MPD and the control group comprising 65 patients with CVD. According to MPD subtype, the patients were divided into three groups: patients with ET (37%, n = 38, male/female 7/31, age 52 ± 7 years, those with PV (40%, n = 41, male/female 20/21, age 50 ± 6 years and those with PM (23%, n = 23, male/female 5/18, age 54 ± 4 years. Results: In 79% (n = 81 of cases in the study group (with Ph-MPD, patients had chronic CVD, with the most frequently identified symptoms being asthenia (92% and headache (72%. Headache in Ph-MPD patients was more frequently (86% associated with PM, while in patients with PV and ET it was equally distributed (70%. Neurological symptoms in 53% of cases were associated with focal changes of the brain on MRI localized in the subcortical area of the frontal and parietal lobes. Twenty-one (21% patients suffered an acute cerebrovascular accident, 8 of them had thrombotic occlusion of one of the internal carotid arteries leading to hemispheric infarcts. Endothelial function (as measured by flow-dependent dilation of the brachial artery was severely impaired in all study groups (median 5% with normal cut-off at 10%, the lowest degree of vasodilator activity being specific for patients with a history of stroke (p = 0.011. Conclusion: Patients suffering from MPD had asymptomatic focal changes in the brain in the absence of concomitant vascular disease (hypertension

  15. Spontaneous T-cell responses against the immune check point programmed-death-ligand 1 (PD-L1) in patients with chronic myeloproliferative neoplasms correlate with disease stage and clinical response

    DEFF Research Database (Denmark)

    Holmström, Morten Orebo; Riley, Caroline Hasselbalch; Skov, Vibe

    2018-01-01

    The Chronic Myeloproliferative Neoplasms (MPN) are cancers characterized by hyperinflammation and immune deregulation. Concurrently, the expression of the immune check point programmed death ligand 1 (PD-L1) is induced by inflammation. In this study we report on the occurrence of spontaneous T cell...... responses against a PD-L1 derived epitope in patients with MPN. We show that 71% of patients display a significant immune response against PD-L1, and patients with advanced MPN have significantly fewer and weaker PD-L1 specific immune responses compared to patients with non-advanced MPN. The PD-L1 specific...... T cell responses are CD4+ T cell responses, and by gene expression analysis we show that expression of PD-L1 is enhanced in patients with MPN. This could imply that the tumor specific immune response in MPN could be enhanced by vaccination with PD-L1 derived epitopes by boosting the anti...

  16. [Development of the registry for Philadelphia-negative chronic myeloproliferative neoplasia in Hungary].

    Science.gov (United States)

    Dombi, Péter; Illés, Árpád; Demeter, Judit; Homor, Lajos; Simon, Zsófia; Udvardy, Miklós; Egyed, Miklós

    2016-01-17

    The establishment and operation of disease registry can be used to collect data on epidemiology cases. In addition, the registry can help to work out medical and health economical and political decisions for longer term. The aim of the authors was to collect and analyse data of patients with Philadelphia negative neoplasia in Hungary and draw conclusions about the basic types and features of the relevant disease. An online electronic data collection system has been established, based on the permission of the Regional and Institutional Committee of Science and Research Ethics obtained in April 8, 2013. Data collection has been initiated by hematology centres in Hungary. In addition to collection of the epidemiologic data, blood and bone marrow analysis data have been collected, as well. Also, based on cardiovascular factors, risk stratification has been established. Finally, the authors have investigated the method and practice of patient treatment in Hungary. Data of 901 patients from 15 Hungarian haematology centres have been recorded up to the date of June 30, 2015. After clarification of the data, 426 polycythaemia vera, 350 essential thrombocythaemia and 82 myelofibrosis cases were used for analysis. An online registry has been established which helps to clarify and analyse the basic features of certain medical cases and their treatment in Hungary. Including additional medical centres could help to improve the accuracy of medical analysis.

  17. International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) & European Competence Network on Mastocytosis (ECNM) consensus response criteria in advanced systemic mastocytosis

    NARCIS (Netherlands)

    Gotlib, Jason; Pardanani, Animesh; Akin, Cem; Reiter, Andreas; George, Tracy; Hermine, Olivier; Kluin-Nelemans, Hanneke; Hartmann, Karin; Sperr, Wolfgang R.; Brockow, Knut; Schwartz, Lawrence B.; Orfao, Alberto; DeAngelo, Daniel J.; Arock, Michel; Sotlar, Karl; Horny, Hans-Peter; Metcalfe, Dean D.; Escribano, Luis; Verstovsek, Srdan; Tefferi, Ayalew; Valent, Peter

    2013-01-01

    Systemic mastocytosis (SM) is characterized by accumulation of neoplastic mast cells and is classified into indolent and aggressive forms. The latter include aggressive SM (ASM), mast cell leukemia (MCL), and SM associated with a myeloid neoplasm wherein 1 or both disease compartments exhibit

  18. Neoplasias mieloproliferativas: revisão dos critérios diagnósticos e dos aspectos clínicos Myeloproliferative neoplasms: a review of diagnostic criteria and clinical aspects

    Directory of Open Access Journals (Sweden)

    Maria de Lourdes L. F. Chauffaille

    2010-01-01

    Full Text Available As síndromes mieloproliferativas crônicas, atualmente denominadas neoplasias mieloproliferativas (NMP, de acordo com a 4ª. edição da classificação da Organização Mundial da Saúde (OMS, são doenças clonais de célula-tronco hematopoética, nas quais há a proliferação aumentada de uma ou mais das séries mieloides (granulocítica, eritrocítica, megacariocítica ou mastocítica com maturação eficaz. A progressão de todas é caracterizada por fibrose medular ou transformação leucêmica. Pela classificação da OMS, as NMP incluem: leucemia mieloide crônica (LMC, policitemia vera (PV, mielofibrose idiopática crônica (MF, trombocitemia essencial (TE, leucemia neutrofílica crônica (LNC, leucemia eosinofílica crônica não especificada(LEC, mastocitose (M e neoplasia mieloproliferativa inclassificável (NMI. É interessante notar que tanto a LMC (BCR/ABL1 como PV, MF e TE (JAK2 V617F e éxon 12, MPLW515L/K e M (KITD816V tiveram suas bases moleculares desvendadas e apresentam em comum a ativação constitutiva de tirosino-quinase graças às mutações adquiridas pela célula-tronco hematopoética. A mutação JAK2 V617F é observada em mais de 90% dos casos de PV, mas também em cerca de 50%-60% das MF e TE, levando ao questionamento de como uma única lesão molecular desencadeia três manifestações clínicas diversas. Já há evidências de que eventos genéticos e epigenéticos adicionais contribuem para a patogênese, tais como MPLW515L e MPLW515K. No presente manuscrito são apresentados os aspectos clínicos, a fisiopatologia e os critérios diagnósticos das diferentes NMP.Chronic myeloproliferative disorders, currently called myeloproliferative neoplasms (MPN, according to the 4th edition of the World Health Organization (WHO classification are clonal diseases of hematopoietic stem cells, in which there is increased proliferation of the myeloid series (granulocytic, erythrocytic, megakaryocytic series or mast cells

  19. No development of neutralizing antibodies against recombinant interferon-alpha in Ph-negative myeloproliferative neoplasms-a prospective study

    DEFF Research Database (Denmark)

    Ocias, Lukas Frans; Lund Hansen, Dennis; Kielsgaard Kristensen, Thomas

    2015-01-01

    neutralizing antibodies (nAbs) against the drug leading to treatment failure. Most data on type 1 IFN immunogenicity are available from studies of patients with multiple sclerosis treated with rIFN-beta, and patients with hepatitis C treated with rIFN-alpha. A few reports have demonstrated nAbs in MPN patients......: ET: 67% CR, 29 % PR; PV: 64% CR, 31% PR (ELN 2009 criteria); PMF: 50% had at least a minor response (EUMNET). The median serum concentration of bioactive IFN-alpha at 12 months was 12,4 (range...

  20. EXEL-8232, a small-molecule JAK2 inhibitor, effectively treats thrombocytosis and extramedullary hematopoiesis in a murine model of myeloproliferative neoplasm induced by MPLW515L.

    Science.gov (United States)

    Wernig, G; Kharas, M G; Mullally, A; Leeman, D S; Okabe, R; George, T; Clary, D O; Gilliland, D G

    2012-04-01

    About 10% of patients with essential thrombocythemia (ET) or myelofibrosis (MF) that lack mutations in JAK2 harbor an activating mutation in the thrombopoietin receptor, MPLW515L. Distinct from the JAK2V617F retroviral transplant model, the MPLW515L model recapitulates many features of ET and MF, including severe fibrosis and thrombocytosis. We have tested EXEL-8232, an experimental potent JAK2 inhibitor, for efficacy in suppression of thrombocytosis in vivo and for its ability to attenuate MPLW515L myeloproliferative disease. EXEL-8232 was administered for 28 days q12 h by oral gavage at doses of 30 or 100 mg/kg, prospectively. Animals treated with EXEL-8232 at 100 mg/kg had normalized high platelet counts, eliminated extramedullary hematopoiesis in the spleen and eliminated bone marrow fibrosis, whereas the wild-type controls did not develop thrombocytopenia. Consistent with a clinical response in this model, we validated surrogate end points for response to treatment, including a reduction of endogenous colony growth and signaling inhibition in immature erythroid and myeloid primary cells both in vitro and upon treatment in vivo. We conclude that EXEL-8232 has efficacy in treatment of thrombocytosis in vivo in a murine model of ET and MF, and may be of therapeutic benefit for patients with MPL-mutant MPN.

  1. [Epidemiologic features of Philadelphia-negative chronic myeloproliferative disorders in Szabolcs-Szatmár-Bereg county, Hungary. Analysis of data of a 33-year period].

    Science.gov (United States)

    Jakó, János; Szerafin, László

    2017-04-01

    In their previous work, the authors reported findings from 30 years on the incidence of hematological malignancies in Szabolcs-Szatmár-Bereg county, Hungary. Until now there are no other studies on this topic available in Hungary. Detailed analysis of epidemiologic features of patients with Philadelphia-negative chronic myeloproliferative disorders was carried out. During a 33-year period (between January 1, 1983 and December 31, 2015) 4523 adult patients with hematologic malignancies were recorded in the leukaemia/lymphoma registry of Szabolcs-Szatmár-Bereg county. Among them, 255 patients with polycytaemia vera, 102 with primary myelofibrosis, and 331 with essential thrombocytaemia were registered. The incidence of polycythaemia vera and essential thrombocythaemia in Szabolcs-Szatmár-Bereg county showed an increasing tendency, with an overall incidence rate of 1.35 and 1.75/100 000 inhabitants/year, respectively; while the incidence of primary myelofibrosis decreased in the course of years (0.54/100 000 inhabitants/year). In cases of polycythaemia vera and primary myelofibrosis the male:female ratio was found to be equal, however essential thrombocythaemia showed a female dominance. The mean age of patients with polycythaemia vera was 65 (21-95) years, similar to essential thrombocythaemia with 65 (19-85) years, and to primary myelofibrosis with 65.5 (33-84) years. There were only two villages found in this county where the occurrence of patients with Philadelphia-negative chronic myeloproliferative disorders per one thousand inhabitants was significantly higher, than the average (1.22). In every familial cases of these, the manifestation of the disease in the second and the third generations became earlier than in the first genetration. The perceived average degree of the anteposition (anticipation) was found to be 22 years. The epidemiologic features of Philadelphia-negative chronic myeloproliferative disorders in Szabolcs-Szatmár-Bereg county are

  2. Differences in treatment goals and perception of symptom burden between patients with myeloproliferative neoplasms (MPNs) and hematologists/oncologists in the United States: Findings from the MPN Landmark survey.

    Science.gov (United States)

    Mesa, Ruben A; Miller, Carole B; Thyne, Maureen; Mangan, James; Goldberger, Sara; Fazal, Salman; Ma, Xiaomei; Wilson, Wendy; Paranagama, Dilan C; Dubinski, David G; Naim, Ahmad; Parasuraman, Shreekant; Boyle, John; Mascarenhas, John O

    2017-02-01

    This analysis of the myeloproliferative neoplasm (MPN) Landmark survey evaluated gaps between patient perceptions of their disease management and physician self-reported practices. The survey included 813 patient respondents who had MPNs (myelofibrosis [MF], polycythemia vera [PV], or essential thrombocythemia [ET]) and 457 hematologist/oncologist respondents who treated patients with these conditions. Greater proportions of physician respondents reported using prognostic risk classifications (MF, 83%; PV, 59%; ET, 77%) compared with patient recollections (MF, 54%; PV, 17%; ET, 31%). Most physician respondents reported that their typical symptom assessments included asking patients about the most important symptoms or a full list of symptoms, whereas many patient respondents reported less specific assessments (eg, they were asked how they were feeling). Many patient respondents did not recognize common symptoms as MPN-related. For example, approximately one-half or more did not believe difficulty sleeping resulted from their MPN (MF, 49%; PV, 64%; ET, 76%). Physician respondents underestimated the proportion of patients who had symptomatic PV or ET at diagnosis compared with patient respondents. There was discordance regarding treatment goals: among patient respondents with MF or PV, "slow/delay progression of condition" was the most important treatment goal, whereas physician respondents reported "symptom improvement" and "prevention of vascular/thrombotic events," respectively. Finally, more than one-third of patient respondents were not "very satisfied" with their physician's overall management/communication. The care and satisfaction of patients with MPN may be improved with increased patient education and improved patient-physician communication. Cancer 2017;123:449-458. © 2016 The Authors. Cancer published by Wiley Periodicals, Inc. on behalf of American Cancer Society. © 2016 The Authors. Cancer published by Wiley Periodicals, Inc. on behalf of American

  3. Objective, planimetry-based assessment of megakaryocyte histological pictures in Philadelphia-chromosome-negative chronic myeloproliferative disorders: a perspective for a valuable adjunct diagnostic tool.

    Science.gov (United States)

    Rudzki, Zbigniew; Kawa, Rafał; Okoñ, Krzysztof; Szczygieł, Ewa; Stachura, Jerzy

    2006-01-01

    Philadelphia-chromosome-negative chronic myeloproliferative disorders (Ph- CMPDs)--essential thrombocythemia (ET), chronic idiopathic myelofibrosis (CIMF), and polycythemia vera (PV)--may show clinical and morphological similarities, particularly at the early stages. The differential diagnosis of Ph- CMPDs is important due to their different treatment and prognosis. Cytological features of megakaryocytes are considered valuable in this differentiation. To establish an objective measure of megakaryocyte dysplasia in Ph- CMPDs, we performed computer-assisted morphometry of more than 4,000 cells from 20 cases of ET, 10 of CIMF, 10 of PV, and 10 controls. Megakaryocyte sets from three Ph- CMPDs differed significantly in respect to many planimetric parameters, but not a single shape or size parameter could have been used as a discriminative tool between the entities. However, the discriminant function analysis with the simultaneous assessment of 12 planimetric variables allowed for a proper classification of 20 of 20 ET, 10 of 10 PV, and 9 of 10 CIMF cases based solely on the morphometric features of megakaryocytes. Additionally, we identified certain new patterns of megakaryocytes specific for ET, PV, and CIMF, which, although not dominating in one Ph- CMPD, are unlikely to occur in two others. Objective measurements of megakaryocyte sizes and shapes may assist the diagnosis of Ph- CMPDs.

  4. WHO classification 2008 of myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Madelung, Ann B; Bondo, Henrik; Stamp, Inger

    2015-01-01

    .004). There was no significant effect on the isolated morphological characteristics. Agreement between cases with histological consensus and clinical diagnosis was 86% without significant change during workshop sessions. Our study demonstrates that experienced hematopathologists can significantly improve their diagnostic...

  5. Treatment Options for Myelodysplastic/Myeloproliferative Neoplasms

    Science.gov (United States)

    ... symptoms of chronic myelomonocytic leukemia include fever, weight loss, and feeling very tired. Certain factors affect prognosis (chance of recovery) and treatment options. Chronic myelomonocytic leukemia ...

  6. General Information about Myelodysplastic/Myeloproliferative Neoplasms

    Science.gov (United States)

    ... symptoms of chronic myelomonocytic leukemia include fever, weight loss, and feeling very tired. Certain factors affect prognosis (chance of recovery) and treatment options. Chronic myelomonocytic leukemia ...

  7. Treatment Option Overview (Myelodysplastic/Myeloproliferative Neoplasms)

    Science.gov (United States)

    ... symptoms of chronic myelomonocytic leukemia include fever, weight loss, and feeling very tired. Certain factors affect prognosis (chance of recovery) and treatment options. Chronic myelomonocytic leukemia ...

  8. Treatment Options for Chronic Myeloproliferative Neoplasms

    Science.gov (United States)

    ... response ) and help fight infections caused by certain parasites . In chronic eosinophilic leukemia , there are too many eosinophils in the blood , bone marrow , and other tissues . Chronic eosinophilic leukemia ...

  9. Treatment Option Overview (Chronic Myeloproliferative Neoplasms)

    Science.gov (United States)

    ... response ) and help fight infections caused by certain parasites . In chronic eosinophilic leukemia , there are too many eosinophils in the blood , bone marrow , and other tissues . Chronic eosinophilic leukemia ...

  10. MYELODYSPLASTIC/MYELOPROLIFERATIVE DISEASES

    OpenAIRE

    I. N. Subortseva; A. I. Melikyan

    2016-01-01

    Chronic myeloid malignancies that have characteristics of both the myelodysplastic and myeloproliferative disorders allocated to myelodysplastic/myeloproliferative diseases (MDS/MPD) group in 2008 World Health Organization classification. This group includes chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, atypical chronic myeloid leukemia, refractory anemia with ringed sideroblasts and thrombocytosis (RARS-T) and unclassified MDS/MPD. Except RARS-T diseases in this group ha...

  11. MYELODYSPLASTIC/MYELOPROLIFERATIVE DISEASES

    Directory of Open Access Journals (Sweden)

    I. N. Subortseva

    2016-01-01

    Full Text Available Chronic myeloid malignancies that have characteristics of both the myelodysplastic and myeloproliferative disorders allocated to myelodysplastic/myeloproliferative diseases (MDS/MPD group in 2008 World Health Organization classification. This group includes chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, atypical chronic myeloid leukemia, refractory anemia with ringed sideroblasts and thrombocytosis (RARS-T and unclassified MDS/MPD. Except RARS-T diseases in this group have similar molecular characteristics and clinical manifestations, which require the study of biology, specific molecular markers, morphological features and a clearer definition of nosology. This review provides information on international guidelines for the diagnosis and treatment of MDS/MPD.

  12. Increase in circulating CD4CD25Foxp3 T cells in patients with Philadelphia-negative chronic myeloproliferative neoplasms during treatment with IFN-α

    DEFF Research Database (Denmark)

    Riley, C.H.; Morten Krogh, Jensen; Brimnes, M.K.

    2011-01-01

    in these patients. Foxp3 regulatory T cells play a pivotal role in maintaining immune homeostasis and, importantly, preventing immune reactivity to self-antigens; however, their suppressive activity can compromise an effective antitumor immune response, and high frequencies of regulatory T cells in peripheral blood......Recent reports have described complete or major molecular remission in patients with polycythemia vera after long-term treatment with the immunomodulatory agent IFN-α2. Accordingly, there are reasons to believe that the immune system is a key player in eradicating the JAK2 mutated clone...

  13. World Health Organization-defined classification of myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Madelung, Ann Brinch; Bondo, Henrik; Stamp, Inger

    2013-01-01

    marrow biopsies including 43 controls. Diagnoses were determined according to the 2008 criteria of the World Health Organization (WHO). The participants were blinded to all clinical data except patient age. After initial evaluation all hematopathologists participated in a 3-day meeting with a leading...

  14. Inflammation as a Driver of Clonal Evolution in Myeloproliferative Neoplasm

    Science.gov (United States)

    2017-10-01

    concentrations but these effects can be restored by increasing the concentration of IL- 10. Together, these data suggest that administration of IL-10 may reduce...to IL-10 concentration, providing support for the therapeutic use of exogenous administration of high doses of IL-10 to reduce inflammation in MPN...commercial firms, state or local governments, schools or school systems, or other organizations (foreign or domestic) - that were involved with the project

  15. Group G streptococcal myositis in a patient with myeloproliferative neoplasm

    Directory of Open Access Journals (Sweden)

    Monica Midha, MD MBS

    2016-01-01

    Full Text Available While many cases of streptococcal infection are due to Lancefield groups A and B, there has been a rise in reported cases of infections due to group G streptococcus. We present a case of an individual with a hematologic malignancy who developed myositis secondary to group G streptococcus, with no clearly identifiable source of infection. The patient was managed with antibiotic therapy rather than surgical intervention due to high surgical risk related to severe thrombocytopenia. Targeted antibiotics initiated early in the course of disease may prevent the need for surgical intervention. Early diagnosis and treatment are critical to avoid the high morbidity and mortality of life-threatening infections caused by group G streptococcus.

  16. Colon neoplasm

    International Nuclear Information System (INIS)

    Kimura F, K.

    1991-01-01

    The main aspects of colon neoplasms are described, including several factors that predispose the disease, the occurrence, the main biomedical radiography and the evaluation after the surgery. (C.G.C.)

  17. Myeloid Neoplasms.

    Science.gov (United States)

    Subtil, Antonio

    2017-09-01

    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). Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Vascular neoplasms.

    Science.gov (United States)

    Williams, H B

    1980-07-01

    Vascular neoplasms in the broad sense represent a very common group of tumors or hamartomas that show great variability in gross appearance, microscopic appearance, and clinical course. Generally, neoplasms are composed of one cell type, but vascular neoplasms are collections of endothelial-lined tubes or tubules with connective tissue walls that may contain smooth muscle cells, pericytes, and nerve elements according to the specific tissues of origin. The classification of vascular neoplasms as outlined in this article attempts to delineate each tumor or hamartoma according to its histologic appearance and clinical behavior. The clinical course ranges from completely benign, self-involuting malformations such as the strawberry hemangioma to highly malignant angiosarcomas with their rapid growth and frequent metastases. Defects in the lymphatic system show gradations from simple lymphangiomas through lymphedema and lymphangiectasia, which can probably be explained by faulty embryologic development. Management of these lesions has been discussed, including brief descriptions of most of the currently accepted treatment methods for these frequently encountered clinical problems.

  19. Malignant lymphomas (including myeloproliferative disorders)

    International Nuclear Information System (INIS)

    Todd, I.D.H.

    1985-01-01

    This chapter deals with the radiotherapy and cytotoxic chemotherapy of the malignant lymphomas. Included within this group are Hodgkin's disease, non-Hodgkin's lymphoma, mycosis fungoides, and chronic lymphatic leukaemia. A further section deals with the myeloproliferative disorders, including granulocytic leukaemia, polycythaemia vera, and primary thrombocythaemia. Excluded are myeloma and reticulum cell sarcoma of bone and acute leukaemia. With regard to Hodgkin's disease, the past 25 years have seen general recognition of the curative potential of radiotherapy, at least in the local stages, and, more recently, awareness of the ability to achieve long-term survival after combination chemotherapy in generalised or in recurrent disease. At the same time the importance of staging has become appreciated and the introduction of procedures such as lymphography, staging laparotomy, and computer tomography (CT) has enormously increased its reliability. Advances have not been so dramatic in the complex group of non-Hodgkins's lymphomas, but are still very real

  20. Increase in circulating CD4⁺CD25⁺Foxp3⁺ T cells in patients with Philadelphia-negative chronic myeloproliferative neoplasms during treatment with IFN-α

    DEFF Research Database (Denmark)

    Riley, Caroline Hasselbalch; Jensen, Morten Krogh; Brimnes, Marie Klinge

    2011-01-01

    in these patients. Foxp3(+) regulatory T cells play a pivotal role in maintaining immune homeostasis and, importantly, preventing immune reactivity to self-antigens; however, their suppressive activity can compromise an effective antitumor immune response, and high frequencies of regulatory T cells in peripheral......Recent reports have described complete or major molecular remission in patients with polycythemia vera after long-term treatment with the immunomodulatory agent IFN-a2. Accordingly, there are reasons to believe that the immune system is a key player in eradicating the JAK2 mutated clone...

  1. Nye molekylaere markører ved de kroniske myeloproliferative sygdomme

    DEFF Research Database (Denmark)

    Larsen, Thomas Stauffer; Pallisgaard, Niels; Christensen, Jacob Haaber

    2006-01-01

    The Philadelphia-negative chronic myeloproliferative disorders feature autonomous myeloid hyperproliferation and hypersensitivity to a number of growth factors, which most recently have been shown to be explained by a guanine-to-thymidine mutation in the Janus tyrosine kinase (JAK2) gene, implica...

  2. Molecular genetics and cytogenetics of myeloproliferative disorders.

    Science.gov (United States)

    Bench, A J; Nacheva, E P; Champion, K M; Green, A R

    1998-12-01

    The myeloproliferative disorders are believed to represent clonal malignancies resulting from transformation of a pluripotent stem cell. X-inactivation patterns of peripheral blood cells have been proposed as a useful diagnostic tool but this method is limited by the finding of a clonal X-inactivation pattern in a significant proportion of normal elderly women. There is no pathognomonic chromosomal abnormality associated with the myeloproliferative disorders. However, consistent acquired cytogenetic changes include del(20q), del(13q), trisomy 8 and 9 and duplication of segments of 1q, all of which have been observed at diagnosis or before cytoreductive therapy and therefore represent early lesions which contribute to the pathogenesis of these disorders. Although, the acquired molecular defects underlying most myeloproliferative disorders have not yet been elucidated, translocations associated with the rare 8p11 syndrome have permitted identification of a novel fusion protein. The role of a number of candidate genes in the other myeloproliferative disorders has also been studied, but no mutations have been identified so far. It is likely that a number of genes will be involved, given the varied phenotypes of the diseases. Identification of causal genes will be of considerable interest to both clinicians, who currently lack a specific and sensitive diagnostic test, and scientists interested in fundamental issues of stem cell behaviour.

  3. Outcomes following splenectomy in patients with myeloid neoplasms.

    Science.gov (United States)

    Rialon, Kristy L; Speicher, Paul J; Ceppa, Eugene P; Rendell, Victoria R; Vaslef, Steven N; Beaven, Anne; Tyler, Douglas S; Blazer, Dan G

    2015-03-15

    Myeloid neoplasms are classified into five major categories. These patients may develop splenomegaly and require splenectomy to alleviate mechanical symptoms, to ameliorate transfusion-dependent cytopenias, or to enhance stem cell transplantation. The objective of this study was to determine which clinical variables significantly impacted morbidity, mortality, and survival in patients with myeloid neoplasms undergoing splenectomy, and to determine if operative outcomes have improved over time. The records of all patients with myeloid neoplasms undergoing splenectomy from 1993 to 2010 were retrospectively reviewed. Eighty-nine patients (n = 89) underwent splenectomy for myeloid neoplasms. Over half of patients who had symptoms preoperatively had resolution of their symptoms post-splenectomy. The morbidity rate was 38%, with the most common complications being bleeding (14%) or infection (20%). Thirty-day mortality rate was 18% and median survival after splenectomy was 278 days. Decreased survival was associated with a diagnosis of myelodysplastic syndrome/myeloproliferative neoplasm, anemia, abnormal white blood cell count, and hypoalbuminemia. Patients who underwent stem cell transplantation did not show an increased risk for morbidity or mortality. Patients with myeloid neoplasms have a poor prognosis after splenectomy and the decision to operate is a difficult one, associated with high morbidity and mortality. © 2014 Wiley Periodicals, Inc.

  4. Myeloid neoplasms in the World Health Organization 2016 classification.

    Science.gov (United States)

    Asou, Norio

    In the 2016 revision of the World Health Organization (WHO) classification, the categories of myeloid neoplasms have not been revised significantly from the 2008 fourth edition. However, recent discovery of molecular abnormalities provides a new perspective regarding the diagnostic and prognostic markers. In myeloproliferative neoplasms, the identification of CALR gene mutation, in addition to the JAK2 and MPL mutations, has impacted the diagnostic criteria. In myelodysplastic syndromes and acute myeloid leukemia, in addition to alterations in the transcription factors and signal transduction pathways, discovery of gene mutations in the epigenetic regulators that are involved in DNA methylation, histone modification, cohesin complex, and RNA splicing, by comprehensive genetic analyses, has improved our understanding of the pathobiology of these diseases. Moreover, recent large-scale sequencing studies have revealed the acquisition of clonal somatic mutations, in the myeloid neoplasm-associated genes of the hematopoietic cells. Such mutations were detected in people with normal blood cell counts, without any apparent disease. Presence of these mutations confers an increased risk for subsequent hematological neoplasms, indicating the concept of clonal hematopoiesis of indeterminate potential. This updated WHO classification incorporates the criteria of new clinical, prognostic, morphologic, immunophenotypic, and genetic findings in myeloid neoplasms.

  5. Megakaryocytic features useful for the diagnosis of myeloproliferative disorders can be obtained by a novel unsupervised software analysis

    OpenAIRE

    Tripodo, C.; Valenti, C.; Ballarò, B.; Rudzki, Z.; Tegolo, D.; Di Gesù, V.; Florena, A.M.; Franco, V.

    2006-01-01

    An unsupervised method for megakaryocyte detection and analysis is proposed, in order to validate supplementary tools which can be of help in supporting the pathologist in the classification of Philadelphia negative chronic myeloproliferative disorders with thrombocytosis. The experiment was conducted on high power magnification photomicrographs taken from hematoxylin-and-eosin 3 µm thick sections of formalin fixed, paraffin embedded bone marrow biopsies from ...

  6. Gestational trophoblastic neoplasms

    International Nuclear Information System (INIS)

    Demas, B.E.; Hricak, H.; Braga, C.

    1988-01-01

    Twenty-four women with suspected gestational trophoblastic neoplasms were evaluated prospectively to identify imaging algorithms optimal for treatment planning. All underwent chest radiography, chest CT, hepatic and cranial CT or MR imaging, and pelvic MR imaging. Ten also underwent pelvic CT, 13 pelvic US. The most sensitive imaging combination was chest CT, hepatic and cranial CT or MR imaging, and pelvic MR imaging. However, correct assignment to ACOG therapeutic categories was achieved by means of history, physical examination, beta subunit of human chorionic gonadotropin measurements, and chest radiography in 81% of patients. Hepatic and cranial imaging defined the need for radiation therapy. Chest CT was needed only when chest radiographs were negative. Pelvic imaging aided diagnosis but did not assist in treatment planning

  7. The Bone Marrow-Mediated Protection of Myeloproliferative Neoplastic Cells to Vorinostat and Ruxolitinib Relies on the Activation of JNK and PI3K Signalling Pathways.

    Directory of Open Access Journals (Sweden)

    Bruno A Cardoso

    Full Text Available The classical BCR-ABL-negative Myeloproliferative Neoplasms (MPN are a group of heterogeneous haematological diseases characterized by constitutive JAK-STAT pathway activation. Targeted therapy with Ruxolitinib, a JAK1/2-specific inhibitor, achieves symptomatic improvement but does not eliminate the neoplastic clone. Similar effects are seen with histone deacetylase inhibitors (HDACi, albeit with poorer tolerance. Here, we show that bone marrow (BM stromal cells (HS-5 protected MPN-derived cell lines (SET-2; HEL and UKE-1 and MPN patient-derived BM cells from the cytotoxic effects of Ruxolitinib and the HDACi Vorinostat. This protective effect was mediated, at least in part, by the secretion of soluble factors from the BM stroma. In addition, it correlated with the activation of signalling pathways important for cellular homeostasis, such as JAK-STAT, PI3K, JNK, MEK-ERK and NF-κB. Importantly, the pharmacological inhibition of JNK and PI3K pathways completely abrogated the BM protective effect on MPN cell lines and MPN patient samples. Our findings shed light on mechanisms of tumour survival and may indicate novel therapeutic approaches for the treatment of MPN.

  8. The Bone Marrow-Mediated Protection of Myeloproliferative Neoplastic Cells to Vorinostat and Ruxolitinib Relies on the Activation of JNK and PI3K Signalling Pathways

    Science.gov (United States)

    Cardoso, Bruno A.; Belo, Hélio; Barata, João T.; Almeida, António M.

    2015-01-01

    The classical BCR-ABL-negative Myeloproliferative Neoplasms (MPN) are a group of heterogeneous haematological diseases characterized by constitutive JAK-STAT pathway activation. Targeted therapy with Ruxolitinib, a JAK1/2-specific inhibitor, achieves symptomatic improvement but does not eliminate the neoplastic clone. Similar effects are seen with histone deacetylase inhibitors (HDACi), albeit with poorer tolerance. Here, we show that bone marrow (BM) stromal cells (HS-5) protected MPN-derived cell lines (SET-2; HEL and UKE-1) and MPN patient-derived BM cells from the cytotoxic effects of Ruxolitinib and the HDACi Vorinostat. This protective effect was mediated, at least in part, by the secretion of soluble factors from the BM stroma. In addition, it correlated with the activation of signalling pathways important for cellular homeostasis, such as JAK-STAT, PI3K, JNK, MEK-ERK and NF-κB. Importantly, the pharmacological inhibition of JNK and PI3K pathways completely abrogated the BM protective effect on MPN cell lines and MPN patient samples. Our findings shed light on mechanisms of tumour survival and may indicate novel therapeutic approaches for the treatment of MPN. PMID:26623653

  9. The 8p12 myeloproliferative syndrome | John-Olabode | Nigerian ...

    African Journals Online (AJOL)

    8p12 myeloproliferative syndrome (EMS) / stem cell leukaemia-lymphoma syndrome (SCLL) belongs to the tyrosine kinase fusion genes chronic myeloproliferative diseases. The patient was managed conservatively with hydroxyurea, allopurinol and blood component therapy. The patient eventually died of intracerebral ...

  10. Genomic profile of a patient with triple negative essential thrombocythemia, unresponsive to therapy: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Uzma Zaidi

    2017-07-01

    Full Text Available Clonal analysis of patients with triple negative myeloproliferative neoplasm (MPN has provided evidence of additional aberrations, including epigenetic alterations. To discover such novel genetic aberrations, patients were screened through next-generation sequencing using a myeloid sequencing panel of 54 genes using a genetic analyser. Genetic variants in 28 genes, including TET2, BCOR, BCR, and ABL1 were identified in a triple negative essential thrombocythemia (ET patient. The individual role of some of these variants in disease pathogenesis has yet to be studied. Somatic mutations in the same genes have been reported with variable frequencies in myeloid malignancies. However, no pathogenic impact of these variants could be found; therefore, long-term follow up of patients with genetic analysis of a large cohort and the use of whole genome sequencing is required to assess the effects of these variants.

  11. Vascularity in thyroid neoplasms

    DEFF Research Database (Denmark)

    Larsen, Karen Kjaer; Andersen, Niels Frost; Melsen, Flemming

    2006-01-01

    The aim of the present study was to evaluate the reliability of four different methods (vascular grading, Chalkley count, microvessel density (MVD) and stereological estimation) for quantifying intratumoral microvascularity in thyroid neoplasms, by comparing the variability within and between...... count should be the preferred method for assessing microvascularity in thyroid neoplasms. The diagnostic evaluation revealed a tendency towards higher degree of vascularity in FA compared to both FC and PC for all methods. No statistically significant association was seen between vascular density...

  12. Age-Related Macular Degeneration in Patients With Chronic Myeloproliferative Neoplasms

    DEFF Research Database (Denmark)

    Bak, Marie; Sørensen, Torben Lykke; Flachs, Esben Meulengracht

    2017-01-01

    . Using Cox proportional hazards regression models, smoking and risk-time adjusted hazard ratios (HRs) between patients and controls were calculated. In addition, HRs of neovascular AMD after 2006 were calculated since antivascular endothelial growth factor treatment was introduced nationwide at hospitals...

  13. Extensive Bone Marrow Necrosis in a Case of Acute Myeloid Leukemia Transformed from a Myeloproliferative Neoplasm

    Directory of Open Access Journals (Sweden)

    Roman Shapiro

    2015-08-01

    Full Text Available Extensive necrosis affecting more than 50% of the bone marrow is an extremely rare histopathological finding. Relatively little is known about its clinical significance because it is most commonly identified at autopsy - whether it is an independent prognostic marker or whether it is a surrogate marker of underlying disease burden remains unclear. We describe herein a case of a 66-year-old patient with acute myeloid leukemia who presented with acute bone marrow failure and was found to have extensive necrosis. We include presenting clinical features, pathology attained at biopsy, and the challenge of treatment. Bone marrow necrosis is a rare but important clinicopathological entity whose recognition may herald the way for more effective prognostication of underlying disease.

  14. Associations between Fatigue, Physical Activity, and QoL in Patients with Myeloproliferative Neoplasms

    DEFF Research Database (Denmark)

    Tolstrup Larsen, Rasmus; Tang, Lars Hermann; Brochmann, Nana

    2018-01-01

    is suggested as a fatigue-reducing and QoL-enhancing intervention in MPN patients. The interrelationship between physical activity, fatigue, and QoL have, to our knowledge, never been reported. METHODS: We analyzed data from 1,807 MPN patients. The primary analysis included a multiple regression model allowing...... fatigue to mediate the relationship between physical activity and QoL. RESULTS: We herein report the first and the largest study of patients with MPNs, in whom we have investigated the interrelationship between fatigue, physical activity, and QoL. Sedentary patients were more likely to report fatigue...

  15. Unusual Case of Simultaneous Presentation of Plasma Cell Myeloma, Chronic Myelogenous Leukemia, and a Jak2 Positive Myeloproliferative Disorder

    Directory of Open Access Journals (Sweden)

    J. Maerki

    2014-01-01

    Full Text Available Background. Multiple articles discuss the rare incidence and potential causes of second hematologic disorders arising after treatment of Chronic Myelogenous Leukemia (CML, leading to the theory of imatinib, the current treatment regimen for CML, as a possible trigger for the development of secondary neoplasms. Our case eliminates the possibility of imatinib as the sole cause since our patient received a diagnosis of simultaneous plasma cell myeloma, CML, and a Jak2 mutation positive myeloproliferative disorder (MPD arising de novo, prior to any treatment. We will further investigate into alternative theories as potential causes for multiple hematopathologic disorders. Case Report. There are currently no reported cases with the diagnosis of simultaneous plasma cell myeloma, chronic myelogenous leukemia, and Jak2 positive myeloproliferative disorder. We present a case of a 77-year-old male who was discovered to have these three concurring hematopathologic diagnoses. Our review of the literature includes a look at potential associations linking the three coexisting hematologic entities. Conclusion. The mechanism resulting in simultaneous malignancies is most likely multifactorial and potentially includes factors specific to the host, continuous stimulation of the immune system, previous chemotherapy or radiation, a potential common pluripotent stem cell, or, lastly, preexisting myeloma which may increase the susceptibility of additional malignancies.

  16. The Jak2 Inhibitor, G6, Alleviates Jak2-V617F–Mediated Myeloproliferative Neoplasia by Providing Significant Therapeutic Efficacy to the Bone Marrow

    Directory of Open Access Journals (Sweden)

    Annet Kirabo

    2011-11-01

    Full Text Available We recently developed a Janus kinase 2 (Jak2 small-molecule inhibitor called G6 and found that it inhibits Jak2-V617F– mediated pathologic cell growth in vitro, ex vivo, and in vivo. However, its ability to inhibit Jak2-V617F–mediated myeloproliferative neoplasia, with particular emphasis in the bone marrow, has not previously been examined. Here, we investigated the efficacy of G6 in a transgenic mouse model of Jak2-V617F–mediated myeloproliferative neoplasia. We found that G6 provided therapeutic benefit to the peripheral blood as determined by elimination of leukocytosis, thrombocytosis, and erythrocytosis. G6 normalized the pathologically high plasma concentrations of interleukin 6 (IL-6. In the liver, G6 eliminated Jak2-V617F–driven extramedullary hematopoiesis. With respect to the spleen, G6 significantly reduced both the spleno-megaly and megakaryocytic hyperplasia. In the critically important bone marrow, G6 normalized the pathologically high levels of phospho-Jak2 and phospho–signal transducer and activator of transcription 5 (STAT5. It significantly reduced the megakaryocytic hyperplasia in the marrow and completely normalized the M/E ratio. Most importantly, G6 selectively reduced the mutant Jak2 burden by 67% on average, with virtual elimination of mutant Jak2 cells in one third of all treated mice. Lastly, clonogenic assays using marrow stem cells from the myeloproliferative neoplasm mice revealed a time-dependent elimination of the clonogenic growth potential of these cells by G6. Collectively, these data indicate that G6 exhibits exceptional efficacy in the peripheral blood, liver, spleen, and, most importantly, in the bone marrow, thereby raising the possibility that this compound may alter the natural history of Jak2-V617F–mediated myeloproliferative neoplasia.

  17. The World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues: an overview with emphasis on the myeloid neoplasms.

    Science.gov (United States)

    Vardiman, James W

    2010-03-19

    The World Health Organization (WHO) classification of myeloid and lymphoid neoplasms utilizes morphology, immunophenotype, genetics and clinical features to define disease entities of clinical significance. It is a consensus classification in which a number of experts have agreed on the classification and diagnostic criteria. In general, the classification stratifies neoplasms according to their lineage (myeloid, lymphoid, histiocytic/dendritic) and distinguishes neoplasms of precursor cells from those comprised of functionally mature cells. Lymphoid neoplasms are derived from cells that frequently have features that recapitulate stages of normal B-, T-, and NK-cell differentiation and function, so to some extent they can be classified according to the corresponding normal counterpart, although additional features, such as genotype, clinical features and even location of the tumor figure into the final classification listing as well. Five major subgroups of myeloid neoplasms are recognized based mainly on their degree of maturation and biologic properties: myeloproliferative neoplasms (MPNs) which are comprised primarily of mature cells with effective proliferation; myeloid (and lymphoid) neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB and FGFR1, defined largely by the finding of significant eosinophilia and specific genetic abnormalities; myelodysplastic/myeloproliferative neoplasms (MDS/MPN), comprised mainly of mature cells with both effective and ineffective proliferation of various lineages; myelodysplastic syndromes (MDS), in which immature and mature cells are found with abnormal, dysplastic and ineffective maturation, and acute myeloid leukemia (AML), comprised of precursor cells with impaired maturation. Genetic abnormalities play an important role as diagnostic criteria for further sub-classification of some myeloid neoplasms, particularly of AML. Although therapy-related MDS and AML (t-MDS/AML) often have genetic defects identical to

  18. Anagrelide treatment in 52 patients with chronic myeloproliferative diseases

    DEFF Research Database (Denmark)

    Penninga, E; Jensen, B A; Hansen, P B

    2004-01-01

    In this retrospective multi-centre study, we report our experience with anagrelide in the treatment of thrombocytosis in patients with chronic myeloproliferative diseases. Our study included 52 patients (age 20-78 years). The initial anagrelide dose was, in general, 0.5 mg once daily and mean...

  19. Risk factors for neoplasms

    International Nuclear Information System (INIS)

    Brachner, A.; Grosche, B.

    1991-06-01

    A broad survey is given of risk factors for neoplasms. The main carcinogenic substances (including also ionizing radiation and air pollution) are listed, and are correlated with the risk factors for various cancers most frequently explained and discussed in the literature. The study is intended to serve as a basis for a general assessment of the incidence of neoplasms in children, and of cancer mortality in the entire population of Bavaria in the years 1983-1989, or 1979-1988, respectively, with the principal idea of drawing up an environment-related health survey. The study therefore takes into account not only ionizing radiation as a main risk factor, but also other risk factors detectable within the ecologic context, as e.g. industrial installations and their effects, refuse incineration plants or waste dumps, or the social status. (orig./MG) [de

  20. Translocation t(3;12(q26;q21 In Jak2V617F Point Mutation Negative Chronic Idiopathic Myelofibrosis: A Case Report

    Directory of Open Access Journals (Sweden)

    S. Mešanović

    2014-06-01

    Full Text Available The myeloproliferative diseases (MPDs or myelo-proliferative neoplasms (MPNs are a group of diseases of the bone marrow in which excess cells are produced. Chronic idiopathic myelofibrosis (CIMF is a stem cell defect characterized by splenomegaly with multiorgan extramedullary hematopoiesis, immature peripheral blood granulocytes and erythrocytes and progressive bone marrow fibrosis. The most common chromosomal abnormalities seen in CIMF patients include numerical changes of chromosomes 7, 8 and 9, and structural changes of 1q, 5q, 13q and 20q. At least 75.0% of patients with bone marrow abnormalities have one or more of these chromosomal anomalies. Detection of the Janus kinase 2 (JAK2 mutation may be a potential major breakthrough for understanding the pathobiology of MPNs, and is an essential part of the diagnostic algorithm. In this study, we describe a JAK2V617F mutation negative CIMF patient who has the chromosomal translocation t(3;12(q26;q21 in her karyotype.

  1. Cerebral haemorrhage as the presenting feature of myeloproliferative disorder

    OpenAIRE

    Kondlapudi, Jyothi; O’Connor, Rory J; Mawer, Samantha

    2009-01-01

    Myeloproliferative disorders predispose individuals to bleeding and thrombosis, often with devastating consequences. We report a 41-year-old man who presented with headache, amnesia and dysphagia due to cerebral haemorrhage. Extensive investigation revealed the cause of the neurological syndrome as an underlying essential thrombocytosis. The patient made a full recovery following extensive inpatient and community rehabilitation, returning to work after 6 months. We discuss the diagnosis and m...

  2. New classification of acute myeloid leukemia and precursor-related neoplasms: changes and unsolved issues.

    Science.gov (United States)

    Falini, Brunangelo; Tiacci, Enrico; Martelli, Maria Paola; Ascani, Stefano; Pileri, Stefano A

    2010-10-01

    The World Health Organization (WHO) classification of lympho-hematopoietic neoplasms is increasingly based on genetic criteria. Here, we focus on changes that, as compared to the 2001 edition, were introduced into the 2008 WHO classification of acute myeloid leukemia (AML) and related precursor neoplasms. The category of AML with recurrent genetic abnormalities was expanded to account for 60% of AML by adding three distinct entities, i.e., AML with t(6,9), inv(3), or t(1;22), and two provisional entities, i.e., AML with mutated NPM1 or CEBPA. These changes have greatly modified the approaches to diagnosis and prognostic stratification of AML patients. To emphasize the need of various parameters for diagnosis, including myelodysplasia (MD)-related cytogenetic abnormalities, history of myelodysplasia or myelodysplasia/myeloproliferative neoplasm, and multilineage dysplasia, the category of "AML with multilineage dysplasia" was re-named AML with MD-related changes. Finally, we describe the unique characteristics of myeloid proliferations associated with Down syndrome and blastic plasmacytoid dendritic cell neoplasm.

  3. Síndrome Mieloproliferativa Crônica Inclassificável: Relato de Caso/Nondescript Chronicles Myeloproliferative Syndrome: Case Report

    Directory of Open Access Journals (Sweden)

    Vanessa Parducci Brandão

    2014-06-01

    Full Text Available Introdução: As neoplasias mieloproliferativas são patologias que se originam de células tronco totipotentes e se caracterizam por multiplicação acelerada de uma ou mais séries sanguíneas, sem relevante displasia. São classificadas em diversos subtipos entre eles: Leucemia Mielóide Crônica, Leucemia Neutrofílica Crônica,Mielofribrose Primária e Doença Mieloproliferativa Crônica Inclassificável. Essa classificação justifica-se por mutações genéticas que definem a evolução e a fisiopatologia de cada doença. Casuística: Apresentamos o caso de uma paciente, sexo feminino, 58 anos, com queixas inespecíficas, que por achado laboratorial suspeitou-se de leucemia. Foi submetida à investigação clínica, laboratorial,radiológica, e realizada a biópsia de MO, sendo diagnosticada com Síndrome Mieloproliferativa Crônica Inclassificável. Foi orientada a fazer uso de hidroxiureiapara controle hematimétrico, porém sem melhora, iniciou citarabina. Paciente encontra-se na fase blástica da doença e devido à ausência de remissão da patologia,foi encaminhada para lista de transplante. Discussão: o caso foi bem conduzido quanto aos métodos diagnósticos utilizados na investigação da mesma, sendo fundamental o RT-PCR para identificação do cromossomo Philadelphia. O tratamento foi semelhante à literatura em relação aos medicamentos usados, porém algumas posologias não foram as mesmas. A condução do caso levou a paciente paraa fila de transplante, visto que não houve resposta terapêutica. Conclusão: este relato de caso trata-se, portanto, de uma Neoplasia Mieloproliferativa Crônica do tipo inclassificável, pois não preenche os critérios diagnósticos preconizados pela Organização Mundial de Saúde, visto que foi classificada como Leucemia Mielóide Crônica atípica BCR/ABL negativa, para fins terapêuticos. Introduction: Myeloproliferative diseases are neoplasms that originate from to tipotent stem cell

  4. Clinicopathological features of transient myeloproliferative syndrome and congenital leukaemia

    International Nuclear Information System (INIS)

    Sajid, N.; Ahmed, N.; Mahmood, S.

    2010-01-01

    The objectives of the study were to determine the spectrum of the clinical and pathological findings, the management and prognosis of patients of transient myeloproliferative syndrome (TMS) and congenital leukaemia. Study Design: Case series. Place and Duration of Study: The study was conducted over a period of 8 years, from January 2000 to December 2007, at the Children's Hospital and the Institute of Child Health, Lahore. Methodology: Suspected patients presenting with fever, pallor, bruises and hepatosplenomegaly and diagnosed as either transient myeloproliferative disorder or congenital leukaemia were studied. The complete blood count, reticulocyte count, leukocyte alkaline phosphatase score, liver function tests, karyotyping studies and bone marrow aspiration biopsy were performed in all of those patients. Management and out come was noted. Results were described as frequency percentages. Results: Out of 10,000 patients presenting during this period, 24 patients were diagnosed as either of transient myeloproliferative syndrome or congenital leukaemia. Fifteen of these were diagnosed as patients of TMS and 9 as patients of congenital leukaemia. Down syndrome (DS) was diagnosed in 75% of these patients. TMS patients were put on supportive treatment and recovered spontaneously. One DS patient with congenital leukaemia went into spontaneous remission and 2 of DS patients with congenital leukaemia responded to chemotherapy while rest of them either died or lost to follow-up. Conclusion: TMS and congenital leukaemia were not very uncommon in the studied population. Majority had Down syndrome. It is important to differentiate their clinical and pathological presentations for proper management. TMS may resolve with supportive treatment while congenital leukaemia is a fatal condition requiring chemotherapy. (author)

  5. Pulmonary hypertension in patients with chronic myeloproliferative disorders

    Directory of Open Access Journals (Sweden)

    Yochai Adir

    2015-09-01

    Full Text Available Pulmonary hypertension (PH is a major complication of several haematological disorders. Chronic myeloproliferative diseases (CMPDs associated with pulmonary hypertension have been included in group five of the clinical classification for pulmonary hypertension, corresponding to pulmonary hypertension for which the aetiology is unclear and/or multifactorial. The aim of this review is to discuss the epidemiology, pathogenic mechanism and treatment approaches of the more common forms of pulmonary hypertension in the context of CMPD's: chronic thromboembolic pulmonary hypertension, precapillary pulmonary hypertension and drug-induced PH.

  6. Dendritic cell neoplasms: an overview.

    Science.gov (United States)

    Kairouz, Sebastien; Hashash, Jana; Kabbara, Wadih; McHayleh, Wassim; Tabbara, Imad A

    2007-10-01

    Dendritic cell neoplasms are rare tumors that are being recognized with increasing frequency. They were previously classified as lymphomas, sarcomas, or histiocytic neoplasms. The World Health Organization (WHO) classifies dendritic cell neoplasms into five groups: Langerhans' cell histiocytosis, Langerhans' cell sarcoma, Interdigitating dendritic cell sarcoma/tumor, Follicular dendritic cell sarcoma/tumor, and Dendritic cell sarcoma, not specified otherwise (Jaffe, World Health Organization classification of tumors 2001; 273-289). Recently, Pileri et al. provided a comprehensive immunohistochemical classification of histiocytic and dendritic cell tumors (Pileri et al., Histopathology 2002;59:161-167). In this article, a concise overview regarding the pathological, clinical, and therapeutic aspects of follicular dendritic, interdigitating dendritic, and Langerhans' cell tumors is presented.

  7. The SKI proto-oncogene enhances the in vivo repopulation of hematopoietic stem cells and causes myeloproliferative disease.

    Science.gov (United States)

    Singbrant, Sofie; Wall, Meaghan; Moody, Jennifer; Karlsson, Göran; Chalk, Alistair M; Liddicoat, Brian; Russell, Megan R; Walkley, Carl R; Karlsson, Stefan

    2014-04-01

    The proto-oncogene SKI is highly expressed in human myeloid leukemia and also in murine hematopoietic stem cells. However, its operative relevance in these cells remains elusive. We have over-expressed SKI to define its intrinsic role in hematopoiesis and myeloid neoplasms, which resulted in a robust competitive advantage upon transplantation, a complete dominance of the stem and progenitor compartments, and a marked enhancement of myeloid differentiation at the expense of other lineages. Accordingly, enforced expression of SKI induced a gene signature associated with hematopoietic stem cells and myeloid differentiation, as well as hepatocyte growth factor signaling. Here we demonstrate that, in contrast to what has generally been assumed, the significant impact of SKI on hematopoiesis is independent of its ability to inhibit TGF-beta signaling. Instead, myeloid progenitors expressing SKI are partially dependent on functional hepatocyte growth factor signaling. Collectively our results demonstrate that SKI is an important regulator of hematopoietic stem cell activity and its overexpression leads to myeloproliferative disease.

  8. Histogenesis of salivary gland neoplasms.

    Science.gov (United States)

    Regezi, J A; Batsakis, J G

    1977-06-01

    In an attempt to explain the histogenesis of salivary gland tumors, a bicellular theory of origin has been presented. This theory is supported by indirect evidence from light and electron microscopy. Two cells, the excretory duct reserve cell and the intercalated duct reserve cell, are presented as the hypothetical cells of origin for salivary gland neoplasms. It is argued that the excretory duct reserve cell gives rise to squamous cell carcinomas and mucoepidermoid carcinomas, and that the intercalated duct reserve cell gives rise to all others. It It is also shown that myoepithelial cells are responsible in part for the wide histologic variation of these neoplasms.

  9. Raman spectroscopy of skin neoplasms

    Science.gov (United States)

    Moryatov, A. A.; Kozlov, S. V.; Kaganov, O. I.; Orlov, A. E.; Zaharov, V. P.; Batrachenko, I. A.; Artemiev, D. N.; Blinov, N. V.

    2017-09-01

    Skin melanoma is spread inhomogeneously worldwide, particularly in Samara region there are high figures of skin neoplasms sick rate as well—18.6%. Research goal: to develop a new method of early non-invasive differential diagnostics of skin neoplasms. Registration of Raman spectrum was implemented in the distance of 3-4 mm, the spectrum registration from pathologically changed zone was subsequently conducted, then from healthy skin zone. The test time for 1 patient was no longer than 3-5 min. In a range of experiments ex vivo there were the following results: melanoma—24, basal cell cancer—25, squamosus cell sarcinoma—7, nevus pigmentosis—9, other malignant neoplasms—6; in vivo: melanoma—9, basal cell cancer—8, nevus pigmentosis—2, other benign neoplasms—2. The first results of the research dedicated to studying permissive opportunities of Raman spectroscopy, with successive two-phase analysis of received parameters display high efficiency of method of differential diagnostic for skin melanoma and other malignant neoplasms, pigment and benign skin neoplasms. Safety and rapidity of the research reveal a high potential of the technique.

  10. Gastrointestinal Surgery of Neuroendocrine Neoplasms

    DEFF Research Database (Denmark)

    Hansen, Carsten Palnæs; Olsen, Ingrid Marie Holst; Knigge, Ulrich

    2015-01-01

    Surgery is the only treatment that may cure the patient with gastroenteropancreatic (GEP) neuroendocrine neoplasms (NENs) and should always be considered as the first-line treatment if radical resection can be achieved. Even in cases where radical surgery is not possible, palliative resection may...

  11. Methemoglobinemia in Young Patients With Hematologic Cancer or Aplastic Anemia Treated With Dapsone

    Science.gov (United States)

    2017-04-13

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Methemoglobinemia; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Nonmalignant Neoplasm

  12. Tissue, Blood, and Body Fluid Sample Collection From Patients With Hematologic Cancer

    Science.gov (United States)

    2017-09-20

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Nonmalignant Neoplasm

  13. Paediatric atypical spitzoid melanocytic neoplasm

    Directory of Open Access Journals (Sweden)

    Aileen F. Egan

    2017-12-01

    Full Text Available Cutaneous malignant melanoma is a rare occurrence in children, with an incidence of less than one case per million per year in children under ten years of age. However this statistic is reportedly increasing. Mortality rates in paediatric melanoma are not well described, however reports suggest that 5-year survival rates are improving beyond those of adults. This may be partly attributable to more intensive classification and staging of melanocytic lesions. Atypical spitzoid neoplasms (ASN are a subcategorisation of the diagnostic spectrum which extends from Spitz naevi to spitzoid melanoma, and are relatively more common than the latter. The clinical and histopathological features of such lesions are imprecise, leading to difficulties in making diagnoses and subsequent management. This report documents one such case arising within an atypical spitzoid melanocytic neoplasm and the clinical process undertaken. In particular we wished to highlight the molecular diagnostics utilised and their impact on the decision-making pathway.

  14. Hemorrhagic and thrombotic complications in patients with myeloproliferative diseases

    Directory of Open Access Journals (Sweden)

    Carla Valladares Vignal

    Full Text Available OBJECTIVE: To correlate the incidence of hemorrhage and thrombosis to bleeding time (BT and platelet aggregation in 27 consecutive patients with myeloproliferative diseases (MPD. DESIGN: Retrospective study. SETTING: Public tertiary referral center. PATIENTS: Eighteen patients with chronic myelogenous leukemia (CML, 5 with polycytemia vera (PV, 2 with essential thrombocytemia (ET and 2 with idiopathic myelofibrosis (MF. Duke's BT and epinephrine-induced platelet aggregation were performed on the patients and on 10 healthy individuals. RESULTS: Eleven patients presented symptoms (41 %: 9 with hemorrhage (33% and 5 with thrombosis (19%.There were less symptomatic patients in the CML group (28% than in the other MPD (67%, without statistical significance (Fisher, p=0.06. Duke's BT was longer in symptomatic patients (Mann-Whitney, p<0.05. Platelet aggregation was abnormal in 7 patients (26% and 71% of them were symptomatic (Fisher, p = 0.07. CONCLUSIONS: The high incidence of bleeding and thrombosis in patients with MPD was related to prolonged BT, but not to platelet aggregation abnormalities.

  15. Chronic myelocytic leukemia and the myeloproliferative diseases during the child-bearing years

    Energy Technology Data Exchange (ETDEWEB)

    Miller, J.B.

    1976-10-01

    Chronic myelocytic leukemia and the myeloproliferative diseases may present as asymptomatic elevations of peripheral blood counts or with relatively nonspecific symptoms. Full diagnostic evaluation is necessary to eliminate the many other causes of blood count elevation. Coincidence of a myeloproliferative disease and pregnancy is unusual. The pregnancy has no adverse effect on the course of the mother's hematologic disease. However, the myeloproliferative diseases, especially if uncontrolled, result in increased fetal prematurity and mortality. Treatment of the pregnant patient should be conservative, and cytotoxic therapy should be avoided until at least after the first trimester whenever possible. Busulfan has been the drug most widely used during pregnancy, and it appears not to cause fetal malformations when used alone.

  16. Prenatal ultrasound findings of fetal neoplasms

    International Nuclear Information System (INIS)

    Lee, Soo Hyun; Cho, Jeong Yeon; Song, Mi Jin; Min, Jee Yeon; Han, Byoung Hee; Lee, Young Ho; Cho, Byung Jae; Kim, Seung Hyup

    2002-01-01

    A variety of neoplasms can develop in each tetal organ. Most fetal neoplasms can be detected by careful prenatal ultrasonographic examination. Some neoplosms show specific ultrasonographic findings suggesting the differential diagnosis, but others do not. Knowledge of the presence of a neoplasm in the fetus may alter the prenatal management of a pregnancy and the mode of delivery, and facilitates immediate postnatal treatment. During the last five years, we experienced 32 cases of fetal neoplasms in a variety of organs. We describe their typical and ultrasonographic findings with correlating postnatal CT, MRI, and pathologic findings

  17. Tyrosine-rich crystals associated with oncocytic salivary gland neoplasms.

    Science.gov (United States)

    Gilcrease, M Z; Nelson, F S; Guzman-Paz, M

    1998-07-01

    Crystalloids have been identified ultrastructurally within the epithelial cells of Warthin's tumors, but there have been no studies characterizing crystals or crystalloids in Warthin's tumors by light microscopy. The finding of abundant needle-shaped crystals in a fine-needle aspirate of a cystadenoma of the parotid prompted us to examine the prevalence of crystals and crystalloids in oncocytic salivary gland neoplasms. Ninety-seven oncocytic neoplasms (93 Warthin's tumors, 3 cystadenomas, and 1 oncocytoma) excised at our institution between 1950 and 1996 were examined, to identify crystals. Neoplasms with crystals were further characterized by means of a variety of histochemical stains and electron microscopy. Ninety-nine pleomorphic adenomas were similarly reviewed. Seven cases with crystals were identified. Five of these were Warthin's tumors, 1 was a cystadenoma, and 1 was an oncocytoma. The crystals were noted within tumor cysts but were not limited to the neoplasms. The crystals were predominantly either needle-shaped or tabular, but some cases contained mixtures of both as well as intermediate forms. They stained pink with hematoxylin-eosin, although the tabular forms also exhibited a focal yellow hue. The crystals were not discernible under polarized light. They stained a red-brown color with Millon's reagent, which indicated the presence of tyrosine. Trichrome, periodic acid-Schiff stain with diastase, alcian blue (pH 2.5), and Congo red stains were negative. Electron microscopy revealed sharply defined, elongate, electron-dense structures with periodicity, both extracellular and within epithelial cells. No crystals or crystalloids were identified in any of 99 pleomorphic adenomas reviewed. The findings indicate that tyrosine-rich crystals associated with several oncocytic salivary gland neoplasms are morphologically, histochemically, and ultrastructurally distinct from previously described tyrosine-rich crystalloids and collagenous crystalloids of

  18. Anal channel neoplasm: a neoplasm radio chemo curable

    International Nuclear Information System (INIS)

    Torres Lopez, M.; Avondet, I.; Vazquez, J.; Santini Blasco, A.

    1997-01-01

    Presently work is made an exhaustive revision of the anatomy of the region, the history of the treatments and of the current treatments of channel cancer anal. It makes emphasis in the importance of the conservative treatment with radiochemotherapy (RQT). The present is a prospective study,longitudinal and descriptive. Material and method: between January of 1989 and December of 1994 20 patients attended with cancer of anal channel with an illness metastasis. An average age it was of 62.4 years.The sex, 16 men and 4 women. The performance status 0,1 or 2 of the scale of the ECOQ. In the pathological anatomy: 15 patient epidermic neoplasm, 5 patient basal neoplasm. State I: 2 patients, II: 12 patients, III: 6 patients, IV: 0 patients.Treatment: the radiotherapy one carries out with cobalt 60 and it irradiates the primary tumour and the ganglion structures region, pelvic and inguinal. It surrendered to Gy/dia from Monday to Friday up to 50 Gy. The chemotherapy one carries out with mitomicine C 10 mg/ previous day to the radiotherapy and 5-UGH 1 intravenous g/my in infusion the days from 1 to 4 and from 29 to 32 after the radiotherapy.Results: to) control locorregional patient RC-16 (80%) ,RP 2 patients (10%) , without answer or with progression lesional a patient (5%) .b) State vital: living 15 patients, died 5 patients(continuation 12 to 60 months) .e)Tolerance: there were not deaths for the gastrointestinal treatment and haematological with toxicity moderate.To conclude:1) The radiochemotherapy is the treatment of elect.2)A feasible treatment of being carried out in our environment.3)Required of a good relationship predictable interdisciplinary.4)Toxicity and tolerable.5)Results of conservation of the sphincter in 80%(AU) [es

  19. Meningiomas among intracranial neoplasms in Johannesburg ...

    African Journals Online (AJOL)

    Background: Worldwide there are varying reports on the prevalence of meningiomas among intracranial neoplasms. Different reports state intracranial meningiomas, gliomas or metastatic tumours as the most common tumour among intracranial neoplasms. We present our institutions' experience of patients with intracranial ...

  20. Stages of Plasma Cell Neoplasms (Including Multiple Myeloma)

    Science.gov (United States)

    ... Treatment Health Professional Plasma Cell Neoplasms Treatment Research Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Patient Version General Information About Plasma Cell Neoplasms Go to Health Professional Version Key ...

  1. Basaloid Carcinoma of the Breast Mimicking Cutaneous Basaloid Neoplasms.

    Science.gov (United States)

    Solus, Jason F; Goyal, Amrita; Duncan, Lyn M; Nazarian, Rosalynn M

    2015-09-01

    Basaloid carcinoma of the breast (BCB) is a rare, triple-negative aggressive primary breast tumor that can closely mimic cutaneous basal cell carcinoma (BCC), neuroendocrine tumors, adnexal neoplasms, and other primary breast tumors. Accurate diagnosis of this tumor is critical for appropriate clinical management. We add to the literature 2 female patients with BCB presenting with a nipple mass. Histopathologic findings from both patients showed dermal nests and cords of atypical basaloid cells with epidermal involvement, closely resembling cutaneous BCC. A panel of immunohistochemical stains, including the novel use of CK17, is essential for differentiating BCB from mimickers. BCB is a rare primary breast tumor that follows an aggressive clinical course and closely mimics many basaloid neoplasms, including cutaneous BCC clinicopathologically. Increased awareness of BCB among dermatologists and dermatopathologists is critical for accurate diagnosis and patient care.

  2. Blastic plasmacytoid dendritic cell neoplasm with absolute monocytosis at presentation

    Directory of Open Access Journals (Sweden)

    Jaworski JM

    2015-02-01

    Full Text Available Joseph M Jaworski,1,2 Vanlila K Swami,1 Rebecca C Heintzelman,1 Carrie A Cusack,3 Christina L Chung,3 Jeremy Peck,3 Matthew Fanelli,3 Micheal Styler,4 Sanaa Rizk,4 J Steve Hou1 1Department of Pathology and Laboratory Medicine, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA; 2Department of Pathology, Mercy Fitzgerald Hospital, Darby, PA, USA; 3Department of Dermatology, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA; 4Department of Hematology/Oncology, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA Abstract: Blastic plasmacytoid dendritic cell neoplasm is an uncommon malignancy derived from precursors of plasmacytoid dendritic cells. Nearly all patients present initially with cutaneous manifestations, with many having extracutaneous disease additionally. While response to chemotherapy initially is effective, relapse occurs in most, with a leukemic phase ultimately developing. The prognosis is dismal. While most of the clinical and pathologic features are well described, the association and possible prognostic significance between peripheral blood absolute monocytosis (>1.0 K/µL and blastic plasmacytoid dendritic cell neoplasm have not been reported. We report a case of a 68-year-old man who presented with a rash for 4–5 months. On physical examination, there were multiple, dull-pink, indurated plaques on the trunk and extremities. Complete blood count revealed thrombocytopenia, absolute monocytosis of 1.7 K/µL, and a negative flow cytometry study. Biopsy of an abdominal lesion revealed typical features of blastic plasmacytoid dendritic cell neoplasm. Patients having both hematologic and nonhematologic malignancies have an increased incidence of absolute monocytosis. Recent studies examining Hodgkin and non-Hodgkin lymphoma patients have suggested that this is a negative prognostic factor. The association between

  3. The janus kinase 2 (JAK2 mutation: A review of its role in polycythaemia vera and other myeloproliferative disorders

    Directory of Open Access Journals (Sweden)

    M U Nwagu

    2011-01-01

    Results: JAK2 mutation plays important role in the general pathophysiology of non-leukemic myeloproliferative diseases. Proper understanding of these roles is being utilized in research and development of novel therapeutic agents.

  4. Recurrent Benign Salivary Gland Neoplasms.

    Science.gov (United States)

    Witt, Robert Lee; Nicolai, Piero

    2016-01-01

    The most important causes of recurrence of benign pleomorphic adenoma are enucleation with intraoperative spillage and incomplete tumor excision in association with characteristic histologic findings for the lesion (incomplete pseudocapsule and the presence of pseudopodia). Most recurrent pleomorphic adenomas (RPAs) are multinodular. MRI is the imaging method of choice for their assessment. Nerve integrity monitoring may reduce morbidity of RPA surgery. Although treatment of RPA must be individualized, total parotidectomy is generally recommended given the multicentricity of the lesions. However, surgery alone may be inadequate for controlling RPA over the long term. There is growing evidence from retrospective series that postoperative radiotherapy results in significantly better local control. A high percentage of RPAs are incurable. All patients should therefore be informed about the possibility of needing multiple treatment procedures, with possible impairment of facial nerve function, and radiation therapy for RPA. Reappearance of Warthin tumor is a metachronous occurrence of a new focus or residual incomplete excision of all primary multicentric foci of Warthin tumor. Selected cases can be observed. Conservative surgical management can include partial superficial parotidectomy or extracapsular dissection. Not uncommonly, other major and minor salivary gland neoplasms, including myoepithelioma, basal cell adenoma, oncocytoma, canalicular adenoma, cystadenoma, and ductal papilloma, follow an indolent course after surgical resection, with rare cases of recurrence. © 2016 S. Karger AG, Basel.

  5. Brain and spinal cord neoplasms

    International Nuclear Information System (INIS)

    Anderson, R.E.; Bragg, D.G.; Youker, J.E.

    1985-01-01

    Traditional means of detecting CNS neoplasms include plain film studies, isotope brain scans, angiography, pneumoencephalography, and myelography. Computed tomography (CT) scanning has replaced nearly all of these studies in both the initial detection and follow-up of brain tumors. Air studies (pneumoencephalography and ventriculography) have been virtually eliminated, except in certain unusual circumstances when two positions need to be checked, or hydrocephalus followed. The nuclear brain scan has a very limited role at present, being useful primarily for detecting skull or meningeal metastases. Myelography, however, remains a valuable imaging tool for the assessment of tumors of the spinal canal. CT scanning has not only improved our ability to detect smaller brain tumors, but also CT guided stereotactic biopsy techniques provide a safer means of obtaining tissue from these smaller lesions, regardless of location. Surgical techniques, guided by CT sterotactic techniques, show promise as well, but the impact of these therapeutic techniques on survival statistics remains to be defined. CT has revolutionized the approach to the detection and diagnosis of space-occupying lesions in the brain. Tumors can be detected at a smaller site

  6. RENAL DAMAGE WITH MALIGNANT NEOPLASMS

    Directory of Open Access Journals (Sweden)

    I. B. Kolina

    2015-01-01

    Full Text Available The relationship between renal damage and malignant neoplasms is one of the most actual problems of the medicine of internal diseases. Very often, exactly availability of renal damage determines the forecast of cancer patients. The range of renal pathologies associated with tumors is unusually wide: from the mechanical effect of the tumor or metastases on the kidneys and/or the urinary tract and paraneoplastic manifestations in the form of nephritis or amyloidosis to nephropathies induced with drugs or tumor lysis, etc. Thrombotic complications that develop as a result of exposure to tumor effects, side effects of certain drugs or irradiation also play an important role in the development of the kidney damage. The most frequent variants of renal damage observed in the practice of medical internists (therapists, urologists, surgeons, etc., as well as methods of diagnosis and treatment approaches are described in the article. Timely and successful prevention and treatment of tumor-associated nephropathies give hope for retaining renal functions, therefore, a higher life standard after completion of anti-tumor therapy. Even a shortterm episode of acute renal damage suffered by a cancer patient must be accompanied with relevant examination and treatment. In the caseof transformation of acute renal damage into the chronic kidney disease, such patients need systematic and weighted renoprotective therapy and correct dosing of nephrotoxic drugs.

  7. Cytokeratins in epithelia of odontogenic neoplasms

    NARCIS (Netherlands)

    Crivelini, MM; de Araujo, VC; de Sousa, SOM; de Araujo, NS

    Neoplasms and tumours related to the odontogenic apparatus may be composed only of epithelial tissue or epithelial tissue associated with odontogenic ectomesenchyme. The immunohistochemical detection of different cytokeratins (CKs) polypeptides and vimentin has made it easier to explain the

  8. HISTOPATHOLOGIC CHARACTERISTICS OF THYROID GLAND NEOPLASMS IN THOMSON'S GAZELLES ( EUDORCUS THOMSONII).

    Science.gov (United States)

    French, Stephanie J; Garner, Michael M; Kiupel, Matti

    2018-03-01

    Published reports of neoplasms in Thomson's gazelles ( Eudorcas thomsonii) are very rare, but thyroid tumors were the most common neoplasm of this species, accounting for 12% of reported pathologies in a 1998-2012 retrospective study of cases submitted for histologic review of grossly enlarged thyroid glands. This report describes the histological and immunohistochemical characteristics of thyroid neoplasms in 10 Thomson's gazelles from five different zoological collections. Neoplasms were submitted as biopsies from six gazelles or collected during necropsy from four gazelles. The most common clinical findings included a palpable mass on the ventral neck and progressive weight loss. Radiographic mineral density was detected in one of the neoplastic masses. Histologically, the neoplasms were classified as microfollicular thyroid adenoma ( n = 2), solid thyroid adenoma ( n = 2), papillary thyroid adenoma ( n = 1), and solid thyroid carcinoma ( n = 5). Neoplastic cells in all 10 neoplasms were positive for thyroid transcription factor 1 and thyroglobulin, but negative for calcitonin. While five cases had histologic features of malignancy, there was no evidence of metastatic disease either clinically (biopsies) or on necropsy. Numerous concurrent diseases, including cardiomyopathies and nephropathies, were present and led to choice for euthanasia in several cases.

  9. Somatostatin-Immunoreactive Pancreaticoduodenal Neuroendocrine Neoplasms

    DEFF Research Database (Denmark)

    Engelund Luna, Iben; Monrad, Nina; Binderup, Tina

    2016-01-01

    OBJECTIVE: Neuroendocrine neoplasms in the pancreas and duodenum with predominant or exclusive immunoreactivity for somatostatin (p-dSOMs) are rare, and knowledge on tumour biology, treatment, survival and prognostic factors is limited. This study aimes to describe clinical, pathological, and bio......OBJECTIVE: Neuroendocrine neoplasms in the pancreas and duodenum with predominant or exclusive immunoreactivity for somatostatin (p-dSOMs) are rare, and knowledge on tumour biology, treatment, survival and prognostic factors is limited. This study aimes to describe clinical, pathological...

  10. Geographical distribution for malignant neoplasm of the pancreas in relation to selected climatic factors in Japan

    Directory of Open Access Journals (Sweden)

    Okada Masafumi

    2007-07-01

    Full Text Available Abstract Background Malignant neoplasm of the pancreas has become one of the leading causes of death from malignant neoplasm in Japan (the 5th in 2003. Although smoking is believed to be a risk factor, other risk factors remain unclear. Mortality from malignant neoplasm of the pancreas tends to be higher in northern Japan and in northern European countries. A recent study reported that standardized mortality ratios (SMRs for malignant neoplasm of the pancreas were negatively correlated to global solar radiation level. People residing in regions with lower solar radiation and lower temperatures may be at higher risk of development of malignant neoplasm of the pancreas. Therefore, this study aimed to examine the relationship between SMRs for malignant neoplasm of the pancreas and climatic factors, such as the amount of global solar radiation and the daily maximum temperature in Japan. Results The study used multiple linear regression models. Number of deaths and demographic data from 1998 to 2002 were used for the calculation of SMR. We employed mesh climatic data for Japan published in 2006 by the Japan Meteorological Agency. Regression coefficients for the amount of global solar radiation and the daily maximum temperature in males were -4.35 (p = 0.00034 and -2.81 (p Conclusion This study suggested that low solar radiation and low temperature might relate to the increasing risk of malignant neoplasm of the pancreas. Use of group data has a limitation in the case of evaluating environmental factors affecting health, since the impact of climatic factors on the human body varies according to individual lifestyles and occupations. Use of geographical mesh climatic data, however, proved useful as an index of risk or beneficial factors in a small study area. Further research using individual data is necessary to elucidate the relationship between climatic factors and the development of malignant neoplasm of the pancreas.

  11. Light-Emitting Diode Therapy in Preventing Mucositis in Children Receiving Chemotherapy With or Without Radiation Therapy Before Bone Marrow Transplantation

    Science.gov (United States)

    2013-09-19

    Chronic Myeloproliferative Disorders; Kidney Cancer; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neuroblastoma; Oral Complications; Ovarian Cancer; Pain; Sarcoma

  12. American Ginseng in Treating Patients With Fatigue Caused by Cancer

    Science.gov (United States)

    2016-12-19

    Chronic Myeloproliferative Disorders; Fatigue; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  13. Sirolimus, Tacrolimus, Thymoglobulin and Rituximab as Graft-versus-Host-Disease Prophylaxis in Patients Undergoing Haploidentical and HLA Partially Matched Donor Hematopoietic Cell Transplantation

    Science.gov (United States)

    2017-05-26

    Chronic Myeloproliferative Disorders; Graft Versus Host Disease; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms

  14. Lactobacillus in Preventing Infection in Patients Undergoing a Donor Stem Cell Transplant for Hematologic Cancer or Myelodysplastic Syndrome

    Science.gov (United States)

    2017-02-02

    Breast Cancer; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  15. Acral lividosis--a sign of myeloproliferative diseases. Hyperleukocytosis syndrome in chronic myelogenous leukemia.

    Science.gov (United States)

    Frankel, D H; Larson, R A; Lorincz, A L

    1987-07-01

    Acral ischemia with lividity is a well-described dermatologic sign in the myeloproliferative diseases polycythemia vera and essential thrombocythemia. It has not previously been reported as a sign of chronic myelogenous leukemia (CML). We suggest the term acral lividosis to describe this clinical entity in patients with any myeloproliferative disease. We propose that the pathophysiology of acral lividosis in CML involves occlusion of small blood vessels of the skin by large, nondeformable myeloblasts, a process that has been shown histologically to occur in other organs in patients with CML. This process, called leukostasis, occurs in patients with CML who have over 50.0 X 10(9)/L (50,000/mm3) circulating myeloblasts. Patients manifest cardiorespiratory and central nervous system compromise, a clinical constellation known as the hyperleukocytosis syndrome. Acral lividosis occurred in a patient with CML in whom nearly every organ demonstrated leukostasis on autopsy.

  16. JAK2 V617F mutation, mesenteric vein thrombosis, and myeloproliferative disorders.

    Science.gov (United States)

    Owens, Christopher D

    2010-07-01

    Mesenteric vein thrombosis is a rare disorder that is often the first manifestation of a systemic condition such as a hypercoagulable state or cancer. In particular, myeloproliferative disorders can present as mesenteric vein thrombosis even in the setting of relatively normal peripheral blood counts. A recent novel mutation in the Janus activated kinase 2 gene involving a gain-of-function substitute of valine to phenylalanine at position 617 (JAK2 V617F) has been discovered to be prevalent in patients with mesenteric vein thrombosis and myeloproliferative disorders. This article reports a patient who presented with mesenteric vein thrombosis and relatively normal peripheral blood counts. He was diagnosed with essential thrombocythemia after he tested positive for the JAK2 V617F mutation. Copyright (c) 2010 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.

  17. Perspectives on chronic inflammation in essential thrombocythemia, polycythemia vera, and myelofibrosis

    DEFF Research Database (Denmark)

    Hasselbalch, Hans K

    2012-01-01

    The morbidity and mortality of patients with the chronic Philadelphia-negative myeloproliferative neoplasms (MPNs), essential thrombocythemia, polycythemia vera, and primary myelofibrosis are mainly caused by cardiovascular diseases, thrombohemorrhagic complications, and bone marrow failure because...

  18. Splanchnic venous thrombosis driven by a constitutively activated ...

    African Journals Online (AJOL)

    Introduction: Splanchnic venous thrombosis (SVT) has varied etiology with Philadelphia-negative myeloproliferative neoplasms (MPNs) being the most frequent underlying prothrombotic factor. Hematological indices often remain within normal range because of portal hypertension and its sequelae, causing diagnostic ...

  19. Splanchnic venous thrombosis driven by a constitutively activated ...

    African Journals Online (AJOL)

    Abstract. Introduction: Splanchnic venous thrombosis (SVT) has varied etiology with Philadelphia- negative myeloproliferative neoplasms (MPNs) being the most frequent underlying prothrombotic factor. Hematological indices often remain within normal range because of portal hypertension and its sequelae, causing ...

  20. [The lymph nodes imprint for the diagnosis of lymphoid neoplasms].

    Science.gov (United States)

    Peniche-Alvarado, Carolina; Ramos-Peñafiel, Christian Omar; Martínez-Murillo, Carlos; Romero-Guadarrama, Mónica; Olarte-Carrillo, Irma; Rozen-Fuller, Etta; Martínez-Tovar, Adolfo; Collazo-Jaloma, Juan; Mendoza-García, Carlos Alberto

    2013-01-01

    lymphoma is the most frequent lymphoid neoplasm in our country. Its diagnosis is based on histopathological findings. The lymph node imprint has been used for more than 40 years. The aim was to establish the sensitivity, specificity, positive predictive value and negative predictive value of lymph node imprint and estimate the inter-observer rate. we did an observational, retrospective, prolective study, based on the lymph node imprint obtained by excisional biopsies over a period of 6 years. the inclusion criteria was met on 199 samples, 27.1 % were considered as reactive (n = 54), 16.1 % Hodgkin lymphoma (n = 32), 40.2 % (n = 80) non-Hodgkin lymphoma and 16.6 % (n = 33) as metastatic carcinoma. Comparing with the final histopathology report, the sensitivity and specificity of lymph node imprint were 88 % (0.81-0.95) and 64 % (0.55-0.73) respectively, the positive predictive value was 67 % (0.59-0.76) and the negative predictive value was 86 % (0.79-0.94). The interobserver kappa index was 0.467. the lymph node imprint remains as a useful tool for the diagnosis of lymphoid neoplasm. The agreement between observers was acceptable.

  1. Orbital roof encephalocele mimicking a destructive neoplasm.

    Science.gov (United States)

    Alsuhaibani, Adel H; Hitchon, Patrick W; Smoker, Wendy R K; Lee, Andrew G; Nerad, Jeffrey A

    2011-01-01

    The purpose of this case report is to report an orbital roof encephalocele mimicking a destructive orbital neoplasm. Orbital roof encephalocele is uncommon but can mimic neoplasm. One potential mechanism for the orbital roof destruction is a post-traumatic "growing orbital roof fracture." The growing fracture has been reported mostly in children but can occur in adults. Alternative potential etiologies for the encephalocele are discussed, including Gorham syndrome. Orbital roof encephalocele is uncommon in adults, and the findings can superficially resemble an orbital neoplasm. Radiographic and clinical features that might suggest the correct diagnosis include a prior history of trauma, overlying frontal lobe encephalomalacia without significant mass effect or edema, and an orbital roof defect. The "growing fracture" mechanism may be a potential explanation for the orbital roof destruction in some cases.

  2. Intrathoracic neoplasms in the dog and cat

    International Nuclear Information System (INIS)

    Weller, R.E.

    1991-06-01

    Neoplasms of the thoracic cavity are as diverse as the structures and tissues that comprise the thorax. This paper summarizes the clinical signs, diagnosis and treatment of thoracic neoplasms in the dog and cat. Specific diagnostic techniques are evaluated, as is the utility of imaging techniques for clinical staging. Surgery is recommended as the treatment of choice for intrathoracic neoplasms, with exception for multiple tumor masses, metastasis, or poor patient health. Radiation therapy, chemotherapy, and hyperthermia are discussed individually or in combination with surgery or each other. Prognosis for specific tumors is discussed, as is lymph node involvement as a prognostic indicator. As the use of newer diagnostic procedures become more available in veterinary medicine, it should be possible to offer patients a variety of positive choices that will enhance their survival and quality of life

  3. Pigmented poroid neoplasm mimicking nodular melanoma.

    Science.gov (United States)

    Mitsuishi, Tsuyoshi; Ansai, Shin-ichi; Ueno, Takashi; Kawana, Seiji

    2010-06-01

    We reported the case of a 92-year-old woman with a pigmented and non-pigmented surface of the pedunculated nodule on her lower leg. Microscopic examination revealed that this nodule consisted of a component of small, dark, homogenous, poroid cells and cuticular cells in the dermis. The histopathological features of the lesion were consistent with poroid neoplasm. Immunohistochemistry showed that HMB-45 and Melan-A were positive in malanocytes and melanophages of the pigmented areas. Unlike most poroid neoplasms, this case showed pigmented lesion mimicked nodular melanoma.

  4. Challenges in Consolidated Reporting of Hematopoietic Neoplasms.

    Science.gov (United States)

    Ohgami, Robert S; Arber, Daniel A

    2013-12-01

    This article focuses on the challenges of generating comprehensive diagnostic reports in hematopathology. In particular, two main challenges that diagnosticians face are (1) interpreting and understanding the rapid advances in molecular and genetic pathology, which have gained increasing importance in classifications of hematopoietic neoplasms, and (2) managing the logistics of reporting ancillary studies and incorporating them effectively into a final synthesized report. This article summarizes many important genetic findings in hematopoietic neoplasms, which are required for accurate diagnoses, and discusses practical issues to generating accurate and complete hematopathology reports. Copyright © 2013 Elsevier Inc. All rights reserved.

  5. Solid and papillary neoplasm of the pancreas

    DEFF Research Database (Denmark)

    Jørgensen, L J; Hansen, A B; Burcharth, F

    1992-01-01

    In two cases of solid and papillary neoplasm of the pancreas (SPN), positive staining for argyrophil granules, chromogranin-A, neuron-specific enolase, chymotrypsin, alpha 1-antitrypsin, vimentin, cytokeratin, and estrogen receptors was present. Ultrastructurally, neurosecretory as well as zymoge...

  6. Solid Pseudopapillary Neoplasm of the Pancreas | Waithaka ...

    African Journals Online (AJOL)

    Solid pseudopapillary neoplasm is a rare pancreatic tumour predominantly affecting young women. We present two cases in young female patients. Both tumours were surgically removed as abdominal masses, one from the pancreatic tail and the other posterior to the stomach with an unclear organ of origin. On gross ...

  7. The new WHO nomenclature: lymphoid neoplasms.

    Science.gov (United States)

    Leclair, Susan J; Rodak, Bernadette F

    2002-01-01

    The development of the WHO classification of lymphoid neoplasms is a remarkable example of cooperation and communication between pathologists and oncologists from around the world. Joint classification committees of the major hematopathology societies will periodically review and update this classification, facilitating further progress in the understanding and treatment of hematologic malignancies.

  8. Radiation treatment of spinal cord neoplasms

    International Nuclear Information System (INIS)

    Smirnov, R.V.

    1982-01-01

    Results of radiation treatment of spinal cord neoplasms are presented. The results of combined (surgical and radiation) treatment of tumors are studied. On the whole it is noted that radiation treatment of initial spinal cord tumours is not practised on a large scale because of low radiostability of spinal cord

  9. Radiologic features of cystic, endocrine and other pancreatic neoplasms

    International Nuclear Information System (INIS)

    Balci, N. Cem; Semelka, Richard C.

    2001-01-01

    This article presents imaging features of cystic, endocrine and other pancreatic neoplasms. Microcystic adenoma which is composed of small cysts ( 2 cm) are accounted for mucinous cystic neoplasms, its variant along pancreatic duct is ductectatic mucinous cystic neoplasm. Endocrine tumors of pancreas are hypervascular and can be depicted on early dynamic enhanced crosssectional imaging modalities or on angiography when they are <1 cm. Pancreatic metastases and lymphomas are rare neoplasms which should also be included in differential diagnosis for pancreatic masses

  10. INTRACRANIAL NEOPLASMS IN IBADAN, NIGERIA B.J. OLASODE ...

    African Journals Online (AJOL)

    hi-tech

    2000-01-01

    Jan 1, 2000 ... embryogenetic classifications of intracranial neoplasms in which terms like neuroblastoma, spongioblastoma, astroblastoma and ependymoblastoma were coined to indicate neoplasms arising from these primitive cells(1). Advances in our understanding of the morphobiology of intracranial neoplasms have ...

  11. Transmission of a common intestinal neoplasm in zebrafish by cohabitation.

    Science.gov (United States)

    Burns, A R; Watral, V; Sichel, S; Spagnoli, S; Banse, A V; Mittge, E; Sharpton, T J; Guillemin, K; Kent, M L

    2017-10-11

    Intestinal neoplasms are common in zebrafish (Danio rerio) research facilities. These tumours are most often seen in older fish and are classified as small cell carcinomas or adenocarcinomas. Affected fish populations always contain subpopulations with preneoplastic lesions, characterized by epithelial hyperplasia or inflammation. Previous observations indicated that these tumours are unlikely caused by diet, water quality or genetic background, suggesting an infectious aetiology. We performed five transmission experiments by exposure of naïve fish to affected donor fish by cohabitation or exposure to tank effluent water. Intestinal lesions were observed in recipient fish in all exposure groups, including transmissions from previous recipient fish, and moribund fish exhibited a higher prevalence of neoplasms. We found a single 16S rRNA sequence, most similar to Mycoplasma penetrans, to be highly enriched in the donors and exposed recipients compared to unexposed control fish. We further tracked the presence of the Mycoplasma sp. using a targeted PCR test on individual dissected intestines or faeces or tank faeces. Original donor and exposed fish populations were positive for Mycoplasma, while corresponding unexposed control fish were negative. This study indicates an infectious aetiology for these transmissible tumours of zebrafish and suggests a possible candidate agent of a Mycoplasma species. © 2017 John Wiley & Sons Ltd.

  12. Clinical features of pancreatic cystic neoplasms and its therapeutic strategy

    Directory of Open Access Journals (Sweden)

    ZHANG Jiali

    2016-05-01

    Full Text Available The detection rate of pancreatic cystic neoplasms obviously increases with the development of imaging technology. However, it is still difficult to make the differential diagnosis between different types of pancreatic cystic neoplasms. For pancreatic cystic neoplasms, the physical condition of the patient, tumor size, location, patient preference, and other potential factors should be considered to develop an individualized treatment. The incidence rate of complications is high, although the operative mortality of pancreatic cystic neoplasms is very low. Therefore, it is necessary to strictly make the treatment decision for pancreatic cystic neoplasms.

  13. Migrated herniated disc mimicking a neoplasm.

    Science.gov (United States)

    Hoch, Benjamin; Hermann, George

    2010-12-01

    Disc sequestration is defined as migration of a herniated disc fragment into the epidural space such that it is completely separated from the parent disc. We report a case of a migrated herniated disc that was initially pathologically diagnosed as a cartilage neoplasm. In addition to confounding morphological features, this interpretation may have been influenced by an initial radiological interpretation that did not include herniated disc in the differential diagnosis of a spinal lesion with prominent peripheral contrast enhancement. MR imaging is most helpful in considering other lesions in the differential diagnosis including abscess, hematoma, and primary or metastatic neoplasms. Pathologically, degenerative changes in herniated discs, including clustering of chondrocytes and neovascularization, may be severe resulting in a pseudoneoplastic appearance. Increased awareness of the radiological and pathological features of migrated herniated disc should limit confusion with other tumors.

  14. Primary bone neoplasms in dogs: 90 cases

    Directory of Open Access Journals (Sweden)

    Maria E. Trost

    2012-12-01

    Full Text Available A retrospective study of necropsy and biopsy cases of 90 primary bone tumors (89 malignant and one benign in dogs received over a period of 22 years at the Laboratório de Patologia Veterinária, Universidade Federal de Santa Maria, was performed. Osteosarcoma was the most prevalent bone tumor, accounting for 86.7% of all malignant primary bone neoplasms diagnosed. Most cases occurred in dogs of large and giant breeds with ages between 6 and 10-years-old. The neoplasms involved mainly the appendicular skeleton, and were 3.5 times more prevalent in the forelimbs than in the hindlimbs. Osteoblastic osteosarcoma was the predominant histological subtype. Epidemiological and pathological findings of osteosarcomas are reported and discussed.

  15. Intrathoracic neoplasms in the dog and cat

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    Very little is known regarding the epidemiology, etiology, and mechanisms of spontaneous intrathoracic neoplasia in companion animals. Much of what we know or suspect about thoracic neoplasia in animals has been extrapolated from experimentally-induced neoplasms. Most studies of thoracic neoplasia have focused on the pathology of primary and metastatic neoplasms of the lung with little attention given to diagnostic and therapeutic considerations. Although the cited incidence rate for primary respiratory tract neoplasia is low, 8.5 cases per 100,000 dogs and 5.5 cases per 100,000 cats, intrathoracic masses often attract attention out of proportion to their actual importance since they are often readily visualized on routine thoracic radiographs.

  16. Surgery for Malignant Submandibular Gland Neoplasms.

    Science.gov (United States)

    Silver, Natalie L; Chinn, Steven B; Bradley, Patrick J; Weber, Randal S

    2016-01-01

    For many decades, surgery has been the primary treatment for malignant submandibular gland neoplasms. Nonetheless, due to the heterogeneity and rarity of submandibular gland malignant tumors and the high frequency of chronic benign processes in this region, management can be complex. Preoperative investigations, such as fine-needle aspiration and imaging, are critical to achieve the correct diagnosis so that appropriate surgery can be planned. In general, for malignant submandibular gland neoplasms, the minimal treatment necessary is excision of the submandibular gland with level I lymph node dissection. Salivary gland cancer in the submandibular gland is generally more aggressive than the same histologic type in the parotid gland. Neck dissection may be required and primarily depends on the stage and histological grade. Adjuvant therapy most frequently consists of radiation and can improve overall survival. Some factors that influence prognosis after surgical treatment include the histologic grade, stage at presentation, and positive surgical margins. © 2016 S. Karger AG, Basel.

  17. Neoplasms HIV associated Kaposi sarcoma not

    International Nuclear Information System (INIS)

    Lombardo, K.; Sosa, A.; Krygier, G.; Muse, I.

    2004-01-01

    Abstract - The incidence of malignancies in virus carriers acquired immunodeficiency (HIV) has increased in conjunction with the disease during the past decade. 40% of all AIDS patients develop cancer during the course of HIV infection. Kaposi's sarcoma (KS), Non-Hodgkin lymphoma (NHL) and cervical cancer have an impact extremely high in HIV infected patients, and they are considered as disease AIDS-defining stage. Many reports suggest that other neoplasms they can have a high impact on the population of HIV carrier, including head and neck carcinoma, rectal cancer - anal, plasma cytomas, and melanoma lung cancer. Methods - We examined the spectrum of cancer in HIV-infected patients, specifically neoplasms except Kaposi sarcoma diagnosed between 1/1998 - 6/2004. Information on age, sex, factors was gathered risk for AIDS, neoplasms and mortality rate. Results: The total number of patients in our study was 21 patients, what 15 were male (71%) and 6 females (29%); the median age was 36 (29-70). Tumors were reported: 11 Non-Hodgkin lymphomas (52%), 2 Hodgkin's lymphoma (6.6%), 1 medullary thyroid cancer (6.6%), 1 melanoma (6.6%), 1 rectal cancer (5%) and three head and neck cancers (14%), 1 cancer 1 lung and breast cancer. Five of the patients were intravenous drug abusers (24%); 4 patients were homosexual, bisexual March 8 straight, on 6 patients know the data. Conclusions - The spectrum of malignancies associated with infection HIV in our study was similar to that described in other populations. ratio between the immune system and the epidemiology of the virus-induced tumors is to importance to identify new therapeutic approaches in the treatment and / or prevention of these neoplasms

  18. MR appearance of skeletal neoplasms following cryotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Richardson, M.L. [Dept. of Radiology SB-05, Washington Univ., Seattle, WA (United States); Lough, L.R. [Pitts Radiological Associates, Columbia, SC (United States); Shuman, W.P. [Dept. of Radiology, Medical Center Hospital of Vermont, Burlington, VT (United States); Lazerte, G.D. [Dept. of Pathology RC-72, Washington Univ., Medical Center Hospital of Vermont, Burlington, VT (United States); Conrad, E.U. [Dept. of Orthopedic Surgery RK-10, Washington Univ., Medical Center of Vermont, Burlington, VT (United States)

    1994-02-01

    Cryotherapy is an increasingly popular mode of therapy adjunctive to surgical curettage in the treatment of certain skeletal neoplasms, such as giant cell tumors or chondrosarcomas. The magnetic resonance (MR) findings following cryotherapy have not been previously reported. We reviewed the MR findings in seven patients with skeletal neoplasms following curettage and cryotherapy. In six cases we found a zone of varying thickness extending beyond the surgical margins, corresponding to an area of cryoinjury to medullary bone. This zone displayed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, consistent with the presence of marrow edema. This zone of edema almost certainly reflects underlying thermal osteonecrosis. This zone may vary in size and intensity over time as the area of cryoinjury evolves or resolves. MR is currently the imaging procedure of choice for follow-up of most musculoskeletal neoplasms. Knowledge of the MR findings following cryotherapy should help prevent confusion during the interpretation of follow-up MR examinations. (orig.)

  19. MR appearance of skeletal neoplasms following cryotherapy

    International Nuclear Information System (INIS)

    Richardson, M.L.; Lough, L.R.; Shuman, W.P.; Lazerte, G.D.; Conrad, E.U.

    1994-01-01

    Cryotherapy is an increasingly popular mode of therapy adjunctive to surgical curettage in the treatment of certain skeletal neoplasms, such as giant cell tumors or chondrosarcomas. The magnetic resonance (MR) findings following cryotherapy have not been previously reported. We reviewed the MR findings in seven patients with skeletal neoplasms following curettage and cryotherapy. In six cases we found a zone of varying thickness extending beyond the surgical margins, corresponding to an area of cryoinjury to medullary bone. This zone displayed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, consistent with the presence of marrow edema. This zone of edema almost certainly reflects underlying thermal osteonecrosis. This zone may vary in size and intensity over time as the area of cryoinjury evolves or resolves. MR is currently the imaging procedure of choice for follow-up of most musculoskeletal neoplasms. Knowledge of the MR findings following cryotherapy should help prevent confusion during the interpretation of follow-up MR examinations. (orig.)

  20. Endocrine neoplasms in familial syndromes of hyperparathyroidism.

    Science.gov (United States)

    Li, Yulong; Simonds, William F

    2016-06-01

    Familial syndromes of hyperparathyroidism, including multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the hyperparathyroidism-jaw tumor (HPT-JT), comprise 2-5% of primary hyperparathyroidism cases. Familial syndromes of hyperparathyroidism are also associated with a range of endocrine and nonendocrine tumors, including potential malignancies. Complications of the associated neoplasms are the major causes of morbidities and mortalities in these familial syndromes, e.g., parathyroid carcinoma in HPT-JT syndrome; thymic, bronchial, and enteropancreatic neuroendocrine tumors in MEN1; and medullary thyroid cancer and pheochromocytoma in MEN2A. Because of the different underlying mechanisms of neoplasia, these familial tumors may have different characteristics compared with their sporadic counterparts. Large-scale clinical trials are frequently lacking due to the rarity of these diseases. With technological advances and the development of new medications, the natural history, diagnosis, and management of these syndromes are also evolving. In this article, we summarize the recent knowledge on endocrine neoplasms in three familial hyperparathyroidism syndromes, with an emphasis on disease characteristics, molecular pathogenesis, recent developments in biochemical and radiological evaluation, and expert opinions on surgical and medical therapies. Because these familial hyperparathyroidism syndromes are associated with a wide variety of tumors in different organs, this review is focused on those endocrine neoplasms with malignant potential. © 2016 Society for Endocrinology.

  1. Mucinous Neoplasms of the Vermiform Appendix.

    Science.gov (United States)

    Xiao, Shu-Yuan

    2010-06-01

    Most epithelial neoplasms of the vermiform appendix are of mucinous type and can be stratified into 3 main diagnostic categories: (1) adenoma, (2) mucinous neoplasms of uncertain malignant potential or low-grade mucinous neoplasm, and (3) adenocarcinoma. Clinically, appendiceal mucinous adenomas and adenocarcinomas may present as right lower abdominal pain mimicking acute appendicitis, a mass, or pseudomyxoma peritonei. Nomenclature currently in use to describe and diagnose mucinous tumors of the appendix, particularly those of low morphologic grade, varies among surgical pathologists and centers, resulting in different histologic and clinical features being attributed to these entities in the literature. It may be of help, as already attempted by some investigators, to simply apply algorithmic parameters for such lesions (grade of the primary lesion, extensiveness and composite of extra-appendiceal involvement, and so forth), instead of adopting rigid classification categories. This approach allows for more objective data to be collected in hopes that it will provide a more nuanced understanding of the clinical behavior of the spectrum of mucinous appendiceal tumors. Remaining focused on histopathologic parameters of the primary and secondary sites of involvement may help in avoiding circular reasoning. Copyright © 2010 Elsevier Inc. All rights reserved.

  2. Novel t(5;11)(q32;q13.4) with NUMA1-PDGFRB fusion in a myeloid neoplasm with eosinophilia with response to imatinib mesylate.

    Science.gov (United States)

    Zou, Ying S; Hoppman, Nicole L; Singh, Zeba N; Sawhney, Sameer; Kotiah, Sandy D; Baer, Maria R

    2017-04-01

    We report a NUMA1-PDGFRB fusion in a myeloproliferative neoplasm with eosinophilia in a 61-year old man, with response to imatinib mesylate therapy. A t(5;11) chromosome translocation involving bands 5q32 and 11q13.4 was identified by metaphase chromosome analysis, and rearrangement of the platelet-derived growth factor receptor beta (PDGFRB) gene on 5q32 was demonstrated by FISH using a PDGFRB break-apart probe set. Bacterial artificial chromosome (BAC) FISH mapping of the PDGFRB fusion partner gene narrowed the breakpoint at 11q13.4 to a 150 kb genomic region containing three genes, including NUMA1. Mate pair sequencing analysis demonstrated NUMA1-PDGFRB fusion. The fusion protein includes coiled-coil domains of nuclear mitotic apparatus protein 1 (NuMA1, involved in protein homodimerization and heteroassociation) and tyrosine kinase domains of PDGFRB. Diverse rearrangements involving the PDGFRB gene have been identified in myeloid and lymphoid neoplasms with eosinophilia, but rearrangement of the nuclear mitotic apparatus protein 1 (NUMA1) gene has previously been reported in a human malignancy in only one instance, a NUMA1-RARA fusion caused by a t(11;17) translocation in a patient with acute promyelocytic leukemia. The NUMA1-PDGFRB fusion is the second instance of rearrangement of NUMA1, encoding an element of the mitotic apparatus, in human cancer. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Agreement Between Cytology and Histopathology for Regional Lymph Node Metastasis in Dogs With Melanocytic Neoplasms.

    Science.gov (United States)

    Grimes, Janet A; Matz, Brad M; Christopherson, Pete W; Koehler, Jey W; Cappelle, Kelsey K; Hlusko, Katelyn C; Smith, Annette

    2017-07-01

    Melanocytic neoplasms are common in dogs and frequently occur within the oral cavity or in haired skin. The behavior of melanocytic neoplasms is variable and depends on tumor location, size, and histopathologic features. This study compared cytopathology and histopathology of 32 lymph nodes from 27 dogs diagnosed with melanocytic neoplasms. Agreement between the original cytology report, cytology slide review, original histopathology report, and histopathology slide review was determined for each lymph node. A subset of lymph nodes was subjected to immunohistochemistry (Melan-A) and additional histochemical stains/techniques (Prussian blue, bleach) to assist in differentiation of melanocytes and melanophages. Agreement ranged from slight to fair for each of the variables evaluated with weighted kappa (κ w ) or kappa (κ) analysis (original cytology vs cytology review κ w = 0.24; original cytology vs original histopathology κ w = 0.007; original cytology vs histopathology review κ w = 0.23; cytology review vs original histopathology κ w = 0.008; cytology review vs histopathology review κ w = 0.006; and original histopathology vs histopathology review κ = 0.18). The diagnoses (metastatic, equivocal, or negative for metastasis) of the original report and slide review for both cytology and histopathology were not significantly correlated with survival in this population of patients. Overall, agreement between cytology and histopathology was poor even with a single clinical or anatomic pathologist performing slide review. Consensus between routine cytology and histopathology for staging of lymph nodes in patients with melanocytic neoplasms is poor and does not correlate with survival.

  4. Molecular pathology of chondroid neoplasms: part 1, benign lesions

    Energy Technology Data Exchange (ETDEWEB)

    Bell, W.C. [University of Alabama at Birmingham, Department of Pathology, Birmingham, AL (United States); University of Alabama at Birmingham, Center for Metabolic Bone Disease, Birmingham, AL (United States); University of Alabama at Birmingham, Department of Diagnostic Radiology, Birmingham, AL (United States); Klein, M.J. [University of Alabama at Birmingham, Center for Metabolic Bone Disease, Birmingham, AL (United States); University of Alabama at Birmingham, Department of Pathology, Birmingham, AL (United States); University of Alabama at Birmingham, Department of Diagnostic Radiology, Birmingham, AL (United States); Pitt, M.J. [University of Alabama at Birmingham, Department of Diagnostic Radiology, Birmingham, AL (United States); University of Alabama at Birmingham, Center for Metabolic Bone Disease, Birmingham, AL (United States); Siegal, G.P. [University of Alabama at Birmingham, Departments of Pathology, Cell Biology, and Surgery, and the Center for Metabolic Bone Disease, Birmingham, AL (United States)

    2006-11-15

    This two-part review presents an overview of the molecular findings associated with both benign and malignant chondroid neoplasms. This first part presents a brief review of methods in molecular pathology along with a review of the cytogenetic and molecular genetic findings in benign chondroid neoplasms. Clinical aspects of the various lesions are briefly discussed, and each tumor is illustrated with representative radiographic and pathologic images. Malignant chondroid neoplasms will be considered in the second part of this review. (orig.)

  5. Molecular pathology of chondroid neoplasms: part 1, benign lesions

    International Nuclear Information System (INIS)

    Bell, W.C.; Klein, M.J.; Pitt, M.J.; Siegal, G.P.

    2006-01-01

    This two-part review presents an overview of the molecular findings associated with both benign and malignant chondroid neoplasms. This first part presents a brief review of methods in molecular pathology along with a review of the cytogenetic and molecular genetic findings in benign chondroid neoplasms. Clinical aspects of the various lesions are briefly discussed, and each tumor is illustrated with representative radiographic and pathologic images. Malignant chondroid neoplasms will be considered in the second part of this review. (orig.)

  6. Metastases in cranean of differential neoplasm tyroids

    International Nuclear Information System (INIS)

    Lopez Chapuis, D.; Garrido Vazquez, P.; Vallverdu Carbajal, M.

    1994-01-01

    Two cases of matastases are presented in cranial calota of differentiated neoplasm of tyroids, one of them without other distance lesions ,in which the cranial tumours was the element that it take was to the diagnose. For the local control the surgical resection of the metastasis is recommended in calota, associated to external radiotherapy , while that the total thyroidectomy allows the detection and treatment of other metastasis with Iodine. In this situation the prediction it is unfavourable, with a half survive of 4,5 year(AU) [es

  7. Application of MRI for the Diagnosis of Neoplasms

    Directory of Open Access Journals (Sweden)

    Ewa Bejer-Oleńska

    2018-01-01

    Full Text Available Aim. The aim of the study was to determine the most commonly diagnosed neoplasms in the MRI scanned patient population and indicate correlations based on the descriptive variables. Methods. The SPSS software was used to determine the incidence of neoplasms within the specific diagnoses based on the descriptive variables of the studied population. Over a five year period, 791 patients and 839 MRI scans were identified in neoplasm category (C00-D48 according to the International Statistical Classification of Diseases and Related Health Problems ICD-10. Results. More women (56% than men (44% represented C00-D48. Three categories of neoplasms were recorded. Furthermore, benign neoplasms were the most numerous, diagnosed mainly in patients in the fifth decade of life, and included benign neoplasms of the brain and other parts of the central nervous system. Conclusions. Males ≤ 30 years of age with neoplasms had three times higher MRI scans rate than females of the same age group; even though females had much higher scans rate in every other category. The young males are more often selected for these scans if a neoplasm is suspected. Finally, the number of MRI-diagnosed neoplasms showed a linear annual increase.

  8. Myeloproliferative Disease: An Unusual Cause of Raynaud's Phenomenon and Digital Ischaemia.

    Science.gov (United States)

    Beynon, Celia; Huws, Gwenan; Lawson, Tom

    2016-01-01

    We describe a 59-year-old female who presented with ischaemic digits, preceded by a 6-month history of Raynaud's phenomenon affecting her fingers and toes. There were no clinical or laboratory features of primary vasculitis or connective tissue disease, Doppler imaging was normal, and bloods were unremarkable aside from a platelet count of 786 × 109/L (150-400) and white cells of 16 × 109/L (4-11). In view of the thrombocytosis a JAK2 mutation assay was requested which confirmed a JAK2 V617F mutation, suggesting essential thrombocytosis (ET) as the cause. She received treatment with hydroxycarbamide which normalised her platelet count and led to a complete resolution of her Raynaud's symptoms. Raynaud's phenomenon is a rare manifestation of ET. Myeloproliferative disorders such as ET should be considered in the differential diagnosis of Raynaud's phenomenon and vasculitis.

  9. Mesenteric venous thrombosis secondary to an unsuspected JAK2 V617F-positive myeloproliferative disorder.

    LENUS (Irish Health Repository)

    2012-01-31

    BACKGROUND: Mesenteric venous thrombosis (MVT) is a rare but potentially fatal cause of mesenteric ischaemia. It presents insidiously and often diagnosis is made at emergency surgery. In half of the cases MVT develops without a causative factor, while in cases in which a pro-thrombotic state is found to exist MVT may be the first clinically detected consequence of that state. The myeloproliferative disorders (MPD) are known to contribute to the development of pro-thrombotic states. Recently, the JAK2 V617F mutation has been associated with the MPDs. CONCLUSION: We describe a case of MVT occurring secondary to an unsuspected MPD, in which the patient was subsequently found to carry this mutation. We highlight the necessity to screen for this mutation in cases of intra-abdominal thromboses so that appropriate systemic anticoagulation may be instituted, and the patient may be followed so as to detect the development of an overt MPD.

  10. Peptichemio in pretreated patients with plasmacell neoplasms.

    Science.gov (United States)

    Paccagnella, A; Salvagno, L; Chiarion-Sileni, V; Bolzonella, S; De Besi, P; Frizzarin, M; Pappagallo, G L; Fosser, V P; Fornasiero, A; Segati, R

    1986-09-01

    Twenty-one patients with alkylator-resistant plasmacell neoplasms were treated with Peptichemio (PTC) at a dose of 40 mg/m2 for 3 days every 3 weeks or, in the case of persistent leukopenia and/or thrombocytopenia, at the single dose of 70 mg/m2 every 2-3 weeks according to haematological recovery. Seventeen patients, 10 with multiple myeloma and seven with extramedullary plasmacytoma (EMP), were fully evaluable. Six of 17 patients (35%) responded: three of seven EMP patients had a complete remission and 3 of 10 multiple myeloma patients had an objective response greater than 50%. The median duration of response was 8.5 months. An EMP patient obtained a complete response lasting for 16 months. The most frequent toxic effect were phlebosclerosis, occurring in all the patients, and myelosuppression, which was severe in only one case. PTC appears to be an active drug in patients with plasmacell neoplasms even if resistant to alkylating agents.

  11. Patterns of second malignant neoplasms in children

    International Nuclear Information System (INIS)

    Meadows, A.T.; D'Angio, G.J.; Mike, V.; Banfi, A.; Harris, C.; Jenkin, R.D.T.; Schwartz, A.

    1977-01-01

    A search of the records of 10 pediatric oncology centers revealed 102 children with more than one malignant neoplasm. In this group of 102 patients, all pediatric cancers were seen as initial lesions, but Wilms' tumor and retinoblastoma were over-represented and leukemia and brain tumors underrepresented. Survival variation as well as tumor susceptibility may be responsible for this disproportion. Osteosarcomas and chondrosarcomas were the most frequent second malignant neoplasms (SMN). Embryonal tumors were rare as SMN and adult-type tumors (carcinomas) appeared at earlier than expected ages, whether arising after irradiation or not related to that form of therapy. Radiation was associated with 69 SMN, genetic disease accounted for 27 SMN and both conditions were noted in 15 SMN. In the group of 21 patients for whom neither radiation nor a known genetic disorder could be implicated, there were three with colon carcinoma and glioma and five with leukemia or lymphoma and glioma. These combinations may reflect new tissue-specific hereditary cancer syndromes

  12. Pancreatic neuroendocrine neoplasms; Neuroendokrine Neoplasien des Pankreas

    Energy Technology Data Exchange (ETDEWEB)

    Beiderwellen, K.; Lauenstein, T.C. [Universitaetsklinikum Essen, Institut fuer Diagnostische und Interventionelle Radiologie und Neuroradiologie, Essen (Germany); Sabet, A.; Poeppel, T.D. [Universitaetsklinikum Essen, Klinik fuer Nuklearmedizin, Essen (Germany); Lahner, H. [Universitaetsklinikum Essen, Klinik fuer Endokrinologie und Stoffwechselerkrankungen, Essen (Germany)

    2016-04-15

    Pancreatic neuroendocrine neoplasms (NEN) account for 1-2 % of all pancreatic neoplasms and represent a rare differential diagnosis. While some pancreatic NEN are hormonally active and exhibit endocrine activity associated with characteristic symptoms, the majority are hormonally inactive. Imaging techniques such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) or as combined PET/CT play a crucial role in the initial diagnosis, therapy planning and control. Endoscopic ultrasound (EUS) and multiphase CT represent the reference methods for localization of the primary pancreatic tumor. Particularly in the evaluation of small liver lesions MRI is the method of choice. Somatostatin receptor scintigraphy and somatostatin receptor PET/CT are of particular value for whole body staging and special aspects of further therapy planning. (orig.) [German] Neuroendokrine Neoplasien (NEN) des Pankreas stellen mit einem Anteil von 1-2 % aller pankreatischen Tumoren eine seltene Differenzialdiagnose dar. Ein Teil der Tumoren ist hormonell aktiv und faellt klinisch durch charakteristische Symptome auf, wohingegen der ueberwiegende Anteil hormonell inaktiv ist. Bildgebende Verfahren wie Sonographie, Computertomographie (CT), Magnetresonanztomographie (MRT) und nicht zuletzt Positronenemissionstomographie (PET oder kombiniert als PET/CT) spielen eine zentrale Rolle fuer Erstdiagnose, Therapieplanung und -kontrolle. Die Endosonographie und die multiphasische CT stellen die Referenzmethoden zur Lokalisation des Primaertumors dar. Fuer die Differenzierung insbesondere kleiner Leberlaesionen bietet die MRT die hoechste Aussagekraft. Fuer das Ganzkoerperstaging und bestimmte Aspekte der Therapieplanung lassen sich die Somatostatinrezeptorszintigraphie und v. a. die Somatostatinrezeptor-PET/CT heranziehen. (orig.)

  13. Cytogenetic Evolution in Myeloid Neoplasms at Relapse after Allogeneic Hematopoietic Cell Transplantation: Association with Previous Chemotherapy and Effect on Survival.

    Science.gov (United States)

    Ertz-Archambault, Natalie; Kosiorek, Heidi; Slack, James L; Lonzo, Melissa L; Greipp, Patricia T; Khera, Nandita; Kelemen, Katalin

    2017-05-01

    Cytogenetic evolution (CGE) in patients with myeloid neoplasms who relapsed after an allogeneic (allo) hematopoietic cell transplantation (HCT) has been evaluated by only few studies. The effect of the CGE on survival of relapsed allo-HCT recipients is not clear. The effect of previously received chemotherapy to induce CGE in this patient population has not been studied. The aims of our study are to (1) characterize the patterns of cytogenetic change in patients with myeloid neoplasms who relapsed after an allo-HCT, (2) evaluate the effect of CGE on survival, and (3) explore the association of CGE with previous chemotherapy (including the lines of salvage therapy, type of induction, and conditioning therapy). Of 49 patients with a myeloid malignancy (27 acute myeloid leukemia [AML], 19 myelodysplastic syndrome [MDS]/myeloproliferative neoplasm [MPN], and 3 chronic myelogenous leukemia) who relapsed after an allo-HCT, CGE was observed in 25 (51%), whereas 24 patients had unchanged cytogenetic findings at relapse. The CGE group carried more cytogenetic abnormalities at original diagnosis. The most frequent cytogenetic change was the acquisition of 3 or more new chromosomal abnormalities followed by acquisition of unbalanced abnormalities, aneuploidy, and emergence of apparently new clones unrelated to the original clone. The CGE cohort had higher proportion of MDS and MPN and fewer patients with de novo AML. Disease risk assessment category showed a trend to higher frequency of high-risk patients in the CGE group, though the difference was not statistically significant. Time from diagnosis to transplantation and time from transplantation to relapse were not different between the CGE and non-CGE groups. CGE and non-CGE cohorts had similar exposures to salvage therapy and to induction chemotherapy, as well as similar conditioning regimens; thus, no particular type of chemotherapy emerged as a predisposing factor to CGE. CGE was associated with significantly shortened

  14. The incidence of malignancy in neoplasms of the submandibular ...

    African Journals Online (AJOL)

    Objective. To test the validity of the reported high incidence (50%) of malignancy in neoplasms of the submandibular salivary gland, and to compare it with that of the parotid gland. Methods. This is a retrospective analysis of major salivary gland neoplasms in 127 patients who were treated between August 1988 and ...

  15. Multiple neoplasms, single primaries, and patient survival

    Directory of Open Access Journals (Sweden)

    Amer MH

    2014-03-01

    Full Text Available Magid H Amer Department of Medicine, St Rita's Medical Center, Lima, OH, USA Background: Multiple primary neoplasms in surviving cancer patients are relatively common, with an increasing incidence. Their impact on survival has not been clearly defined. Methods: This was a retrospective review of clinical data for all consecutive patients with histologically confirmed cancer, with emphasis on single versus multiple primary neoplasms. Second primaries discovered at the workup of the index (first primary were termed simultaneous, if discovered within 6 months of the index primary were called synchronous, and if discovered after 6 months were termed metachronous. Results: Between 2005 and 2012, of 1,873 cancer patients, 322 developed second malignancies; these included two primaries (n=284, and three or more primaries (n=38. Forty-seven patients had synchronous primaries and 275 had metachronous primaries. Patients with multiple primaries were predominantly of Caucasian ancestry (91.0%, with a tendency to develop thrombosis (20.2%, had a strong family history of similar cancer (22.3%, and usually presented with earlier stage 0 through stage II disease (78.9%. When compared with 1,551 patients with a single primary, these figures were 8.9%, 15.6%, 18.3%, and 50.9%, respectively (P≤0.001. Five-year survival rates were higher for metachronous cancers (95% than for synchronous primaries (59% and single primaries (59%. The worst survival rate was for simultaneous concomitant multiple primaries, being a median of 1.9 years. The best survival was for patients with three or more primaries (median 10.9 years and was similar to the expected survival for the age-matched and sex-matched general population (P=0.06991. Conclusion: Patients with multiple primaries are usually of Caucasian ancestry, have less aggressive malignancies, present at earlier stages, frequently have a strong family history of similar cancer, and their cancers tend to have indolent

  16. File list: Unc.Prs.05.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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    Lifescience Database Archive (English)

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    Lifescience Database Archive (English)

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  7. File list: His.Prs.05.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  8. Prognostic factors in childhood intracranial neoplasms

    International Nuclear Information System (INIS)

    Ampil, F.L.

    1987-01-01

    Thirty-six cases of primary intracranial neoplasm in children (over 1 year but under 13 years of age) seen at the university medical center between 1951 and 1982 were reviewed because of concern as to the results and after-effects of applied therapy. The overall 5-year actuarial survival rate was 17 %. Several factors of possible prognostic relevance, such as patient's age, intracranial location of the tumor, application or nonapplication of therapy, single or multiple modes of therapy, and extent of surgery, were analyzed. Completeness of surgical removal of the tumor proved to be the only statistically significant factor that correlated with survival. There was only one recorded case of severe learning disability and abnormal neuropsychologic development among the 12 living patients. The influence of patient's age (and technical factors) at the time of irradiation in correlation with the child's subsequent posttreatment functional performance, as reported in the literature, is reviewed. (author)

  9. Proteotypic classification of spontaneous and transgenic mammary neoplasms

    International Nuclear Information System (INIS)

    Mikaelian, Igor; Blades, Natalie; Churchill, Gary A; Fancher, Karen; Knowles, Barbara B; Eppig, Janan T; Sundberg, John P

    2004-01-01

    Mammary tumors in mice are categorized by using morphologic and architectural criteria. Immunolabeling for terminal differentiation markers was compared among a variety of mouse mammary neoplasms because expression of terminal differentiation markers, and especially of keratins, provides important information on the origin of neoplastic cells and their degree of differentiation. Expression patterns for terminal differentiation markers were used to characterize tumor types and to study tumor progression in transgenic mouse models of mammary neoplasia (mice overexpressing Neu (Erbb2), Hras, Myc, Notch4, SV40-TAg, Tgfa, and Wnt1), in spontaneous mammary carcinomas, and in mammary neoplasms associated with infection by the mouse mammary tumor virus (MMTV). On the basis of the expression of terminal differentiation markers, three types of neoplasm were identified: first, simple carcinomas composed exclusively of cells with a luminal phenotype are characteristic of neoplasms arising in mice transgenic for Neu, Hras, Myc, Notch4, and SV40-TAg; second, 'complex carcinomas' displaying luminal and myoepithelial differentiation are characteristic of type P tumors arising in mice transgenic for Wnt1, neoplasms arising in mice infected by the MMTV, and spontaneous adenosquamous carcinomas; and third, 'carcinomas with epithelial to mesenchymal transition (EMT)' are a characteristic feature of tumor progression in Hras-, Myc-, and SV40-TAg-induced mammary neoplasms and PL/J and SJL/J mouse strains, and display de novo expression of myoepithelial and mesenchymal cell markers. In sharp contrast, EMT was not detected in papillary adenocarcinomas arising in BALB/cJ mice, spontaneous adenoacanthomas, neoplasms associated with MMTV-infection, or in neoplasms arising in mice transgenic for Neu and Wnt1. Immunohistochemical profiles of complex neoplasms are consistent with a stem cell origin, whereas simple carcinomas might originate from a cell committed to the

  10. Multiple neoplasms, single primaries, and patient survival

    International Nuclear Information System (INIS)

    Amer, Magid H

    2014-01-01

    Multiple primary neoplasms in surviving cancer patients are relatively common, with an increasing incidence. Their impact on survival has not been clearly defined. This was a retrospective review of clinical data for all consecutive patients with histologically confirmed cancer, with emphasis on single versus multiple primary neoplasms. Second primaries discovered at the workup of the index (first) primary were termed simultaneous, if discovered within 6 months of the index primary were called synchronous, and if discovered after 6 months were termed metachronous. Between 2005 and 2012, of 1,873 cancer patients, 322 developed second malignancies; these included two primaries (n=284), and three or more primaries (n=38). Forty-seven patients had synchronous primaries and 275 had metachronous primaries. Patients with multiple primaries were predominantly of Caucasian ancestry (91.0%), with a tendency to develop thrombosis (20.2%), had a strong family history of similar cancer (22.3%), and usually presented with earlier stage 0 through stage II disease (78.9%). When compared with 1,551 patients with a single primary, these figures were 8.9%, 15.6%, 18.3%, and 50.9%, respectively (P≤0.001). Five-year survival rates were higher for metachronous cancers (95%) than for synchronous primaries (59%) and single primaries (59%). The worst survival rate was for simultaneous concomitant multiple primaries, being a median of 1.9 years. The best survival was for patients with three or more primaries (median 10.9 years) and was similar to the expected survival for the age-matched and sex-matched general population (P=0.06991). Patients with multiple primaries are usually of Caucasian ancestry, have less aggressive malignancies, present at earlier stages, frequently have a strong family history of similar cancer, and their cancers tend to have indolent clinical behavior with longer survival rates, possibly related to genetic predisposition

  11. Percutaneous thermal ablation of renal neoplasms

    International Nuclear Information System (INIS)

    Tacke, J.; Mahnken, A.H.; Guenther, R.W.

    2005-01-01

    Due to modern examination techniques such as multidetector computed tomography and high-field magnetic resonance imaging, the detection rate of renal neoplasms is continually increasing. Even though tumors exceeding 4 cm in diameter rarely metastasize, all renal lesions that are possible neoplasms should be treated. Traditional treatment techniques include radical nephrectomy or nephron-sparing resection, which are increasingly performed laparoscopically. Modern thermal ablation techniques such as hyperthermal techniques like radiofrequency ablation RFA, laser induced thermal ablation LITT, focused ultrasound FUS and microwave therapy MW, as well as hypothermal techniques (cryotherapy) may be a useful treatment option for patients who are unfit for or refuse surgical resection. Cryotherapy is the oldest and best known thermal ablation technique and can be performed laparoscopically or percutaneously. Since subzero temperatures have no antistyptic effect, additional maneuvers must be performed to control bleeding. Percutaneous cryotherapy of renal tumors is a new and interesting method, but experience with it is still limited. Radiofrequency ablation is the most frequently used method. Modern probe design allows volumes between 2 and 5 cm in diameter to be ablated. Due to hyperthermal tract ablation, the procedure is deemed to be safe and has a low complication rate. Although there are no randomized comparative studies to open resection, the preliminary results for renal RFA are promising and show RFA to be superior to other thermal ablation techniques. Clinical success rates are over 90% for both, cryo- and radiofrequency ablation. Whereas laser induced thermal therapy is established in hepatic ablation, experience is minimal with respect to renal application. For lesions of more than 2 cm in diameter, additional cooling catheters are required. MR thermometry offers temperature control during ablation. Microwave ablation is characterized by small ablation volumes

  12. Possible risk for gestational trophoblastic neoplasm in perimenopause and menopause

    Directory of Open Access Journals (Sweden)

    Nikolić Branka

    2011-01-01

    Full Text Available Gestational Trophoblastic Neoplasms (GTN are group of diseases which are known as fertilization disorders and may appear as Complete hydatidiform mole, Mole partialis, Invasive mole, Placental site trophoblastic tumor, Choriocarcinoma. Malignant disease precedes in approxi mately 50% of patients. All cases of GTN must be registrated. The Followe up programme period may last 6 months to 2 years until three sequential beta hCG values are negative. The risk of repeated GTN is low but patient has to be informed that risk is 1 : 74. GTN can appear in perimenopausal or menopausal women. That is the reason why each rapid enlargement of uterus especially with uterine bleeding followed with multiple cystic formations (grape like cysts needs a serious examination on GTN. Patient can complain of nausea, vomiting, painful breasts or hiperthyoidism. Legal abortion can precede GTN in perimenopausal women. In the great number of women with GTN the last pregnancy was 5 or more than 5 years before GTN is diagnosed. During 5 year period from june 1999. till june 2004, 58 GTN cases were diagnosed on our Department. 7 women with confirmed GTN were in perimenopause or menopause. All cases were hystologicalu confirmed with clinical low clinical score. In 1999. (March-June unpowerishment Uranium was used during war in Former Yugoslavia. Potential effect on reproductive potential could be analyzed after collecting data from the whole territory of Serbia and Montenegro in next years. All GTN patients are clinically, laboratory and ultrasonographicaly examined and staged according to FIGO 2002. recommendations

  13. Molecular pathology of chondroid neoplasms: part 2, malignant lesions

    International Nuclear Information System (INIS)

    Bell, W.C.; Klein, M.J.; Pitt, M.J.; Siegal, G.P.

    2006-01-01

    This is the second part of a two-part review presenting an overview of the molecular findings associated with both benign and malignant chondroid neoplasms. The first part presented a brief review of modern methods in molecular pathology, along with a review of the cytogenetic and molecular genetic findings in benign chondroid neoplasms. This second part reviews the cytogenetic and molecular genetic findings in malignant chondroid neoplasms. Clinical aspects of the various lesions are briefly discussed, and each tumor is illustrated with representative radiographic and pathologic images. (orig.)

  14. Molecular pathology of chondroid neoplasms: part 2, malignant lesions

    Energy Technology Data Exchange (ETDEWEB)

    Bell, W.C. [University of Alabama at Birmingham, Department of Pathology, Birmingham, AL (United States); University of Alabama at Birmingham, Center for Metabolic Bone Disease, Birmingham, AL (United States); Klein, M.J. [University of Alabama at Birmingham, Center for Metabolic Bone Disease, Birmingham, AL (United States); University of Alabama at Birmingham, Department of Pathology, Birmingham, AL (United States); Pitt, M.J. [University of Alabama at Birmingham, Department of Diagnostic Radiology, Birmingham, AL (United States); University of Alabama at Birmingham, Center for Metabolic Bone Disease, Birmingham, AL (United States); Siegal, G.P. [University of Alabama at Birmingham, Departments of Pathology, Cell Biology, and Surgery, Birmingham, AL (United States); University of Alabama at Birmingham, Center for Metabolic Bone Disease, Birmingham, AL (United States)

    2006-12-15

    This is the second part of a two-part review presenting an overview of the molecular findings associated with both benign and malignant chondroid neoplasms. The first part presented a brief review of modern methods in molecular pathology, along with a review of the cytogenetic and molecular genetic findings in benign chondroid neoplasms. This second part reviews the cytogenetic and molecular genetic findings in malignant chondroid neoplasms. Clinical aspects of the various lesions are briefly discussed, and each tumor is illustrated with representative radiographic and pathologic images. (orig.)

  15. Small-bowel neoplasms in patients undergoing video capsule endoscopy

    DEFF Research Database (Denmark)

    Rondonotti, E; Pennazio, M; Toth, E

    2008-01-01

    BACKGROUND AND STUDY AIM: Small-bowel tumors account for 1% - 3% of all gastrointestinal neoplasms. Recent studies with video capsule endoscopy (VCE) suggest that the frequency of these tumors may be substantially higher than previously reported. The aim of the study was to evaluate the frequency...... of the neoplasm seen in 29 centers of 10 European Countries. RESULTS: Of 5129 patients undergoing VCE, 124 (2.4%) had small-bowel tumors (112 primary, 12 metastatic). Among these patients, indications for VCE were: obscure gastrointestinal bleeding (108 patients), abdominal pain (9), search for primary neoplasm...

  16. Myeloproliferative disorders in patients with rheumatoid arthritis treated with total body irradiation

    International Nuclear Information System (INIS)

    Urowitz, M.B.; Rider, W.D.

    1985-01-01

    Four patients with refractory rheumatoid arthritis were treated with total body irradiation administered in two sittings, 300 to 400 rads to each half of the body. All four patients had taken antimetabolites prior to receiving total body irradiation, and two continued to use them after total body irradiation. Two patients had taken alkylating agents before, and one had used them after total body irradiation. All patients showed clinical improvement. However, in two patients myeloproliferative disorders developed: a myelodysplastic preleukemia at 40 months after total body irradiation in one and acute myelogenous leukemia at 25 months in the other. Total body irradiation differs from total nodal irradiation in the total dose of irradiation (300 to 400 rads versus 2,000 to 3,000), and in the duration of the therapy (two sittings versus treatment over several weeks to months). Furthermore, the patients in the total body irradiation study frequently used cytotoxic drugs before and/or after irradiation, whereas in one total nodal irradiation study, azathioprine (2 mg/kg per day or less) was permitted, but no other cytotoxic agents were allowed. Rheumatologists may therefore face a binding decision when deciding to treat a patient with rheumatoid arthritis with either a cytotoxic drug or irradiation

  17. Mutation Profile of JAK2 Transcripts in Patients with Chronic Myeloproliferative Neoplasias

    Science.gov (United States)

    Ma, Wanlong; Kantarjian, Hagop; Zhang, Xi; Yeh, Chen-Hsiung; Zhang, Zhong J.; Verstovsek, Srdan; Albitar, Maher

    2009-01-01

    Here, we describe the JAK2 mutation profile in a series of approximately 20,000 blood samples from patients with clinically suspected myeloproliferative neoplasias. Using a sensitive reverse transcription-PCR and direct sequencing approach on RNA rather than DNA, we detected JAK2 mutations in exons 12–15 in approximately 20% of these patients. We identified new mutations in addition to the known V617F and exon 12 mutations, which were the most common. Most of the novel mutations are located in the pseudokinase domain and therefore are expected to relieve the autoinhibitory function of this domain on JAK2 kinase activity. Our data suggest that molecular testing of JAK2 mutations should not be restricted to the V617F and exon 12 mutations, but perhaps should extend to most of the pseudokinase domain coding region as well. Furthermore, mutation screening using RNA is highly sensitive and could replace DNA-based testing because of the relative abundance of target transcripts and the ease in detecting deletion of the entire exon. PMID:19074595

  18. An addition to geographic hematology: chronic myeloproliferative diseases are infrequent in Mexican Mestizos.

    Science.gov (United States)

    Ruiz-Argüelles, Guillermo J; López-Martínez, Briceida; Lobato-Mendizábal, Eduardo; Ruiz-Delgado, Guillermo J

    2002-06-01

    The chronic myeloproliferative diseases (CMPDs) include chronic myelogenous leukemia (CML), primary (essential) thrombocythemia (PT), agnogenic myeloid metaplasia (AMM), and polycythemia vera (PV). Certain hematological malignancies have a different prevalence in our country than in countries with Caucasian populations. Data indicate that the prevalence of CML in our country is similar to that found in Caucasians; however, the prevalence of the other CMPDs has not been studied. In a total of 8069 individuals studied between June 1983 and March 2001 in the Centro de Hematologia y Medicina Interna de Puebla, we assessed the prevalence of CML, PT, AMM, and PV. Some of the clinical features of these individuals were also assessed. Forty-nine patients with CML, 14 with PT, 7 with AMM, and 3 with PV were identified. The clinical presentations of these CMPDs were not different from those described in Caucasians. We found that CML was more than 3 times more frequent than PT, that both PV and AMM were exceptional, and that PT, AMM, and PV were significantly less frequent in Mexican than in Caucasian populations (P < .01).

  19. Epidemiology of hemopoietic system neoplasms in Argentina.

    Science.gov (United States)

    Micheo, E Q; Calcagno, E J; de Sijvarger, S R; Calabria, S I; Maccione, E; Besuschio, S C; Magnasco, J H; Barros, C; Muriel, F S; de Soto, Z C

    1979-08-01

    The mortality caused by blood neoplasms in Argentina shows great irregularity. This was found to be caused in certain ways by (a) differences in the sexes and ages of the populations studied; (b) differences in available health services; and (c) environmental factors. Thus high rates and clusters of lymphomas and multiple mylomas were observed in zones with arsenical water, for example.In rural districts, the rates are lower, especially among old-aged people.Lower rates of leukemias were also observed among Spaniards compared to Italians (p=0.001) residing in Argentina. Turkish, Syrian, and Lebanese showed higher rates than Argentinians, Spaniards, or Italians.The results of a case-control study are given in which the following were observed: (a) Among the ancestors of cases HSN there are fewer Spaniards (not significant) and Latin-Americans (p=0.03) and more people who were born in Central or Eastern Europe (p=0.01). (b) In case group, there was more frequent contact with animals, especially dogs; and a greater exposure to petroleum and its products, and to insecticides.

  20. Eponyms in cardiothoracic radiology: Part I. Neoplasms.

    Science.gov (United States)

    Mohammed, Tan-Lucien H; Saettele, Megan R; Saettele, Timothy; Patel, Vikas; Kanne, Jeffrey P

    2014-01-01

    Eponyms serve the purpose of honoring individuals who have made important observations and discoveries. As with other fields of medicine, eponyms are frequently encountered in radiology, particularly in chest radiology. However, inappropriate use of an eponym may lead to potentially dangerous miscommunication. Moreover, an eponym may honor the incorrect person or a person who falls into disrepute. Despite their limitations, eponyms are still widespread in medical literature. Furthermore, in some circumstances, more than one individual may have contributed to the description or discovery of a particular anatomical structure or disease, whereas in others, an eponym may have been incorrectly applied initially and propagated for years in medical literature. Nevertheless, radiologic eponyms are a means of honoring those who have made lasting contributions to the field of radiology, and familiarity with these eponyms is critical for proper reporting and accurate communication. In addition, the acquisition of some historical knowledge about those whose names are associated with various structures or pathologic conditions conveys a sense of humanity in the field of medicine. In this article, the first of a multipart series, the authors discuss a number of chest radiology eponyms as they relate to neoplasms, including relevant clinical and imaging features, as well biographic information of the respective eponym׳s namesake. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. Squamous neoplasms arising within tattoos: clinical presentation, histopathology and management.

    Science.gov (United States)

    Junqueira, A L; Wanat, K A; Farah, R S

    2017-08-01

    Tattooing, which involves the placement of ink into the skin, is an ancient decorative technique that has remained popular in modern society. Tattoos have long been known to cause cutaneous reactions, which include the emergence of neoplasms such as keratoacanthoma (KA) and squamous cell carcinoma (SCC) in tattooed areas of the skin. We review the clinical presentations, histology and treatment options for squamous neoplasms, primarily KA and SCC, arising in tattoos. © 2017 British Association of Dermatologists.

  2. CT characteristics of primary retroperitoneal neoplasms in children

    Energy Technology Data Exchange (ETDEWEB)

    Xu Yufeng; Wang Jichen [Department of Radiology, Peking University First Hospital, No. 8, Xishike Street, Xicheng District, Beijing 100034 (China); Peng Yun [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Zeng Jinjin, E-mail: jzeng5567@yahoo.co [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China)

    2010-09-15

    Primary retroperitoneal neoplasms are uncommon in children. Retroperitoneal neoplasms are either mesodermal, neurogenic, germ cell ectodermal or lymphatic in origin. In general, primary retroperitoneal neoplasms in children have different spectrum and prevalence compared to those in adults. Neuroblastoma, rhabdomyosarcoma, benign teratoma and lymphoma are the common retroperitoneal neoplasms. In this review, the clinical and CT futures of common retroperitoneal neoplasms in children are described. Coarse, amorphous, and mottled calcification are very common in neuroblastoma. Paraganglioma tends to show marked and early enhancement and may present with clinical symptoms associated with the excess catecholamine. Sarcomas are often very large and have heterogeneous appearance. Imaging cannot be reliably used to identify the type of retroperitoneal sarcomas due to overlapped radiographic features. In children, lipoblastoma is the most common lipomatous tumor in the retroperitoneum. The percentage of visible fat in tumor varies depending on the cellular composition of the lesion. The CT characteristics of teratoma are quite variable, which may be cystic, solid, on a combination of both. Typically teratoma appears as a large complex mass containing fluid, fat, fat-fluid level, and calcifications. Lymphoma is often homogeneous on both enhanced and unenhanced CT scans. Necrosis and calcification are rare on CT. In conclusion, making a final histological diagnosis of retroperitoneal tumor base on CT features is not often possible; however, CT can help to develop a differential diagnosis and determine the size and extent of the retroperitoneal neoplasms.

  3. Anus neoplasm: study of a case series

    Directory of Open Access Journals (Sweden)

    Igor Lima Fernandes

    2011-09-01

    Full Text Available Anus neoplasm accounts for 2 to 4% of colorectal tumors, being more prevalent around the seventh and the eighth decades. Females are mostly affected, and the ratio is 3:1. Its increased prevalence amongst the population in the past years is probably related to the higher number of people that are affected by sexually transmitted diseases, mainly human papillomavirus (types 16 and 18, mostly and/or the human immunodeficiency virus. Diagnosis is based on clinical findings and anatomopathological tests. The treatment of choice is radiochemotherapy, and the rescue surgery with abdominoperineal resection is used for recurrence and persistence cases. A retrospective and prospective longitudinal observational study was performed with 11 patients diagnosed with anal neoplasm from 2004 to 2010. Six (54.5% were females and five (45.5% were males. The incidence was higher in the sixth decade, at the mean age of 54.45 years. The most frequent histological type observed was the epidermoid carcinoma, and the most frequent cell differentiation type was the moderately differentiated. Chemotharapy associated with radiotherapy was used in 81.9% of the patients, and abdominoperineal resection was necessary as a rescue surgery in 18.2% of the patients.Neoplasias do ânus correspondem de 2 a 4% dos tumores de intestino grosso, sendo predominante nas sétima e oitava décadas. A maior prevalência é em gênero feminino, com proporção de 3:1. O aumento da prevalência na população nos últimos anos provavelmente está relacionado ao número maior de pessoas com doenças sexualmente transmissíveis, principalmente o papilomavírus humano (tipos 16 e 18, mais comumente e/ou o vírus da imunodeficiência humana. O diagnóstico é feito a partir de achados clínicos somados ao exame anatomopatológico. O tratamento de escolha baseia-se na radioquimioterapia, sendo a cirurgia de resgate com amputação abdominoperineal utilizada para casos de recidiva ou persist

  4. Brief report: Loss of p15Ink4b accelerates development of myeloid neoplasms in Nup98-HoxD13 transgenic mice.

    Science.gov (United States)

    Humeniuk, Rita; Koller, Richard; Bies, Juraj; Aplan, Peter; Wolff, Linda

    2014-05-01

    Homeostasis of hematopoietic stem and progenitor cells is a tightly regulated process. The disturbance of the balance in the hematopoietic progenitor pool can result in favorable conditions for development of diseases such as myelodysplastic syndromes and leukemia. It has been shown recently that mice lacking p15Ink4b have skewed differentiation of common myeloid progenitors toward the myeloid lineage at the expense of erythroid progenitors. The lack of p15INK4B expression in human leukemic blasts has been linked to poor prognosis and increased risk of myelodysplastic syndromes transformation to acute myeloid leukemia. However, the role of p15Ink4b in disease development is just beginning to be elucidated. This study examines the collaboration of the loss of p15Ink4b with Nup98-HoxD13 translocation in the development of hematological malignancies in a mouse model. Here, we report that loss of p15Ink4b collaborates with Nup98-HoxD13 transgene in the development of predominantly myeloid neoplasms, namely acute myeloid leukemia, myeloproliferative disease, and myelodysplastic syndromes. This mouse model could be a very valuable tool for studying p15Ink4b function in tumorigenesis as well as preclinical drug testing. © 2014 AlphaMed Press.

  5. Changing patterns of radiosensitivity of hematopoietic progenitors from chronically irradiated dogs prone either to aplastic anemia or to myeloproliferative disease

    International Nuclear Information System (INIS)

    Seed, T.M.; Kaspar, L.V.

    1990-01-01

    Hematopoietic patterns have been assessed in chronic 60 Co gamma irradiated dogs during preclinical phases of evolving aplastic anemia (AA) or myeloproliferative disease (MPD), principally myeloid leukemia. The results support the concept that acquired radioresistance of vital granulocyte/monocyte lineage-committed hematopoietic progenitors is temporally, perhaps causally, linked to the processes mediating hematopoietic recovery and accommodation under chronic irradiation, and in turn to preclinical events of evolving MPD. In addition, the marked differential responses of progenitors to gamma and neutron irradiation in vitro might suggest differences in the nature of cellular lesions elicited by chronic gamma irradiation, in vivo. (author)

  6. Studies on 99Tcm-sulfur colloid bone marrow scintigraphy in myeloproliferative disorders

    International Nuclear Information System (INIS)

    Liu Yong; Zhang Yifan; Jia Fangxian; Kang Fu; Jian Shiquan

    2002-01-01

    Objective: To discuss the imaging features and changing patterns of bone marrow scintigraphy in myeloproliferative disorders (MPD) as well as its clinical significance. Methods: Bone marrow scintigraphy using 99 Tc m -sulfur colloid 370-550 MBq was performed on 85 MPD patients, including 40 cases of idiopathic myelofibrosis (IMF), 15 of polycythemia vera (PV), 5 of essential thrombocythaemia (ET), 30 of chronic granulocytic leukemia. Also, 40 cases of myelodysplastic syndromes (MDS) were observed in this study. Results: Abnormal bone marrow imaging was found in 88.2% of the 85 patients. The suppression rate of central bone marrow (CBM) and expansion rate of peripheral bone marrow (PBM) in these MPD patients were 61.2% and 56.5%, respectively. The imaging patterns was classified into three types according to the distribution and activity of bone marrow. 1) reduced imaging (31.8%); 2) increased and expanded imaging (27.1%); 3) depressed and expanded imaging (29.4%). Splenomegaly with minimal residual marrow activity was typical for late stages of MPD. Expansion of PBM was the further feature, but of no major importance for improving hematopoiesis of MPD, and it tended to retract during clinical recovery in chronic granulocytic leukemia (CGL). With expanding PBM, unmatched peripheral blood decreasing was found in MDS. The expansion pattern of PBM in different MPD was of relatively definite features. Conclusions: The imaging pattern of bone marrow was correlated with blood work-up data and clinical course or stages of MPD. Bone marrow scintigraphy may be proven useful in differential diagnosis and evaluation of clinical staging and prognosis of MPD

  7. Topics in histopathology of sweat gland and sebaceous neoplasms.

    Science.gov (United States)

    Ansai, Shin-Ichi

    2017-03-01

    This article reviews several topics regarding sweat gland and sebaceous neoplasms. First, the clinicopathological characteristics of poroid neoplasms are summarized. It was recently reported that one-fourth of poroid neoplasms are composite tumors and one-fourth are apocrine type lesions. Recent progress in the immunohistochemical diagnosis of sweat gland neoplasms is also reviewed. CD117 can help to distinguish sweat gland or sebaceous tumors from other non-Merkel cell epithelial tumors of the skin. For immunohistochemical differential diagnosis between sweat gland carcinoma (SGC) other than primary cutanesous apocrine carcinoma and skin metastasis of breast carcinoma (SMBC), a panel of antibodies may be useful, including p63 (SGC + , SMBC - ), CK5/6 (SGC + , SMBC - ), podoplanin (SGC + , SMBC - ) and mammaglobin (SGC - , SMBC + ). Comparison of antibodies used for immunohistochemical diagnosis of sebaceous carcinoma (SC) suggests that adipophilin has the highest sensitivity and specificity. Some authors have found that immunostaining for survivin, androgen receptor and ZEB2/SIP1 has prognostic value for ocular SC, but not extraocular SC. In situ SC is rare, especially extraocular SC, but there have been several recent reports that actinic keratosis and Bowen's disease are the source of invasive SC. Finally, based on recent reports, classification of sebaceous neoplasms into three categories is proposed, which are sebaceoma (a benign neoplasm with well-defined architecture and no atypia), borderline sebaceous neoplasm (low-grade SC; an intermediate tumor with well-defined architecture and nuclear atypia) and SC (a malignant tumor with invasive growth and evident nuclear atypia). © 2017 Japanese Dermatological Association.

  8. Solute carrier transporters: potential targets for digestive system neoplasms

    Directory of Open Access Journals (Sweden)

    Xie J

    2018-01-01

    Full Text Available Jing Xie,1,2 Xiao Yan Zhu,1,2 Lu Ming Liu,1,2 Zhi Qiang Meng1,2 1Department of Integrative Oncology, Fudan University Shanghai Cancer Center, 2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, People’s Republic of China Abstract: Digestive system neoplasms are the leading causes of cancer-related death all over the world. Solute carrier (SLC superfamily is composed of a series of transporters that are ubiquitously expressed in organs and tissues of digestive systems and mediate specific uptake of small molecule substrates in facilitative manner. Given the important role of SLC proteins in maintaining normal functions of digestive system, dysregulation of these protein in digestive system neoplasms may deliver biological and clinical significance that deserves systemic studies. In this review, we critically summarized the recent advances in understanding the role of SLC proteins in digestive system neoplasms. We highlighted that several SLC subfamilies, including metal ion transporters, transporters of glucose and other sugars, transporters of urea, neurotransmitters and biogenic amines, ammonium and choline, inorganic cation/anion transporters, transporters of nucleotide, amino acid and oligopeptide organic anion transporters, transporters of vitamins and cofactors and mitochondrial carrier, may play important roles in mediating the initiation, progression, metastasis, and chemoresistance of digestive system neoplasms. Proteins in these SLC subfamilies may also have diagnostic and prognostic values to particular cancer types. Differential expression of SLC proteins in tumors of digestive system was analyzed by extracting data from human cancer database, which revealed that the roles of SLC proteins may either be dependent on the substrates they transport or be tissue specific. In addition, small molecule modulators that pharmacologically regulate the functions of SLC proteins were discussed for their possible

  9. Diagnosis of pancreatic neoplasms using a novel method of DNA methylation analysis of mucin expression in pancreatic juice.

    Directory of Open Access Journals (Sweden)

    Seiya Yokoyama

    Full Text Available Mucins (MUC play crucial roles in carcinogenesis and tumor invasion in pancreatic ductal adenocarcinoma (PDAC and intraductal papillary mucinous neoplasms (IPMNs. Our immunohistochemistry (IHC studies have shown a consensus position on mucin expression profiles in pancreatic neoplasms as follows: MUC1-positive but MUC2-negative expression in PDACs; MUC1-negative but MUC2-positive expression in intestinal-type IPMNs (dangerous type; MUC1-negative and MUC2-negative expression in gastric-type IPMNs (safe type; High MUC4 expression in PDAC patients with a poor outcome; and MUC4-positive expression in intestinal-type IPMNs. We also showed that three mucin genes (MUC1, MUC2 and MUC4 expression in cancer cell line was regulated by DNA methylation. We have developed a novel 'methylation-specific electrophoresis (MSE' method to analyze the DNA methylation status of mucin genes by high sensitivity and resolution. By using the MSE method, we evaluated pancreatic juice samples from 45 patients with various pancreatic lesions. The results were compared with final diagnosis of the pancreatic lesions including IHC of mucin expression in the paired pancreatic tissues. The results indicated that the DNA methylation status of MUC1, MUC2 and MUC4 in pancreatic juice matched with the mucin expression in tissue. Analyses of the DNA methylation status of MUC1, MUC2 and MUC4 were useful for differential diagnosis of human pancreatic neoplasms, with specificity and sensitivity of 87% and 80% for PDAC; 100% and 88% for intestinal-type IPMN; and 88% and 77% for gastric-type IPMN, respectively. In conclusion, MSE analysis of human pancreatic juice may provide useful information for selection of treatment for pancreatic neoplasms.

  10. SOLID PSEUDOPAPILLARY NEOPLASM OF THE PANCREAS.

    Science.gov (United States)

    Carlotto, Jorge Roberto Marcante; Torrez, Franz Robert Apodaca; Gonzalez, Adriano Miziara; Linhares, Marcelo Moura; Triviño, Tarcisio; Herani-Filho, Benedito; Goldenberg, Alberto; Lopes-Filho, Gaspar de Jesus; Lobo, Edson José

    2016-01-01

    The solid pseudopapillary neoplasm is a rare tumor of the pancreas. However, it´s etiology still maintain discussions. To analyze it´s clinical data, diagnosis and treatment. A retrospective study of medical records of all patients treated from January 1997 until July 2015. Were identified 17 cases. Most patients were women (94.11%) and the average age was 32.88 years. The main complaint was abdominal mass (47.05%). The most frequent location was in the body/tail of the pancreas (72.22%) and the most frequently performed surgery was distal pancreatectomy with splenectomy (64.70%). No patient had metastases at diagnosis. Conservative surgery for pancreatic parenchyma was performed in only three cases. The rate of complications in the postoperative period was 35.29% and the main complication was pancreatic fistula (29.41%). No patient underwent adjuvant treatment. The treatment is surgical and the most common clinical presentation is abdominal mass. Distal pancreatectomy with splenectomy was the most frequently performed surgery for its treatment. A neoplasia sólida pseudopapilar é tumor raro de pâncreas de tratamento cirúrgico. No entanto, sua causa ainda gera discussões. Analisar os dados clínicos, do diagnóstico e do tratamento da dessa neoplasia. Estudo retrospectivo com dados médicos de pacientes tratados entre janeiro de 1997 a julho de 2015. Foram identificados 17 casos. A maioria era de mulheres (94,11%) e a média de idade foi de 32,88 anos. A principal queixa era massa abdominal (47,05%). A localização mais frequente era no corpo/cauda do pâncreas (72,22%) e a operação mais realizada foi a pancreatectomia corpocaudal com esplenectomia (64,70%). Nenhum caso apresentou metástase no momento do diagnóstico. Operação conservadora de parênquima pancreático foi realizada em apenas três casos. A taxa de complicações no pós-operatório foi de 35,29% e a principal complicação foi fístula pancreática (29,41%). Nenhum paciente realizou

  11. The radiological and histopathological differential diagnosis of chordoid neoplasms in skull base

    Directory of Open Access Journals (Sweden)

    PAN Bin-cai

    2013-07-01

    -called physaliferous cells within myxoid matrix. Areas of cartilage presented in individual cases. The tumor cells of chordoma were diffusely immuno-positive for PCK and EMA. Chordoid meningioma is characterized by a homogeneous contrast-enhancing mass with dural tail sign. The epithelioid cells were arranged in cords and clusters within a myxoid matrix, which was highly reminiscent of chordoma. The ratio of myxoid component and conventional meningioma was different in tumors. EMA was detectable in all chordoid meningiomas, and 4/5 cases were positive for D2-40. That was a finding which was helpful in distinguishing chordoid meningioma from chordoma. Chordoid gliomas were localized in the third ventricle with homogeneous enhancement on MRI examination. The tumors were composed of clusters and cords of epithelioid tumor cells within a variably mucinous stroma containing lymphoplasmacytic infiltratation. The most distinctive immunohistochemical feature of chordoid gliomas is their strong diffuse reactivity for GFAP. EMA and PCK positive expression can also be seen focally in individual cases. Although extraskeletal myxoid chondrosarcomas share the similar radiological and histological appearance with chordoma, all chondrosarcomas in the present study are completely negative for epithelial markers except for immuno-positivity of S-100 focally. In addition, Galectin-3 can be detected in most of chordoid neoplasms in skull base. Conclusion The specific localization and MRI features of tumors are useful diagnostic clues for the differential diagnosis of chordoid neoplasms in skull base. However, combining with histological features, a panel of selected immunostains, including PCK, EMA, GFAP, S-100 and D2-40, is helpful in making an accurate diagnosis for those diagnostically challenging cases which usually appear to have atypical radiological features or in an unusual site. Ki-67 index and Galectin-3 are not recommended to use as diagnostic markers for chordoid neoplasms of skull base

  12. [Indications for liver transplantation in neoplasms of the liver].

    Science.gov (United States)

    Stauber, R E; Mischinger, H J; Trauner, M; Pristautz, H

    1993-01-01

    Orthotopic liver transplantation for hepatic neoplasms is controversial. In the past, liver transplantation was utilized to treat various advanced hepatic neoplasms such as hepatocellular carcinoma including the fibrolamellar variant, cholangiocellular carcinoma, epithelioid hemangio-endothelioma, and liver metastases. In many cases, total hepatectomy with orthotopic liver replacement is the only treatment option with intent to cure because of reduced liver function in cirrhotic patients limiting resectability. On the other hand, results of transplantation are poor; for hepatocellular carcinoma, the 5-year-survival probability averages only 20%. Thus, hepatic neoplasms have to compete with benign liver diseases for a limited supply of donor organs. However, success rates of liver transplantation were higher for fibrolamellar carcinoma and for epithelioid hemangioendothelioma. New treatment strategies for hepatocellular carcinoma including neoadjuvant chemotherapy and chemoembolization are currently being investigated. Results of liver transplantation for cholangiocellular carcinoma or hepatic metastases have been disappointing. Single cases have been successfully treated with the "cluster operation" designed by Starzl in 1988.

  13. Interdisciplinary Management of Cystic Neoplasms of the Pancreas

    Directory of Open Access Journals (Sweden)

    Linda S. Lee

    2012-01-01

    Full Text Available Cystic neoplasms of the pancreas are increasingly recognized due to the frequent use of abdominal imaging. It is reported that up to 20% of abdominal cross-sectional scans identify incidental asymptomatic pancreatic cysts. Proper characterization of pancreatic cystic neoplasms is important not only to recognize premalignant lesions that will require surgical resection, but also to allow nonoperative management of many cystic lesions that will not require resection with its inherent morbidity. Though reliable biomarkers are lacking, a wide spectrum of diagnostic modalities are available to evaluate pancreatic cystic neoplasms, including radiologic, endoscopic, laboratory, and pathologic analysis. An interdisciplinary approach to management of these lesions which incorporates recent, specialty-specific advances in the medical literature is herein suggested.

  14. Solute carrier transporters: potential targets for digestive system neoplasms.

    Science.gov (United States)

    Xie, Jing; Zhu, Xiao Yan; Liu, Lu Ming; Meng, Zhi Qiang

    2018-01-01

    Digestive system neoplasms are the leading causes of cancer-related death all over the world. Solute carrier (SLC) superfamily is composed of a series of transporters that are ubiquitously expressed in organs and tissues of digestive systems and mediate specific uptake of small molecule substrates in facilitative manner. Given the important role of SLC proteins in maintaining normal functions of digestive system, dysregulation of these protein in digestive system neoplasms may deliver biological and clinical significance that deserves systemic studies. In this review, we critically summarized the recent advances in understanding the role of SLC proteins in digestive system neoplasms. We highlighted that several SLC subfamilies, including metal ion transporters, transporters of glucose and other sugars, transporters of urea, neurotransmitters and biogenic amines, ammonium and choline, inorganic cation/anion transporters, transporters of nucleotide, amino acid and oligopeptide organic anion transporters, transporters of vitamins and cofactors and mitochondrial carrier, may play important roles in mediating the initiation, progression, metastasis, and chemoresistance of digestive system neoplasms. Proteins in these SLC subfamilies may also have diagnostic and prognostic values to particular cancer types. Differential expression of SLC proteins in tumors of digestive system was analyzed by extracting data from human cancer database, which revealed that the roles of SLC proteins may either be dependent on the substrates they transport or be tissue specific. In addition, small molecule modulators that pharmacologically regulate the functions of SLC proteins were discussed for their possible application in the treatment of digestive system neoplasms. This review highlighted the potential of SLC family proteins as drug target for the treatment of digestive system neoplasms.

  15. Canine Central Nervous System Neoplasm Phenotyping Using Tissue Microarray Technique.

    Science.gov (United States)

    Spitzbarth, I; Heinrich, F; Herder, V; Recker, T; Wohlsein, P; Baumgärtner, W

    2017-05-01

    Tissue microarrays (TMAs) represent a useful technique for the simultaneous phenotyping of large sample numbers and are particularly suitable for histopathologic tumor research. In this study, TMAs were used to evaluate semiquantitatively the expression of multiple antigens in various canine central nervous system (CNS) neoplasms and to identify markers with potential discriminative diagnostic relevance. Ninety-seven canine CNS neoplasms, previously diagnosed on hematoxylin and eosin sections according to the World Health Organization classification, were investigated on TMAs, with each tumor consisting of 2 cylindrical samples from the center and the periphery of the neoplasm. Tumor cells were phenotyped using a panel of 28 monoclonal and polyclonal antibodies, and hierarchical clustering analysis was applied to group neoplasms according to similarities in their expression profiles. Hierarchical clustering generally grouped cases with similar histologic diagnoses; however, gliomas especially exhibited a considerable heterogeneity in their positivity scores. Multiple tumor groups, such as astrocytomas and oligodendrogliomas, significantly differed in the proportion of positive immunoreaction for certain markers such as p75 NTR , AQP4, GFAP, and S100 protein. The study highlights AQP4 and p75 NTR as novel markers, helping to discriminate between canine astrocytoma and oligodendroglioma. Furthermore, the results suggest that p75 NTR and proteolipid protein may represent useful markers, whose expression inversely correlates with malignant transformation in canine astrocytomas and oligodendrogliomas, respectively. Tissue microarray was demonstrated to be a useful and time-saving tool for the simultaneous immunohistochemical characterization of multiple canine CNS neoplasms. The present study provides a detailed overview of the expression patterns of different types of canine CNS neoplasms.

  16. Analyses of critical target cell responses during preclinical phases of evolving chronic radiation-induced myeloproliferative disease-exploitation of a unique canine model

    International Nuclear Information System (INIS)

    Seed, T.M.; Kaspar, L.V.; Tolle, D.V.; Fritz, T.E.; Frazier, M.E.

    1988-01-01

    This document briefly summarizes and highlights ongoing studies on the cellular and molecular processes involved in the induction and progression of myeloid leukemia in dogs chronically exposed to low daily doses of wholebody gamma irradiation. Under such conditions, select groups of dogs exhibit extremely high frequencies of myeloproliferative disease (MPD) (i.e., /congruent/50%) of which myeloid leukemia is most prominent. 2 figs

  17. Diagnostic value of JAK2 V617F somatic mutation for myeloproliferative cancer in 49 488 individuals from the general population

    DEFF Research Database (Denmark)

    Nielsen, Camilla; Birgens, Henrik S; Nordestgaard, Børge G

    2013-01-01

    is unknown. We examined this question in 49 488 individuals from the Copenhagen General Population Study. We also examined the association between JAK2 V617F somatic mutation, rs10974944 germline genotype, haematological phenotype, any cancer, haematological cancer, myeloproliferative cancer, ischaemic heart...

  18. Unicentric Castleman’s Disease Masquerading Pancreatic Neoplasm

    Directory of Open Access Journals (Sweden)

    Saurabh Jain

    2012-01-01

    Full Text Available Castleman’s disease is a rare nonclonal proliferative disorder of the lymph nodes with an unknown etiology. Common locations of Castleman’s disease are mediastinum, neck, axilla, and abdomen. Castleman’s disease of a peripancreatic location masquerading as pancreatic neoplasm is an even rarer entity. On search of published data, we came across about 17 cases published on peripancreatic Castleman’s disease until now. Here we are reporting a case of retropancreatic Castleman's disease masquerading as retroperitoneal neoplasm in a 46-year-old male patient.

  19. Assessing the occupational nature of malignant lung neoplasms

    International Nuclear Information System (INIS)

    Sevc, J.; Klener, V.; Plank, V.

    1989-01-01

    The development of lung carcinoma in uranium miners is discussed. In spite of the decreasing radiation risks in mines, the absolute number of neoplasms has increased since the 1960's; this is due to the increasing number of miners, improved diagnostic methods and the aging of miners who thus enter higher age groups where a higher incidence of neoplasms can be expected. The probabilistic method was shown to be of help in deciding whether individual cases of lung carcinoma should be considered an occupational disease; new possible improvements of the method are suggested. (J.J.). 12 refs

  20. Secondary neoplasms of the larynx from a colonic adenocarcinoma

    DEFF Research Database (Denmark)

    Dadkhah, Naser; Hahn, Christoffer

    2015-01-01

    Secondary neoplasms of the larynx are rare and account for 0.09-0,4% of all laryngeal tumours. Cutaneous melanomas are the preponderant primaries metastasizing to the larynx, fol-lowed by renal cell carcinomas, breast and lung carcinomas. Colonic adenocarcinoma metastases to the larynx are extrem......Secondary neoplasms of the larynx are rare and account for 0.09-0,4% of all laryngeal tumours. Cutaneous melanomas are the preponderant primaries metastasizing to the larynx, fol-lowed by renal cell carcinomas, breast and lung carcinomas. Colonic adenocarcinoma metastases to the larynx...

  1. When negation is not negation

    OpenAIRE

    Milicevic, Nataša

    2008-01-01

    In this paper I will discuss the formation of different types of yes/no questions in Serbian (examples in (1)), focusing on the syntactically and semantically puzzling example (1d), which involves the negative auxiliary inversion. Although there is a negative marker on the fronted auxiliary, the construction does not involve sentential negation. This coincides with the fact that the negative quantifying NPIs cannot be licensed. The question formation and sentential negation have similar synta...

  2. The Continuing Value of Ultrastructural Observation in Central Nervous System Neoplasms in Children

    Directory of Open Access Journals (Sweden)

    Na Rae Kim

    2015-11-01

    Full Text Available Central nervous system (CNS neoplasms are the second most common childhood malignancy after leukemia and the most common solid organ neoplasm in children. Diagnostic dilemmas with small specimens from CNS neoplasms are often the result of multifactorial etiologies such as frozen or fixation artifact, biopsy size, or lack of knowledge about rare or unfamiliar entities. Since the late 1950s, ultrastructural examination has been used in the diagnosis of CNS neoplasms, though it has largely been replaced by immunohistochemical and molecular cytogenetic studies. Nowadays, pathologic diagnosis of CNS neoplasms is achieved through intraoperative cytology, light microscopy, immunohistochemistry, and molecular cytogenetic results. However, the utility of electron microscopy (EM in the final diagnosis of CNS neoplasms and investigation of its pathogenetic origin remains critical. Here, we reviewed the distinguishing ultrastructural features of pediatric CNS neoplasms and emphasize the continuing value of EM in the diagnosis of CNS neoplasms.

  3. Childhood neoplasms presenting at autopsy: A 20-year experience.

    Science.gov (United States)

    Bryant, Victoria A; Booth, John; Palm, Liina; Ashworth, Michael; Jacques, Thomas S; Sebire, Neil J

    2017-09-01

    The aims of the review are to establish the number of undiagnosed neoplasms presenting at autopsy in a single centre and to determine the incidence and most common causes of sudden unexpected death due to neoplasia in infancy and childhood (SUDNIC). Retrospective observational study of paediatric autopsies performed on behalf of Her Majesty's Coroner over a 20-year period (1996-2015; n = 2,432). Neoplasms first diagnosed at autopsy were identified from an established database and cases meeting the criteria for sudden unexpected death were further categorised. Thirteen previously undiagnosed neoplasms were identified, including five haematological malignancies, two medulloblastomas, two neuroblastomas, two cardiac tumours and two malignancies of renal origin. Eight cases met the criteria for SUDNIC (0.33% of autopsies), the commonest group of which were haematological malignancies (n = 3). Neoplasms presenting as unexpected death in infancy and childhood and diagnosed at autopsy are rare. The findings suggest that haematological malignancies are the commonest cause of SUDNIC and highlight the importance of specialist autopsy in cases of sudden unexpected death. © 2017 Wiley Periodicals, Inc.

  4. The radiotherapy value in recurrence control mammary neoplasm

    International Nuclear Information System (INIS)

    Torres Lopez, M.; Santini Blasco, A.

    1996-01-01

    In base on the retrospective analysis of 250 patient carrying of mammals neoplasms recurrence, their general characteristics are determined and the results are emphasized obtained by the radiotherapy, as well as the list that the indicative predict. It culminates in the necessity of indicating the adyuvance postoperative radiotherapy when factors unfavorable presage of recurrence(AU ) [es

  5. [Epidermoid neoplasm of the fourth ventricle. Report of two cases].

    Science.gov (United States)

    Santos-Franco, Jorge Arturo; Vallejo-Moncada, Cristóbal; Collado-Arce, Griselda; Villalpando-Navarrete, Edgar; Sandoval-Balanzario, M

    2013-01-01

    epidermoid neoplasm (EN) accounts for 1 % of whole intracranial neoplasms. Usually, it is found at the cerebello-pontine angle and the location in the fourth ventricle (FV) is rare. The aim was to report two cases of EN of the FV. case 1: a female 22 year old presented with an intense headache with a history of 3 months. At the hospital entry, symptoms and signs of high intracranial pressure were found. Tomography images showed hydrocephalus with high pressure in the FV. She was treated with a shunt from ventricular to peritoneal cavity. After that an encapsulated neoplasm was drawn. It had a pearled aspect. The histology report showed an EN originating in the FV. Case 2: a female 44 year old with a history of five years of dizziness; three years before admission she presented intermittent diplopia and disophagia. At the hospital admission the patient presented paresis of the 6th and 7th cranial nerve. The tomography and the magnetic resonance studies showed a mass in the FV. The neoplasm was extirpated. the EN of the FV is an infrequent benign lesion. Magnetic resonance is the standard diagnostic study, but it could lead to confusion with neurocisticercosis. The extirpation and the treatment of the hydrocephalus are indicated.

  6. Solid pseudopapillary epithelial neoplasm – a rare but curable ...

    African Journals Online (AJOL)

    Background. Solid pseudopapillary epithelial neoplasms (SPENs) of the pancreas are rare but curable tumours that have a low-grade malignant potential and occur almost exclusively in young women, with an excellent prognosis after complete resection. This study examines the clinicopathological characteristics of these ...

  7. A retrospective study of ocular neoplasms in Benin City, Nigeria ...

    African Journals Online (AJOL)

    The frequency of retinoblastoma was higher than previously reported while the frequency of choroidal malanoma was less than that seen in Literature. This may necessitate the need for further studies on retinoblastomas in Nigeria. KEY WORDS: Ocular Neoplasms, Malignant tumors, age, sex. Global Jnl Medical Sciences ...

  8. Situs inversus with renal neoplasm: a case report.

    African Journals Online (AJOL)

    Situs inversus with renal neoplasm: a case report. Je\\vell J MCS FACCP. Senior Consultant. Bowa K hlBChB hlsc (Glasgonl). Regstrar. Uni~ersity~Teaching Hospital, School of hledicine. University of Zambia. Correspondence to: Dr. Jewel1 J, Dept. of Surgery, UTH, P 0 Box 501 10, LUSAICA, ZAhlBIX. This is a case report ...

  9. Histologic and Immunohistochemical classification of 41 bovine adrenal gland neoplasms

    DEFF Research Database (Denmark)

    Grossi, Anette Blak; Leifsson, Páll S.; Jensen, Henrik Elvang

    2013-01-01

    Tumors of the adrenal glands are among the most frequent tumors in cattle; however, few studies have been conducted to describe their characteristics. The aim of this study was to classify 41 bovine adrenal neoplasms from 40 animals based on macroscopic and histologic examination, including...

  10. Reconstruction techniques in the treatment of vertebral neoplasms.

    Science.gov (United States)

    Biagini, R; Boriani, S; Casadei, R; Bandiera, S; De Iure, F; Campanacci, L; Demitri, S; Orsini, U; Di Fiore, M

    1997-01-01

    The authors present a new system for the topographical description of vertebral neoplasms. The general criteria of reconstruction after curettage or vertebral resection are evaluated. The literature is reviewed in terms of the use of prostheses, bone grafts, cement and stabilization systems in the treatment of tumors of the spine. Indications for the different methods are discussed.

  11. A Survey Of Cutaneous Neoplasms Among Horses Used For ...

    African Journals Online (AJOL)

    A Survey Of Cutaneous Neoplasms Among Horses Used For Cultural Festivals In Borno State, Nigeria. ... Histologically, the papillomas were deeply subdivided into epithelial lobules with the outer and inner aspects of the epithelium running approximately parallel to each other. Irregular sub-dermal masses or cords of ...

  12. Age-specific incidence of all neoplasms after colorectal cancer.

    Science.gov (United States)

    Levi, Fabio; Randimbison, Lalao; Blanc-Moya, Rafael; La Vecchia, Carlo

    2014-10-01

    Patients diagnosed with a specific neoplasm tend to have a subsequent excess risk of the same neoplasm. The age incidence of a second neoplasm at the same site is approximately constant with age, and consequently the relative risk is greater at younger age. It is unclear whether such a line of reasoning can be extended from a specific neoplasm to the incidence of all neoplasms in subjects diagnosed with a defined neoplasm. We considered the age-specific incidence of all non-hormone-related epithelial neoplasms after a first primary colorectal cancer (n = 9542) in the Vaud Cancer Registry data set. In subjects with a previous colorectal cancer, the incidence rate of all other epithelial non-hormone-related cancers was stable around 800 per 100,000 between age 30 and 60 years, and rose only about twofold to reach 1685 at age 70 to 79 years and 1826 per 100,000 at age 80 years or older. After excluding synchronous cancers, the rise was only about 1.5-fold, that is, from about 700 to 1000. In the general population, the incidence rate of all epithelial non-hormone-related cancers was 29 per 100,000 at age 30 to 39 years, and rose 30-fold to 883 per 100,000 at age 70 to 79 years. Excluding colorectal cancers, the rise of all non-hormone-related cancers was from 360 per 100,000 at age 40 to 49 years to 940 at age 70 to 79 years after colorectal cancer, and from 90 to 636 per 100,000 in the general population (i.e., 2.6- vs. 7.1-fold). The rise of incidence with age of all epithelial non-hormone-related second cancers after colorectal cancer is much smaller than in the general population. This can possibly be related to the occurrence of a single mutational event in a population of susceptible individuals, although alternative models are plausible within the complexity of the process of carcinogenesis. Copyright © 2014 Elsevier Inc. All rights reserved.

  13. Music in Reducing Anxiety and Pain in Adult Patients Undergoing Bone Marrow Biopsy for Hematologic Cancers or Other Diseases

    Science.gov (United States)

    2017-05-25

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Pain; Precancerous Condition; Psychosocial Effects of Cancer and Its Treatment

  14. Methadone, Morphine, or Oxycodone in Treating Pain in Patients With Cancer

    Science.gov (United States)

    2012-11-09

    Brain and Central Nervous System Tumors; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Pain; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  15. Opioid Titration Order Sheet or Standard Care in Treating Patients With Cancer Pain

    Science.gov (United States)

    2012-08-04

    Brain and Central Nervous System Tumors; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Pain; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  16. Survival after Radiofrequency Ablation for 100 Cases of Lung Neoplasms

    Directory of Open Access Journals (Sweden)

    Baodong LIU

    2011-04-01

    Full Text Available Background and objective Surgical resection is the preferred treatment in selected patients with pulmonary neoplasms. In older than 70 years or have compromised cardiopulmonary status or coexistent medical problems patients, radiofrequency ablation (RFA may offer an alternative option. The aim of this study is to evaluate the therapeutic effects after RFA in 100 patients with pulmonary neoplasms. Methods One handred cases of unresectable lung tumors with 106 lesions were underwent RFA therapy. To evaluate the therapeutic effect and complications of lung tumors using spiral CT scanning and SPECT in 1-3 months after RFA. Results One hundred patients underwent RFA for lung neoplasms (62 men, 38 women; median age, 66.6 years; range, 36 to 91 years. Eighty-six patients with primary lung neoplasms and 14 patients with pulmonary metastases underwent RFA. Treatment was complete in all cases, no treatment-related deaths occurred in all of the 100 patients and serious morbidity associated with the procedures. The median overall survival for the entire group of patients was 13.0 months, the one and two years overall survival for total of were 51% and 32.5% respectively. No differences in overall survival noted between patients with primary and metastases lung neoplasms (P=0.922. The median overall survival for the early stage of patients was 28.0 months, 2-year overall survival for early stage primary lung cancer patients were 57.7%. Conclusion RFA is a safe and effective procedure in selective lung tumors. CT-guided radiofrequency ablation is a minimally invasive treatment option. RFA could act as an alternative treatment to inoperable lung cancer.

  17. File list: ALL.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  18. File list: Pol.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  19. File list: ALL.Prs.20.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  20. File list: His.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  1. File list: InP.Prs.10.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  2. File list: ALL.Prs.10.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  3. File list: Oth.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  4. File list: NoD.Prs.20.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  5. File list: Oth.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  6. File list: DNS.Prs.50.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  7. File list: Pol.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Pol.Neu.05.AllAg.Nerve_Sheath_Neoplasms mm9 RNA polymerase Neural Nerve Sheath Neoplasms... http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Pol.Neu.05.AllAg.Nerve_Sheath_Neoplasms.bed ...

  8. File list: NoD.Prs.50.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  9. File list: Oth.Prs.05.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  10. File list: Unc.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  11. File list: Oth.Prs.10.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  12. File list: Pol.Neu.20.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Pol.Neu.20.AllAg.Nerve_Sheath_Neoplasms mm9 RNA polymerase Neural Nerve Sheath Neoplasms... http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Pol.Neu.20.AllAg.Nerve_Sheath_Neoplasms.bed ...

  13. File list: Oth.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.Neu.50.AllAg.Nerve_Sheath_Neoplasms mm9 TFs and others Neural Nerve Sheath Neoplasms... SRX337965 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.Neu.50.AllAg.Nerve_Sheath_Neoplasms.bed ...

  14. File list: ALL.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available ALL.Neu.10.AllAg.Nerve_Sheath_Neoplasms mm9 All antigens Neural Nerve Sheath Neoplasms... SRX337965 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.Neu.10.AllAg.Nerve_Sheath_Neoplasms.bed ...

  15. File list: His.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  16. File list: DNS.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Neu.10.AllAg.Nerve_Sheath_Neoplasms mm9 DNase-seq Neural Nerve Sheath Neoplasms... http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/DNS.Neu.10.AllAg.Nerve_Sheath_Neoplasms.bed ...

  17. File list: Oth.Prs.50.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.Prs.50.AllAg.Prostatic_Neoplasms mm9 TFs and others Prostate Prostatic Neoplasms... SRX739215,SRX739214,SRX739216,SRX739217 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.Prs.50.AllAg.Prostatic_Neoplasms.bed ...

  18. File list: DNS.Prs.10.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Prs.10.AllAg.Prostatic_Neoplasms mm9 DNase-seq Prostate Prostatic Neoplasms htt...p://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/DNS.Prs.10.AllAg.Prostatic_Neoplasms.bed ...

  19. File list: His.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  20. File list: ALL.Prs.50.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  1. File list: ALL.Prs.05.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available ALL.Prs.05.AllAg.Prostatic_Neoplasms mm9 All antigens Prostate Prostatic Neoplasms ...SRX739215,SRX739213,SRX739214,SRX739216,SRX739217 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.Prs.05.AllAg.Prostatic_Neoplasms.bed ...

  2. File list: DNS.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  3. File list: Unc.Neu.20.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  4. File list: DNS.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Neu.05.AllAg.Nerve_Sheath_Neoplasms mm9 DNase-seq Neural Nerve Sheath Neoplasms... http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/DNS.Neu.05.AllAg.Nerve_Sheath_Neoplasms.bed ...

  5. File list: DNS.Prs.20.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Prs.20.AllAg.Prostatic_Neoplasms mm9 DNase-seq Prostate Prostatic Neoplasms htt...p://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/DNS.Prs.20.AllAg.Prostatic_Neoplasms.bed ...

  6. File list: Unc.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  7. File list: Unc.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  8. File list: ALL.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  9. File list: DNS.Prs.05.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  10. File list: Oth.Neu.20.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  11. File list: His.Neu.20.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  12. File list: InP.Prs.05.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  13. File list: Pol.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  14. File list: InP.Prs.20.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  15. File list: Oth.Prs.20.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  16. File list: DNS.Neu.20.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  17. File list: ALL.Neu.20.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  18. File list: NoD.Prs.05.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  19. File list: NoD.Prs.10.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  20. File list: InP.Prs.50.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  1. Negative ... concord?

    NARCIS (Netherlands)

    Giannakidou, A

    The main claim of this paper is that a general theory of negative concord (NC) should allow for the possibility of NC involving scoping of a universal quantifier above negation. I propose that Greek NC instantiates this option. Greek n-words will be analyzed as polarity sensitive universal

  2. Food habits in atomic bomb survivors suffering from malignant neoplasms

    International Nuclear Information System (INIS)

    Morimoto, Kazue; Inoue, Hisako; Uchino, Chito

    1984-01-01

    Food habits were surveyed in patients admitted to 13 hospitals in Nagasaki prefecture and other prefectures to compare the incidence of malignant neoplasms according to the food intake between atomic bomb exposed group and non-exposed group. The incidence of malignant neoplasms was significantly higher in male patients having the low intake of milk and salted fish than in those having the high intake of them in atomic bomb exposed group, while it was significantly higher in male patients having the low intake of potatoes and milk and in female patients having the low intake of boiled fish paste than in those having the high intake of them in non-exposed group. (Namekawa, K.)

  3. Histopathological evaluation of parotid gland neoplasms in Queensland, Australia.

    Science.gov (United States)

    Coombe, R F; Lam, A K; O'Neill, J

    2016-01-01

    Parotid gland tumours are complex neoplasms with a broad histological range. The parotid gland is also a common site of face and scalp skin cancer metastases. Parotidectomies performed by ENT department in the Gold Coast health district from 2006 to 2013. A total of 158 specimens were examined. Of these, 53.80 per cent were benign and 46.20 per cent were malignant. Pleomorphic adenoma was the most common tumour (29.11 per cent), followed by cutaneous squamous cell carcinoma (23.42 per cent) and Warthin's tumour (12.03 per cent). Metastatic squamous cell carcinoma accounted for a large proportion of parotid masses in our case series, reflecting the high prevalence of non-melanoma skin cancer in Australia. Primary parotid neoplasms had similar incidence rates to other studies.

  4. Lesions and Neoplasms of the Penis: A Review.

    Science.gov (United States)

    Heller, Debra S

    2016-01-01

    In addition to practitioners who care for male patients, with the increased use of high-resolution anoscopy, practitioners who care for women are seeing more men in their practices as well. Some diseases affecting the penis can impact on their sexual partners. Many of the lesions and neoplasms of the penis occur on the vulva as well. In addition, there are common and rare lesions unique to the penis. A review of the scope of penile lesions and neoplasms that may present in a primary care setting is presented to assist in developing a differential diagnosis if such a patient is encountered, as well as for practitioners who care for their sexual partners. A familiarity will assist with recognition, as well as when consultation is needed.

  5. DOG1 (clone K9) is seldom expressed and not useful in the evaluation of pancreatic neoplasms.

    Science.gov (United States)

    Hemminger, Jessica; Marsh, William L; Iwenofu, Obiajulu Hans; Frankel, Wendy L

    2012-07-01

    DOG1, a transmembrane calcium-regulated chloride channel protein, is a sensitive and specific marker for gastrointestinal stromal tumors compared with other spindle cell and epithelioid neoplasms. Overexpression has also been described in a variety of both benign and malignant epithelial neoplasms. Recently, DOG1 immunoreactivity has been reported in pancreatic solid pseudopapillary tumors (SPT), suggesting a role as a marker for SPT. Utilizing immunohistochemistry, we evaluated DOG1 expression in pancreatic neoplasms to determine the prevalence of staining and establish diagnostic utility. Multiple tissue microarrays (TMA) were created from cores of formalin-fixed paraffin-embedded blocks containing pancreatic adenocarcinomas (n=112), neuroendocrine tumors (n=99), serous cystadenomas (n=28), and SPT (n=14) as well as normal pancreas (n=12). Immunoreactivity for DOG1 (clone K9) was assessed for intensity (1 to 3+), percentage of tumor positivity and location. Of the 99 cases of neuroendocrine tumors, only 2 (2%) were focally positive. Patchy staining was identified in 8 cases (7%) of adenocarcinoma of 1 to 2+ intensity, involving 15% to 80% of the tumor cells and primarily seen in a membranous and luminal distribution. In contrast to a previous report, no DOG1 positivity was observed in SPT, evaluated by both TMA and full sections. The TMAs of serous cystadenomas and normal pancreas were negative for DOG1. Rarely, pancreatic islets displayed granular, cytoplasmic staining. DOG1 antibody clone K9 is not a useful marker for SPT or other primary pancreatic neoplasms. Additional studies may be helpful to evaluate differences between clones of DOG1.

  6. Laparoscopic Low Anterior Resection and Eversion Technique Combined With a Nondog Ear Anastomosis for Mid- and Distal Rectal Neoplasms

    Science.gov (United States)

    Zhuo, Changhua; Liang, Lei; Ying, Mingang; Li, Qingguo; Li, Dawei; Li, Yiwei; Peng, Junjie; Huang, Liyong; Cai, Sanjun; Li, Xinxiang

    2015-01-01

    Abstract The transanal eversion and prolapsing technique is a well-established procedure, and can ensure an adequate distal margin for patients with low rectal neoplasms. Potential leakage risks, however, are associated with bilateral dog ear formation, which results from traditional double-stapling anastomosis. The authors determined the feasibility of combining these techniques with a commercial stapling set to achieve a nondog ear (end-to-end) anastomosis for patients with mid- and distal rectal neoplasms. Patients with early-stage (c/ycT1–2N0), mid- to distal rectal neoplasms and good anal sphincter function were included in this study. Laparoscopic low anterior resection was performed with a standard total mesorectal excision technique downward to the pelvic floor as low as possible. The bowel was resected proximal to the lesion with an endoscopic linear stapler. An anvil was inserted extracorporeally into the proximal colon via an extended working pore. The distal rectum coupled with the lesion was prolapsed and everted out of the anus. The neoplasm was resected with a sufficient margin above the dentate line under direct sight. A transrectal anastomosis without dog ears was performed intracorporeally to reconstitute the continuity of the bowel. Eleven cases, 6 male and 5 female patients, were included in this study. The mean operative time was 191 (129–292) minutes. The mean blood loss was 110 (30–300) mL. The median distal margin distance from the lower edge of the lesion to the dentate line was 1.5 (0.5–2.5) cm. All the resection margins were negative. Most patients experienced uneventful postoperative recoveries. No patient had anastomotic leak. Most patients had an acceptable stool frequency after loop ileostomy closure. Our preliminary data demonstrated the safety and feasibility of achieving a sound anastomosis without risking potential anastomotic leakage because of dog ear formation. PMID:26683958

  7. Rectum neoplasms treatment advanced with radio and chemotherapy before - surgery

    International Nuclear Information System (INIS)

    Luongo Cespedes, A.; Aguiar Vitacca, S.

    1993-01-01

    In Uruguay the colorectal neoplasms has a can rate of mobility. The surgery has 13-26% local recurrence. The irradiation before surgery has demonstrated to improve the resect and the local control.The objective of this protocol it is to decrease the percentage of local relapse , using radiotherapy(RT) before surgery and concomitant chemotherapy that potencies the effect of the RT, improvement this way the therapeutic quotient (AU) [es

  8. Endoscopic Diagnosis of Leiomyosarcoma of the Esophagus, a Rare Neoplasm

    OpenAIRE

    Ravini, Mario; Torre, Massimo; Zanasi, Giulio; Vanini, Marco; Camozzi, Mario

    1998-01-01

    We report a case of leiomyosarcoma of the distal third of the esophagus in a 51-year-old woman presenting with a six-month history of severe epigastric pain, disphagia and weight loss. The diagnosis, suspected on endoscopic examination, was preoperatively acheived by biopsy and immunohistological stain. Surgical treatment was undertaken with good results. Differentiation between leiomyosarcoma and more common esophageal neoplasm may be difficult if based on radiographic and endoscopic appeara...

  9. MALIGNANT NEOPLASMS IN CHILDREN: CLINICAL MANIFESTATIONS AND DIAGNOSIS

    Directory of Open Access Journals (Sweden)

    Maxim Yu. Rykov

    2017-01-01

    Full Text Available Treatment efficacy for children with cancer depends on the diagnosis timeliness since the earlier expert care has been started, the higher likelihood there is to achieve remission. In this regard, a special role belongs to primary care physicians — district pediatricians who should timely recognize the malignant neoplasm and refer the patient to a pediatric oncologist for advice. Wherein, a limited number of primary patients and atypical course of oncological diseases are the causes of a decrease in oncological alertness. This lecture is aimed at a wide range of specialists (pediatricians, radiologists, pathologists and devoted to clinical manifestations and diagnosis of malignant neoplasms in children — hemoblastosis and solid tumours. The suggested algorithms for the examination of patients will allow to make a diagnosis faster and timely initiate expert care in specialized departments. The article is illustrated with unique pictures — images of histological specimens, MRI, and CT of patients with the most neglected cases of malignant neoplasms being the result of diagnostic errors of pediatricians. 

  10. Molecularly-Driven Doublet Therapy for Recurrent CNS Malignant Neoplasms

    Science.gov (United States)

    2018-02-20

    Anaplastic Astrocytoma; Anaplastic Ependymoma; Anaplastic Ganglioglioma; Anaplastic Meningioma; Anaplastic Oligodendroglioma; Pleomorphic Xanthoastrocytoma, Anaplastic; Atypical Teratoid/Rhabdoid Tumor; Brain Cancer; Brain Tumor; Central Nervous System Neoplasms; Choroid Plexus Carcinoma; CNS Embryonal Tumor With Rhabdoid Features; Ganglioneuroblastoma of Central Nervous System; CNS Tumor; Embryonal Tumor of CNS; Ependymoma; Glioblastoma; Glioma; Glioma, Malignant; Medulloblastoma; Medulloblastoma; Unspecified Site; Medulloepithelioma; Neuroepithelial Tumor; Neoplasms; Neoplasms, Neuroepithelial; Papillary Tumor of the Pineal Region (High-grade Only); Pediatric Brain Tumor; Pineal Parenchymal Tumor of Intermediate Differentiation (High-grade Only); Pineoblastoma; Primitive Neuroectodermal Tumor; Recurrent Medulloblastoma; Refractory Brain Tumor; Neuroblastoma. CNS; Glioblastoma, IDH-mutant; Glioblastoma, IDH-wildtype; Medulloblastoma, Group 3; Medulloblastoma, Group 4; Glioma, High Grade; Neuroepithelial Tumor, High Grade; Medulloblastoma, SHH-activated and TP53 Mutant; Medulloblastoma, SHH-activated and TP53 Wildtype; Medulloblastoma, Chromosome 9q Loss; Medulloblastoma, Non-WNT Non-SHH, NOS; Medulloblastoma, Non-WNT/Non-SHH; Medulloblastoma, PTCH1 Mutation; Medulloblastoma, WNT-activated; Ependymoma, Recurrent; Glioma, Recurrent High Grade; Glioma, Recurrent Malignant; Embryonal Tumor, NOS; Glioma, Diffuse Midline, H3K27M-mutant; Embryonal Tumor With Multilayered Rosettes (ETMR); Ependymoma, NOS, WHO Grade III; Ependymoma, NOS, WHO Grade II; Medulloblastoma, G3/G4; Ependymoma, RELA Fusion Positive

  11. Oral spindle cell neoplasms: a review of 307 cases.

    Science.gov (United States)

    Jordan, Richard C K; Regezi, Joseph A

    2003-06-01

    The infrequent exposure of pathologists to soft tissue spindle cell neoplasms coupled with overlapping histologic patterns can often make diagnosis challenging. We reviewed all nonodontogenic spindle cell neoplasms seen between 1982 and 2002 (86,162 total accessions). Diagnoses were reclassified according to current standards supplemented with immunohistochemistry. Of the 307 neoplasms reviewed (0.36% of total accessions), neural tumors were the most common benign entities, accounting for 21% of total cases. Kaposi's sarcoma was the most common malignancy, accounting for 67% of all cases. Diagnoses were revised for 57 cases. Schwannoma and neurofibroma were most commonly revised to palisaded encapsulated neuroma. There were 8 myofibromas and 1 inflammatory myofibroblastic tumor. There were no oral leiomyomas; that is, all 4 originally reported cases were reclassified as myofibroma, palisaded encapsulated neuroma, and solitary fibrous tumor. With the exception of Kaposi's sarcoma, oral soft tissue sarcomas were rare; most benign lesions were neural in origin. The relatively high prevalence of some tumors, such as myofibroma, likely reflects the use of immunohistochemistry in the diagnosis of spindle cell tumors.

  12. Blastic plasmacytoid dendritic cell neoplasm: challenges and future prospects

    Directory of Open Access Journals (Sweden)

    Trottier AM

    2017-12-01

    Full Text Available Amy M Trottier, Sonia Cerquozzi, Carolyn J Owen Division of Hematology and Hematological Malignancies, University of Calgary, Foothills Medical Centre, Calgary, AB, Canada Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN is a rare CD4+ CD56+ myeloid malignancy that is challenging to diagnose and treat. BPDCN typically presents with nonspecific cutaneous lesions with or without extra-cutaneous manifestations before progressing to leukemia. Currently, there is no standard of care for the treatment of BPDCN and various approaches have been used including acute myeloid leukemia, acute lymphoblastic leukemia, and lymphoma-based regimens with or without stem cell transplantation. Despite these treatment approaches, the prognosis of BPDCN remains poor and there is a lack of prospective data upon which to base treatment decisions. Recent work examining the mutational landscape and gene expression profiles of BPDCN has identified a number of potential therapeutic targets. One such target is CD123, the α subunit of the human interleukin-3 receptor, which is the subject of intervention studies using the novel agent SL-401. Other investigational therapies include UCART123, T-cell immunotherapy, and venetoclax. Prospective trials are needed to determine the best treatment for this uncommon and aggressive neoplasm. Keywords: BPDCN, myeloid, neoplasm, cutaneous, dendritic cell

  13. Action of temperature-sensitive mutants of myeloproliferative sarcoma virus suggests that fibroblast-transforming and hematopoietic transforming viral properties are related.

    Science.gov (United States)

    Ostertag, W; Freshney, M; Vehmeyer, K; Jasmin, C; Rutter, G

    1984-01-01

    The myeloproliferative sarcoma virus is molecularly related to the Moloney sarcoma virus (Pragnell et al., J. Virol. 38:952-957, 1981), but causes both fibroblast transformation in vitro and leukemic changes--including spleen focus formation--in adult mice. The fibroblast transforming properties of myeloproliferative sarcoma virus were used to select viral temperature-sensitive mutants at 39.5 degrees C, the nonpermissive temperature. These mutants are temperature sensitive in the maintenance of the transformed state. This was also shown by cytoskeletal changes of the infected cells at permissive and nonpermissive temperatures. Viruses released from cells maintained at both the permissive and nonpermissive temperature are temperature sensitive in fibroblast transformation functions. All temperature-sensitive mutants show only a low reversion rate to wild-type transforming function. The myeloproliferative sarcoma virus temperature-sensitive mutants are inefficient in causing leukemic transformation (spleen enlargement, focus formation) in mice at the normal temperature. A method to maintain a low body temperature (33 to 34 degrees C) in mice is described. One temperature-sensitive mutant was checked at low body temperature and did not induce leukemia. These data thus indicate that the same or related viral functions are responsible for hematopoietic and fibroblast transformation.

  14. Tumor taxonomy for the developmental lineage classification of neoplasms

    International Nuclear Information System (INIS)

    Berman, Jules J

    2004-01-01

    The new 'Developmental lineage classification of neoplasms' was described in a prior publication. The classification is simple (the entire hierarchy is described with just 39 classifiers), comprehensive (providing a place for every tumor of man), and consistent with recent attempts to characterize tumors by cytogenetic and molecular features. A taxonomy is a list of the instances that populate a classification. The taxonomy of neoplasia attempts to list every known term for every known tumor of man. The taxonomy provides each concept with a unique code and groups synonymous terms under the same concept. A Perl script validated successive drafts of the taxonomy ensuring that: 1) each term occurs only once in the taxonomy; 2) each term occurs in only one tumor class; 3) each concept code occurs in one and only one hierarchical position in the classification; and 4) the file containing the classification and taxonomy is a well-formed XML (eXtensible Markup Language) document. The taxonomy currently contains 122,632 different terms encompassing 5,376 neoplasm concepts. Each concept has, on average, 23 synonyms. The taxonomy populates 'The developmental lineage classification of neoplasms,' and is available as an XML file, currently 9+ Megabytes in length. A representation of the classification/taxonomy listing each term followed by its code, followed by its full ancestry, is available as a flat-file, 19+ Megabytes in length. The taxonomy is the largest nomenclature of neoplasms, with more than twice the number of neoplasm names found in other medical nomenclatures, including the 2004 version of the Unified Medical Language System, the Systematized Nomenclature of Medicine Clinical Terminology, the National Cancer Institute's Thesaurus, and the International Classification of Diseases Oncolology version. This manuscript describes a comprehensive taxonomy of neoplasia that collects synonymous terms under a unique code number and assigns each

  15. Environmental exposure to cooking oil fumes and cervical intraepithelial neoplasm

    International Nuclear Information System (INIS)

    Wu, M.-T.; Lee, L.-H.; Ho, C.-K.; Wu, S.-C.; Lin, L.-Y.; Cheng, B.-H.; Liu, C.-L.; Yang, C.-Y.; Tsai, H.-T.; Wu, T.-N.

    2004-01-01

    The fumes from cooking oil, similar to cigarette smoke, contain numerous carcinogens such as polycyclic aromatic hydrocarbons, aromatic amines, nitro-polycyclic aromatic hydrocarbons, etc. In this study, we examined the association between exposure to cooking oil fumes and the risk of cervical intraepithelial neoplasm. The study population in this nested case-control study consisted of women above the age of 19 years living in Chia-Yi County, located in the southwestern Taiwan, who had received pap smear screening between October, 1999, and December, 2000 (n=32,466). The potential cases were women having lesions greater than cervical intraepithelium neoplasm II (≥CIN2) reconfirmed by cervical biopsy (n=116). The potential controls (case: control=1:2) were age-matched (±2 years) and residence-matched women who had normal pap smears within 6 months of the cases. In total, 100 cases and 197 controls were completely interviewed by public health nurses about cooking methods, ventilation, and other potential risk factors. Women who cooked at home in a kitchen (n=269) without the presence of a fume extractor at least once a week between the ages of 20 and 40 had a 2.29 times higher risk [95% confidence interval (CI)=1.08-4.87] of developing cervical intraepithelial neoplasm than those who did not cook once a week in such a kitchen during the same age span, after adjusting for other potential confounders. This finding was further strengthened by the finding that women who did not use the fume extractors had a 2.47 times higher risk (95% CI=1.15-5.32) of developing cervical intraepithelial neoplasm than women who cooked in kitchens with fume extractors that were always switched on while cooking. We also found a joint protective effect of fume extractor use among women older than 40 years (n=202) if they used the extractors during both age spans of their lives, ages 20-40 and >40 years. Comparing our findings on women more than 40 years old who used fume extractors during

  16. Patients with a resected pancreatic mucinous cystic neoplasm have a better prognosis than patients with an intraductal papillary mucinous neoplasm : A large single institution series

    NARCIS (Netherlands)

    Griffin, James F; Page, Andrew J; Samaha, Georges J; Christopher, Adrienne; Bhaijee, Feriyl; Pezhouh, Maryam K; Peters, Niek A.; Hruban, Ralph H.; He, Jin; Makary, Martin A; Lennon, Anne Marie; Cameron, John L; Wolfgang, Christopher L; Weiss, Matthew J

    2017-01-01

    BACKGROUND/OBJECTIVES: Mucinous cystic neoplasms (MCNs) are rare pancreas tumors distinguished from intraductal papillary mucinous neoplasms (IPMNs) by the presence of ovarian-type stroma. Historical outcomes for MCNs vary due to previously ambiguous diagnostic criteria resulting in confusion with

  17. Patients With Nonpolypoid (Flat and Depressed) Colorectal Neoplasms at Increased Risk for Advanced Neoplasias, Compared With Patients With Polypoid Neoplasms.

    Science.gov (United States)

    McGill, Sarah K; Soetikno, Roy; Rouse, Robert V; Lai, Hobart; Kaltenbach, Tonya

    2017-02-01

    Nonpolypoid colorectal neoplasms (NP-CRNs) are more likely to contain high-grade dysplasia or early-stage cancer than polypoid neoplasms. We aimed to determine the long-term outcomes of patients with at least 1 NP-CRN. We performed a longitudinal cohort study of 4454 patients at a Veterans' Affairs hospital who underwent colonoscopy from 2000 through 2005; 341 were found to have 1 or more NP-CRNs and were matched (3:1) with patients found to have 1 or more polypoid neoplasms (controls, n = 1025). We collected and analyzed data on baseline colonoscopy findings and first follow-up colonoscopy results through August 2014. We calculated the incidence of advanced neoplasia at first follow-up colonoscopy, as defined by the presence of ≥1 tubular or sessile serrated adenomas ≥10 mm in diameter, tubulovillous adenoma, high-grade dysplasia, or invasive cancer. A significantly higher proportion of patients with 1 or more NP-CRNs (16.0%) were found to have advanced neoplasia at their first follow-up colonoscopy than controls (8.6%); the adjusted risk ratio was 1.6 (95% confidence interval, 1.05-2.6; P = .03). A significantly higher proportion of patients with 1 or more NP-CRNs were found to have additional NP-CRNs at the follow-up colonoscopy (17%) than controls (7%; relative risk, 2.3; 95% confidence interval, 1.5-3.5; P < .001). Similar proportions of patients in each group developed cancers after colonoscopy. In a longitudinal cohort study, we found that patients with NP-CRN were more likely to develop additional NP-CRNs and to have advanced neoplasms at their first follow-up colonoscopy than patients with only polypoid neoplasms. However, patients with NP-CRN were not more likely to develop cancers after colonoscopy when surveillance guidelines were followed. Larger studies are needed to determine risk of colorectal cancer in patients with NP-CRN. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

  18. Multiple neoplasms among cervical cancer patients in the material of the lower Silesian cancer registry.

    Science.gov (United States)

    Izmajłowicz, Barbara; Kornafel, Jan; Błaszczyk, Jerzy

    2014-01-01

    According to the definition by the International Agency for Research on Cancer (IARC), primary multiple neoplasms are two or more neoplasms of different histopathological build in one organ, or two or more tumors occurring in one patient, regardless of the time of their occurrence (synchronic - up to 6 months, metachronous - after 6 months), coming from an organ or a tissue and not being an infiltration from another neoplasm, a relapse or a metastasis. It was the aim of the study to analyze the frequency of the occurrence of multiple neoplasms among patients suffering from uterine cervix cancer, with a special interest in coexistent neoplasms, the time of their occurrence and total 5-year survivals. The data from the Lower Silesian Cancer Registry concerning the years 1984-2009 formed the material of the present study. 5.3% of all cervix neoplasms occurred as multiple cancers. Cervix neoplasms were 13.4% of multiple neoplasms. On average, cervical cancer occurred as a subsequent cancer in 6 patients yearly (60.7% of the occurrences of cervical cancer were in the period of 5 years following treatment for the first neoplasm). 5-year survival in patients suffering from primarily multiple cervix neoplasms constituted 57% and was convergent with the results for all patients suffering from cervical cancer. Cervical cancer as the first neoplasm occurred in 287 patients, on average in 11 patients annually. In the period of the first 5 years after the treatment of cervical cancer, there were 42.8% occurrences of other cancers. Cervical neoplasms most frequently coexisted with cancers of the breast, lung and large intestine. The frequency of the occurrence of multiple neoplasm among cervical cancer patients is increasing. Most frequently they coexist with other tobacco-related neoplasms, those related to HPV infections and with secondary post-radiation neoplasms. These facts should be taken into consideration during post-treatment observation and when directing diagnostic

  19. [Epidemiological and clinical characteristics of 705 salivary glands neoplasms].

    Science.gov (United States)

    Ziółkowska, Magdalena; Bień, Stanisław; Okła, Sławomir; Zyłka, Stanisław

    2013-01-01

    The epidemiological data concerning the rare group of tumors derived from salivary glands recorded in the National Cancer Registry is insufficient because it records only malignant salivary glands tumors. The epidemiological and clinical characteristics of 699 patients with salivary glands tumors (n=705). The data were retrieved from ENT Department District Hospital in Kielce (01.09.1989-28.02.2001) and from Department of Otolaryngology Head and Neck Surgery Holy Cross Cancer Centre in Kielce (01.03.2001-31.12.2008). In the analyzed group of 699 patients with 705 salivary glands tumors women predominated, consisting 54,2% of all group. The average age in group of malignant tumors was higher than in nonmalignant group. The risk of malignant neoplasms development increased with patient age. In the analyzed group of 705 salivary glands tumors the nonmalignant neoplasms dominated-78,3%. Out of all cases, 547 (77,6%) were localized in the parotid gland, 80 (11,3%) in submandibular gland and 78 (11,1%) in minor salivary glands. Nonmalignant tumors were more frequent in the parotid gland (82,8%) and submandibular gland (71,3%), whereas in minor salivary glands nonmalignant and malignant neoplasms the occurrence was nearly the same. In general-the smaller the salivary gland, the risk of development malignant tumors was higher. In group of nonmalignant salivary gland tumors two histopathological types dominated - pleomorphic adenoma and Warthin's tumor, which comprised 91,8% of the whole group. In the group of 153 malignant salivary gland tumors the most common histopathology were - adenoid cystic carcinoma, mucoepidermoid carcinoma and adenocarcinoma. In the analyzed period of 20 years' time, the incidence of salivary glands tumors increased with high siginificance, both for nonmalignant, as well malignant tumors. Copyright © 2013 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.

  20. Immunohistochemical study of p16 INK4A and survivin expressions in cervical squamous neoplasm

    Directory of Open Access Journals (Sweden)

    Tan Geok

    2010-01-01

    Full Text Available Introduction:Cervical cancer is the second most common cancer affecting Malaysian women. Despite the implementation of pap smear screening, many women are still diagnosed only in the advanced stage of cervical cancer. This could partly be due to failure of detection of its precursor lesions; hence the need to search for novel biomarkers to assist in the screening and diagnosis of cervical neoplasia. This study aims to determine the expression of p16INK4A and survivin as possible predictive biomarkers in cervical squamous neoplasm. Material and Methods: This is a retrospective study on 201 cases of cervical neoplasm comprising of 129 cervical intraepithelial neoplasia (CIN and 72 squamous cell carcinoma (SCC. All samples were evaluated by two independent observers using p16INK4A and survivin monoclonal antibodies. The p16 INK4A expression was graded as negative, focal and diffuse positivity. The intensity for survivin expression was graded as weak, moderate and intense. Results: It is seen that p16 INK4A expression in CIN 1, CIN 2 and CIN 3 were 25.4%, 42.9% and 95.9% respectively. Majority of SCC (98.6% showed p16 INK4A expression. Survivin expressions in CIN 1, CIN 2, CIN 3 and SCC were 56.7%, 33.4%, 87.5% and 98.6%. There was a linear relationship between increasing grade of CIN and p16 INK4A expressions. Conclusion: Our study showed that p16 INK4A expressions correlate well with the increasing grade of CIN. Although survivin does not correlate well to the increasing grade of CIN, it could be useful in differentiating CIN 3 from SCC.

  1. Second Malignant Neoplasms After Treatment of Childhood Acute Lymphoblastic Leukemia

    DEFF Research Database (Denmark)

    Schmiegelow, K.; Levinsen, Mette Frandsen; Attarbaschi, Andishe

    2013-01-01

    PURPOSE: Second malignant neoplasms (SMNs) after diagnosis of childhood acute lymphoblastic leukemia (ALL) are rare events. PATIENTS AND METHODS: We analyzed data on risk factors and outcomes of 642 children with SMNs occurring after treatment for ALL from 18 collaborative study groups between 1980...... and 2007. RESULTS: Acute myeloid leukemia (AML; n = 186), myelodysplastic syndrome (MDS; n = 69), and nonmeningioma brain tumor (n = 116) were the most common types of SMNs and had the poorest outcome (5-year survival rate, 18.1% ± 2.9%, 31.1% ± 6.2%, and 18.3% ± 3.8%, respectively). Five-year survival...

  2. Actinomycosis of the parotid masquerading as malignant neoplasm

    International Nuclear Information System (INIS)

    Varghese, Bipin T; Sebastian, Paul; Ramachandran, K; Pandey, Manoj

    2004-01-01

    Primary actinomycosis of the parotid gland is of rare occurrence and can mimic a malignant neoplasm both clinically as well as radiologically. We present here a case of primary actinomycosis of the parotid gland presenting with a parotid mass lesion with erosion of skull bones. Clinical presentation of cervico-facial actinomycosis is characterized by the presence of a suppurative or indurative mass with discharging sinuses. The lesion demonstrates characteristic features on fine needle aspiration cytology and histology, however at times the findings are equivocal

  3. Actinomycosis of the parotid masquerading as malignant neoplasm.

    Directory of Open Access Journals (Sweden)

    Ramachandran K

    2004-03-01

    Full Text Available Abstract Background Primary actinomycosis of the parotid gland is of rare occurrence and can mimic a malignant neoplasm both clinically as well as radiologically. Case presentation We present here a case of primary actinomycosis of the parotid gland presenting with a parotid mass lesion with erosion of skull bones. Conclusions Clinical presentation of cervico-facial actinomycosis is characterized by the presence of a suppurative or indurative mass with discharging sinuses. The lesion demonstrates characteristic features on fine needle aspiration cytology and histology, however at times the findings are equivocal.

  4. Branchial osteogenetic neoplasm in barbel Barbus barbus plebejus.

    Science.gov (United States)

    Manera, M; Biavati, S

    1999-09-14

    A branchial osteogenetic neoplasm affecting a barbel Barbus barbus plebejus (Valenciennes, 1829) is described. The osteoblasts' pleomorphism, the lack of a well-developed and complete separation, the presence of eccentric, terminal proliferative edges infiltrating the lining tissues and the abundant tumour matrix suggest a histopathological diagnosis of a 'productive osteoblastic osteosarcoma'. The occurrence of eosinophilic granule cells (EGCs) scattered among neoplastic tissue is discussed in relation to the neoplastic growth and the inflammatory reaction, with reference to recent discoveries in mammalian mast cell biology.

  5. Inflammatory demyelinating pseudotumor with hemorrhage masquerading high grade cerebral neoplasm

    Directory of Open Access Journals (Sweden)

    Amit Agrawal

    2015-03-01

    Full Text Available Demyelinating pseudotumors are rare, benign, solitary intracranial space occupying lesions which masquerade cerebral neoplasms. Contrast MRI shows open ring enhancement which is fairly specific for this entity. Advanced MRI techniques like MR spectroscopy and magnetizing transfer techniques can help differentiating these lesions. NAA/Cr ratio is significantly elevated in central regions of demyelinating pseudotumors than in gliomas and other lesions. Presence of abundant foamy macrophages, lymphoid inflammatory infiltrates around blood vessels, sheets of gemistocytic astrocytes with well-developed processes, well defined border of the lesion absence of neovascularity and necrosis should help us diagnose demyelinating pseudotumor fairly confidently on histopathology.

  6. Distinct molecular features of different macroscopic subtypes of colorectal neoplasms.

    Directory of Open Access Journals (Sweden)

    Kenichi Konda

    Full Text Available Colorectal adenoma develops into cancer with the accumulation of genetic and epigenetic changes. We studied the underlying molecular and clinicopathological features to better understand the heterogeneity of colorectal neoplasms (CRNs.We evaluated both genetic (mutations of KRAS, BRAF, TP53, and PIK3CA, and microsatellite instability [MSI] and epigenetic (methylation status of nine genes or sequences, including the CpG island methylator phenotype [CIMP] markers alterations in 158 CRNs including 56 polypoid neoplasms (PNs, 25 granular type laterally spreading tumors (LST-Gs, 48 non-granular type LSTs (LST-NGs, 19 depressed neoplasms (DNs and 10 small flat-elevated neoplasms (S-FNs on the basis of macroscopic appearance.S-FNs showed few molecular changes except SFRP1 methylation. Significant differences in the frequency of KRAS mutations were observed among subtypes (68% for LST-Gs, 36% for PNs, 16% for DNs and 6% for LST-NGs (P<0.001. By contrast, the frequency of TP53 mutation was higher in DNs than PNs or LST-Gs (32% vs. 5% or 0%, respectively (P<0.007. We also observed significant differences in the frequency of CIMP between LST-Gs and LST-NGs or PNs (32% vs. 6% or 5%, respectively (P<0.005. Moreover, the methylation level of LINE-1 was significantly lower in DNs or LST-Gs than in PNs (58.3% or 60.5% vs. 63.2%, P<0.05. PIK3CA mutations were detected only in LSTs. Finally, multivariate analyses showed that macroscopic morphologies were significantly associated with an increased risk of molecular changes (PN or LST-G for KRAS mutation, odds ratio [OR] 9.11; LST-NG or DN for TP53 mutation, OR 5.30; LST-G for PIK3CA mutation, OR 26.53; LST-G or DN for LINE-1 hypomethylation, OR 3.41.We demonstrated that CRNs could be classified into five macroscopic subtypes according to clinicopathological and molecular differences, suggesting that different mechanisms are involved in the pathogenesis of colorectal tumorigenesis.

  7. Radiological findings of two neoplasms with perivascular epithelioid cell differentiation

    Directory of Open Access Journals (Sweden)

    Timothy Diestelkamp, BS

    2017-12-01

    Full Text Available Perivascular epithelioid cell tumors (PEComas constitute a rare subset of mesenchymal neoplasms classified by the World Health Organization in 2002. We present two cases of PEComas; the first is a cervical PEComa in a 35-year-old woman with no known past medical history who presented with a palpable pelvic mass; the second is an adnexal PEComa in a 39-year-old woman with a history of colitis who presented with abdominal pain and diarrhea. The rarity of these tumors has led to little information about imaging characteristics which we hope these two cases will help expand.

  8. Distinct Molecular Features of Different Macroscopic Subtypes of Colorectal Neoplasms

    Science.gov (United States)

    Konda, Kenichi; Konishi, Kazuo; Yamochi, Toshiko; Ito, Yoichi M.; Nozawa, Hisako; Tojo, Masayuki; Shinmura, Kensuke; Kogo, Mari; Katagiri, Atsushi; Kubota, Yutaro; Muramoto, Takashi; Yano, Yuichiro; Kobayashi, Yoshiya; Kihara, Toshihiro; Tagawa, Teppei; Makino, Reiko; Takimoto, Masafumi; Imawari, Michio; Yoshida, Hitoshi

    2014-01-01

    Background Colorectal adenoma develops into cancer with the accumulation of genetic and epigenetic changes. We studied the underlying molecular and clinicopathological features to better understand the heterogeneity of colorectal neoplasms (CRNs). Methods We evaluated both genetic (mutations of KRAS, BRAF, TP53, and PIK3CA, and microsatellite instability [MSI]) and epigenetic (methylation status of nine genes or sequences, including the CpG island methylator phenotype [CIMP] markers) alterations in 158 CRNs including 56 polypoid neoplasms (PNs), 25 granular type laterally spreading tumors (LST-Gs), 48 non-granular type LSTs (LST-NGs), 19 depressed neoplasms (DNs) and 10 small flat-elevated neoplasms (S-FNs) on the basis of macroscopic appearance. Results S-FNs showed few molecular changes except SFRP1 methylation. Significant differences in the frequency of KRAS mutations were observed among subtypes (68% for LST-Gs, 36% for PNs, 16% for DNs and 6% for LST-NGs) (P<0.001). By contrast, the frequency of TP53 mutation was higher in DNs than PNs or LST-Gs (32% vs. 5% or 0%, respectively) (P<0.007). We also observed significant differences in the frequency of CIMP between LST-Gs and LST-NGs or PNs (32% vs. 6% or 5%, respectively) (P<0.005). Moreover, the methylation level of LINE-1 was significantly lower in DNs or LST-Gs than in PNs (58.3% or 60.5% vs. 63.2%, P<0.05). PIK3CA mutations were detected only in LSTs. Finally, multivariate analyses showed that macroscopic morphologies were significantly associated with an increased risk of molecular changes (PN or LST-G for KRAS mutation, odds ratio [OR] 9.11; LST-NG or DN for TP53 mutation, OR 5.30; LST-G for PIK3CA mutation, OR 26.53; LST-G or DN for LINE-1 hypomethylation, OR 3.41). Conclusion We demonstrated that CRNs could be classified into five macroscopic subtypes according to clinicopathological and molecular differences, suggesting that different mechanisms are involved in the pathogenesis of colorectal

  9. Endoscopic diagnosis of leiomyosarcoma of the esophagus, a rare neoplasm.

    Science.gov (United States)

    Ravini, M; Torre, M; Zanasi, G; Vanini, M; Camozzi, M

    1998-01-01

    We report a case of leiomyosarcoma of the distal third of the esophagus in a 51-year-old woman presenting with a six-month history of severe epigastric pain, disphagia and weight loss. The diagnosis, suspected on endoscopic examination, was preoperatively acheived by biopsy and immunohistological stain. Surgical treatment was undertaken with good results. Differentiation between leiomyosarcoma and more common esophageal neoplasm may be difficult if based on radiographic and endoscopic appearance. Preoperative histological confirmation is therefore mandatory to schedule a wide surgical excision.

  10. Differential labeling of platelet alpha 2 adrenoceptors by 3H dihydroergocryptine and 3H yohimbine in patients with myeloproliferative disorders

    International Nuclear Information System (INIS)

    Swart, S.S.; Wood, J.K.; Barnett, D.B.

    1985-01-01

    Platelet alpha adrenoceptor status was examined using the radioligands 3 H-yohimbine ( 3 H-YOH) and 3 H-dihydroergocryptine ( 3 H-DHE) in 14 patients with myeloproliferative disorder (MPD) and 10 normal controls. Platelets from normal controls and MPD patients sensitive to adrenaline induced aggregation exhibited approximately 50% more binding sites identified by 3 H-DHE than 3 H-YOH, whereas MPD platelets insensitive to adrenaline showed selective loss of these extra 3 H-DHE sites. In functional studies after 30 minutes preincubation with the unlabelled antagonists, DHE was more potent than YOH at inhibiting adrenaline induced aggregation in normal platelets. In addition, the affinity constant for DHE was virtually identical in binding and functional experiments, whereas for YOH the affinity constant for binding was approximately 10 fold more potent than that for aggregation. These results suggest that the alpha adrenoceptor binding site on human platelets labelled by 3 H-DHE may be of more functional relevance than that labelled by 3 H-YOH alone

  11. Minimal residual disease or cure in MPNs?

    DEFF Research Database (Denmark)

    Bjørn, Mads Emil; Hasselbalch, Hans Carl

    2017-01-01

    INTRODUCTION: The therapeutic landscape of the Philadelphia-negative myeloproliferative neoplasms (MPNs) is markedly changing consequent to the development of JAK-inhibitors and the use of ruxolitinib (RUX) in patients with myelofibrosis (MF) and patients with polycythemia vera (PV) who develop...

  12. The JAK2 46/1 haplotype in Budd-Chiari syndrome and portal vein thrombosis

    NARCIS (Netherlands)

    J.H. Smalberg (Jasper); S. Darwish Murad (Sarwa); E.M. Koehler (Edith); A. Plessier (Aurelie); S. Seijo (Susana); J. Trebicka (Jonel); M. Primignani (Massimo); M.P.M. de Maat (Moniek); J.C. García-Pagán (Juan Carlos); D.C. Valla (Dominique Charle); H.L.A. Janssen (Harry); F.W.G. Leebeek (Frank)

    2011-01-01

    textabstractThe germline JAK2 46/1 haplotype has been associated with the development of JAK2V617F-positive as well as JAK2V617F-negative myeloproliferative neoplasms (MPNs). In this study we examined the role of the 46/1 haplotype in the etiology and clinical presentation of patients with

  13. No Evidence for JAK2(V617F) Mutation in Monoclonal B Cells in 2 Patients with Polycythaemia Vera and Concurrent Monoclonal B Cell Disorder

    NARCIS (Netherlands)

    Stijnis, C.; Kroes, W. G. M.; Balkassmi, S.; Marijt, E. W. A.; van Rossum, A. P.; Bakker, E.; Vlasveld, L. T.

    2012-01-01

    Occurrence of Philadelphia chromosome-negative myeloproliferative neoplasms (Ph- MPN) and lymphoproliferative disorders, like B cell chronic lymphocytic leukaemia (B-CLL), in the same patient is rare. JAK2(V617F) mutation was recently introduced as a powerful diagnostic tool for Ph- MPN. JAK2(V617F)

  14. Altered glutamyl-aminopeptidase activity and expression in renal neoplasms

    International Nuclear Information System (INIS)

    Blanco, Lorena; Larrinaga, Gorka; Sanz, Begoña; Perez, Itxaro; Sánchez, Clara E; Cándenas, M Luz; Pinto, Francisco M; Gil, Javier; Casis, Luis; López, José I

    2014-01-01

    Advances in the knowledge of renal neoplasms have demonstrated the implication of several proteases in their genesis, growth and dissemination. Glutamyl-aminopeptidase (GAP) (EC. 3.4.11.7) is a zinc metallopeptidase with angiotensinase activity highly expressed in kidney tissues and its expression and activity have been associated wtih tumour development. In this prospective study, GAP spectrofluorometric activity and immunohistochemical expression were analysed in clear-cell (CCRCC), papillary (PRCC) and chromophobe (ChRCC) renal cell carcinomas, and in renal oncocytoma (RO). Data obtained in tumour tissue were compared with those from the surrounding uninvolved kidney tissue. In CCRCC, classic pathological parameters such as grade, stage and tumour size were stratified following GAP data and analyzed for 5-year survival. GAP activity in both the membrane-bound and soluble fractions was sharply decreased and its immunohistochemical expression showed mild staining in the four histological types of renal tumours. Soluble and membrane-bound GAP activities correlated with tumour grade and size in CCRCCs. This study suggests a role for GAP in the neoplastic development of renal tumours and provides additional data for considering the activity and expression of this enzyme of interest in the diagnosis and prognosis of renal neoplasms

  15. Dermal Squamomelanocytic Tumor: Neoplasm of Uncertain Biological Potential

    Directory of Open Access Journals (Sweden)

    Mirsad Dorić

    2008-05-01

    Full Text Available We report a case of exceedingly rare cutaneous neoplasm with histological features of malignancy and uncertain biological potential. The nodular, darkly pigmented facial tumor with central exulceration, size 12x10x7 mm, of the skin 61-year-old man preauricular left was completely exised.Histologically tumor consists of atypical squamous cells, which express signs of moderate to significant pleomorphism, mitotically active, with foci forming of parakeratotic horn cysts (“pearls”. Characteristically tumor also consists of large number of atypical melanocytes with multifocal pattern, inserted between atypical squamous cells, and which contain large amount of dark brown pigment melanin. Immunohistochemically, squamous cells stain positively with keratin (CK116, melanocytes were stained with S -100 protein, HMB 45, and vimentin, but failed to stain with CK 116.To our knowledge this is the sixth reported case in world literature. The follow-up time of four years no evidence of recurrence or metastasis, similar all reported cases, but it is too short period in estimation to guarantee a benign course. However, it appears that this group of neoplasm may have different prognosis from pure squamous carcinoma or malignant melanoma.

  16. Endoscopic approach for a laryngeal neoplasm in a dog

    Directory of Open Access Journals (Sweden)

    Pedro Paulo Maia Teixeira

    2015-01-01

    Full Text Available Laryngeal and tracheal tumors are rare in pets; some piece of information on their disease behavior, therapy and evolution are limited. Neoplasms in this area are a diagnostic challenge. In many cases, they can be biopsied and excised using endoscopic instruments, but there is no report of this in canines. The goal of this study is to report a successful case of a laryngeal neoplasm removal through endoscopy. A head and neck radiogram revealed a mass in the laryngeal lumen protruding into the trachea. The patient then underwent an endoscopy to confirm the radiographic diagnosis and to surgically remove the tumor. The histopathological diagnosis was poorly differentiated carcinoma. The most appropriate treatment for laryngeal tumors is the resection of the submucosa or a partial laryngectomy however, partial and total laryngectomies are associated with many postoperative complications. In contrast, the endoscopic approach allows for highly magnified visualization of the lesion in situ, which facilitates the surgical removal of the mass through videosurgery. With little manipulation of the affected area, the chances of postoperative complications are reduced, leading to a more rapid recovery.

  17. [Cytopathological alterations and risk factors for uterine cervical neoplasm].

    Science.gov (United States)

    de Melo, Simone Cristina Castanho Sabaini; Prates, Letícia; Carvalho, Maria Dalva de Barros; Marcon, Sonia Silva; Pelloso, Sandra Marisa

    2009-12-01

    The aim of the present study was to verify the occurrence of citopathological alterations and risk factors of Uterine Cervical Neoplasm in women attended by SUS--the Public Healthcare System--in a district situated in the North of Paraná State, Brazil from 2001 to 2006. It was a descriptive transversal observational study. The data collection consisted in collection of test results from medical records and interviews. It was achieved 6.356 tests and, 1.02% (65) of the women examined presented alterations. From the tests made 4.869 (70,8%) were from women aged between 25 and 59 years. And 38,5% of the tests presented Cervical Intraepithelial Neoplasm (CIN) I, 32,3% CIN II, 18,5% CIN I and Human Papiloma Virus (HPV). It was interviewed 25 women from the total sample. Most of them presented a risk factor as: smoking habits, sexually transmitted diseases, use of hormonal contraceptive, number of sexual partners, early sexual intercourse. This study concludes that is required educative and more effective actions in order to reduce the alterations, meanly among teenagers.

  18. Classifying the evolutionary and ecological features of neoplasms

    Science.gov (United States)

    Maley, Carlo C.; Aktipis, Athena; Graham, Trevor A.; Sottoriva, Andrea; Boddy, Amy M.; Janiszewska, Michalina; Silva, Ariosto S.; Gerlinger, Marco; Yuan, Yinyin; Pienta, Kenneth J.; Anderson, Karen S.; Gatenby, Robert; Swanton, Charles; Posada, David; Wu, Chung-I; Schiffman, Joshua D.; Hwang, E. Shelley; Polyak, Kornelia; Anderson, Alexander R. A.; Brown, Joel S.; Greaves, Mel; Shibata, Darryl

    2018-01-01

    Neoplasms change over time through a process of cell-level evolution, driven by genetic and epigenetic alterations. However, the ecology of the microenvironment of a neoplastic cell determines which changes provide adaptive benefits. There is widespread recognition of the importance of these evolutionary and ecological processes in cancer, but to date, no system has been proposed for drawing clinically relevant distinctions between how different tumours are evolving. On the basis of a consensus conference of experts in the fields of cancer evolution and cancer ecology, we propose a framework for classifying tumours that is based on four relevant components. These are the diversity of neoplastic cells (intratumoural heterogeneity) and changes over time in that diversity, which make up an evolutionary index (Evo-index), as well as the hazards to neoplastic cell survival and the resources available to neoplastic cells, which make up an ecological index (Eco-index). We review evidence demonstrating the importance of each of these factors and describe multiple methods that can be used to measure them. Development of this classification system holds promise for enabling clinicians to personalize optimal interventions based on the evolvability of the patient’s tumour. The Evo- and Eco-indices provide a common lexicon for communicating about how neoplasms change in response to interventions, with potential implications for clinical trials, personalized medicine and basic cancer research. PMID:28912577

  19. The Profile of Heparanase Expression Distinguishes Differentiated Thyroid Carcinoma from Benign Neoplasms.

    Science.gov (United States)

    Matos, Leandro Luongo; Suarez, Eloah Rabello; Theodoro, Thérèse Rachell; Trufelli, Damila Cristina; Melo, Carina Mucciolo; Garcia, Larissa Ferraz; Oliveira, Olivia Capela Grimaldi; Matos, Maria Graciela Luongo; Kanda, Jossi Ledo; Nader, Helena Bonciani; Martins, João Roberto Maciel; Pinhal, Maria Aparecida Silva

    2015-01-01

    The search for a specific marker that could help to distinguish between differentiated thyroid carcinoma and benign lesions remains elusive in clinical practice. Heparanase (HPSE) is an endo-beta-glucoronidase implicated in the process of tumor invasion, and the heparanase-2 (HPSE2) modulates HPSE activity. The aim of this study was to evaluate the role of heparanases in the development and differential diagnosis of follicular pattern thyroid lesions. HPSE and HPSE2 expression by qRT-PCR, immunohistochemistry evaluation, western blot analysis and HPSE enzymatic activity were evaluated. The expression of heparanases by qRT-PCR showed an increase of HPSE2 in thyroid carcinoma (P = 0.001). HPSE activity was found to be higher in the malignant neoplasms than in the benign tumors (P<0.0001). On Western blot analysis, HPSE2 isoforms were detected only in malignant tumors. The immunohistochemical assay allowed us to establish a distinct pattern for malignant and benign tumors. Carcinomas showed a typical combination of positive labeling for neoplastic cells and negative immunostaining in colloid, when compared to benign tumors (P<0.0001). The proposed diagnostic test presents sensitivity and negative predictive value of around 100%, showing itself to be an accurate test for distinguishing between malignant and benign lesions. This study shows, for the first time, a distinct profile of HPSE expression in thyroid carcinoma suggesting its role in carcinogenesis.

  20. A mutação JAK2 V617F e as síndromes mieloproliferativas JAK2 V617F mutation and the myeloproliferative disorders

    Directory of Open Access Journals (Sweden)

    Bárbara C. R. Monte-Mór

    2008-01-01

    Full Text Available Síndromes mieloproliferativas (SMPs são doenças hematopoéticas de origem clonal que apresentam amplificação de uma ou mais linhagens mielóides. Policitemia vera (PV, trombocitemia essencial (TE, mielofibrose idiopática (MF e leucemia mielóide crônica (LMC são consideradas SMPs clássicas e apresentam características clínicas e biológicas comuns. Ao contrário de LMC, cuja etiologia está relacionada à proteína constitutivamente ativa Bcr-Abl, o mecanismo molecular de PV, TE e MF permaneceu por muito tempo desconhecido. Esta revisão se foca na recente descoberta da mutação JAK2 V617F em pacientes com PV, TE e MF, sua relação com o fenótipo mieloproliferativo e implicações na abordagem clínica de pacientes.Myeloproliferative disorders are clonal hematopoietic diseases that are characterized by the amplification of one or more myeloid lineages. Polycythemia vera, essential thrombocythemia, idiopathic myelofibrosis and chronic myeloid leukemia are considered classic myeloproliferative disorders and share common clinical and biological features. While the genetic basis of chronic myeloid leukemia is shown to be the constitutive active protein BCR-ABL, the main molecular lesions in polycythemia vera, essential thrombocythemia and idiopathic myelofibrosis remain unknown. This review focuses on the recent discovery of the JAK2 V617F mutation, its relationship to the myeloproliferative phenotype and implications in the clinical approach of patients.

  1. Helicobacter pylori-related chronic gastritis as a risk factor for colonic neoplasms

    Science.gov (United States)

    Inoue, Izumi; Kato, Jun; Tamai, Hideyuki; Iguchi, Mikitaka; Maekita, Takao; Yoshimura, Noriko; Ichinose, Masao

    2014-01-01

    To summarize the current views and insights on associations between Helicobacter pylori (H. pylori)-related chronic gastritis and colorectal neoplasm, we reviewed recent studies to clarify whether H. pylori infection/H. pylori-related chronic gastritis is associated with an elevated risk of colorectal neoplasm. Recent studies based on large databases with careful control for confounding variables have clearly demonstrated an increased risk of colorectal neoplasm associated with H. pylori infection. The correlation between H. pylori-related chronic atrophic gastritis (CAG) and colorectal neoplasm has only been examined in a limited number of studies. A recent large study using a national histopathological database, and our study based on the stage of H. pylori-related chronic gastritis as determined by serum levels of H. pylori antibody titer and pepsinogen, indicated that H. pylori-related CAG confers an increased risk of colorectal neoplasm, and more extensive atrophic gastritis will probably be associated with even higher risk of neoplasm. In addition, our study suggested that the activity of H. pylori-related chronic gastritis is correlated with colorectal neoplasm risk. H. pylori-related chronic gastritis could be involved in an increased risk of colorectal neoplasm that appears to be enhanced by the progression of gastric atrophy and the presence of active inflammation. PMID:24587623

  2. Helicobacter pylori-related chronic gastritis as a risk factor for colonic neoplasms.

    Science.gov (United States)

    Inoue, Izumi; Kato, Jun; Tamai, Hideyuki; Iguchi, Mikitaka; Maekita, Takao; Yoshimura, Noriko; Ichinose, Masao

    2014-02-14

    To summarize the current views and insights on associations between Helicobacter pylori (H. pylori)-related chronic gastritis and colorectal neoplasm, we reviewed recent studies to clarify whether H. pylori infection/H. pylori-related chronic gastritis is associated with an elevated risk of colorectal neoplasm. Recent studies based on large databases with careful control for confounding variables have clearly demonstrated an increased risk of colorectal neoplasm associated with H. pylori infection. The correlation between H. pylori-related chronic atrophic gastritis (CAG) and colorectal neoplasm has only been examined in a limited number of studies. A recent large study using a national histopathological database, and our study based on the stage of H. pylori-related chronic gastritis as determined by serum levels of H. pylori antibody titer and pepsinogen, indicated that H. pylori-related CAG confers an increased risk of colorectal neoplasm, and more extensive atrophic gastritis will probably be associated with even higher risk of neoplasm. In addition, our study suggested that the activity of H. pylori-related chronic gastritis is correlated with colorectal neoplasm risk. H. pylori-related chronic gastritis could be involved in an increased risk of colorectal neoplasm that appears to be enhanced by the progression of gastric atrophy and the presence of active inflammation.

  3. Hürthle Cell Neoplasms of Thyroid in South-Western Region of ...

    African Journals Online (AJOL)

    BACKGROUND: Thyroid Hürthle cell neoplasm (THCN) is relatively rare. OBJECTIVE: To describe the presentation, diagnostic approach and management of THCN in our institution. METHODS: This was a retrospective chart review of all thyroid Hürthle cell neoplasms diagnosed at Aseer Central Hospital (ACH), Saudi ...

  4. Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)—Patient Version

    Science.gov (United States)

    Plasma cell neoplasms occur when abnormal plasma cells or myeloma cells form tumors in the bones or soft tissues of the body. Multiple myeloma, plasmacytoma, lymphoplasmacytic lymphoma, and monoclonal gammopathy of undetermined significance (MGUS) are different types of plasma cell neoplasms. Find out about risk factors, symptoms, diagnostic tests, prognosis, and treatment for these diseases.

  5. Giant solitary fibrous tumour of the pleura: a rare but usually benign intrathoracic neoplasm

    DEFF Research Database (Denmark)

    Bodtger, Uffe; Pedersen, Jesper Holst; Skov, Birgit Guldhammer

    2009-01-01

    BACKGROUND: Low forced expiratory volume (FEV(1)) and low performance status usually preclude surgical treatment of lung neoplasms. Earlier case reports have suggested that curative, safe surgery is possible in extrapulmonal intrathoracic neoplasms. METHODS: A case report of an 83-year-old women ...

  6. Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study

    NARCIS (Netherlands)

    Furukawa, Toru; Klöppel, Günter; Volkan Adsay, N.; Albores-Saavedra, Jorge; Fukushima, Noriyoshi; Horii, Akira; Hruban, Ralph H.; Kato, Yo; Klimstra, David S.; Longnecker, Daniel S.; Lüttges, Jutta; Offerhaus, G. Johan A.; Shimizu, Michio; Sunamura, Makoto; Suriawinata, Arief; Takaori, Kyoichi; Yonezawa, Suguru

    2005-01-01

    Now that more than two decades have passed since the first reports of intraductal papillary-mucinous neoplasms (IPMNs), it has become clear that IPMN consists of a spectrum of neoplasms with both morphological and immunohistochemical variations. At a meeting of international experts on pancreatic

  7. [Incidence of haematological neoplasms in Castilla y León, Spain].

    Science.gov (United States)

    Rodríguez-García, José Antonio; Vázquez, Lourdes; Ramos, Fernando; Cuevas, Beatriz; Martín, Alejandro; Smucler, Alicia; Guerola, Dulce Nombre; Cantalapiedra, Alberto; Alonso, José María; Fernández, Silvia; Díez, Eva; Rodríguez, María Jesús; Calmuntia, María José; Aguilar, Carlos; Sierra, Magdalena; Gracia, José Antonio; Cebeira, María José; Cantalejo, Rosa

    2015-06-08

    We aimed to assess the incidence of haematological neoplasms (HNs) in Castilla y León (2,5 million inhabitants) and its distribution by age, gender and histological type. The epidemiological profile based on the described variables of the 10,943 HNs diagnosed during a 10-years period was analyzed, compared with other studies. The overall age-adjusted incidence was 29.4 cases/10(5) inhabitants-year, with some geographical differences. The mean age was 67.3 years, with a turning point between the 6th-7th decades of life from which there was a very significant increase of incidence. Two relevant facts where simultaneous with advancing age: decreased lymphoid neoplasms incidence and increased low degree neoplasms incidence. Lymphoid low degree neoplasms accounted for half of the registered processes, showed the greatest preference for male and reached the mode before the rest of neoplasms. Myeloid neoplasms incidence (9.5) was higher than that reported in other European registries, specially compared to southern European countries, opposite to lymphoid neoplasms incidence (20.0). A higher myeloid neoplasms incidence and lower lymphoid one than expected was observed. The turning point of incidence is between the 6th-7th decades of life, with a preference for male that decreases with age. There is an increased incidence of HNs in the area where a higher density of potentially polluting facilities is concentrated. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  8. CD34-positive stromal cells and alpha-smooth muscle actin-positive stromal cells in the tumor capsule of skin sweat gland neoplasms.

    Science.gov (United States)

    Nakayama, Hirofumi; Enzan, Hideaki; Miyazaki, Eriko; Moriki, Toshiaki; Toi, Makoto; Zhang, Yanhu

    2002-01-01

    To elucidate the roles of CD34-positive stromal cells and alpha-smooth muscle actin-positive stromal cells at the tumor border of skin sweat gland neoplasms, we examined expression of stromal cell markers in the tumor capsule of 19 skin sweat gland neoplasms (16 mixed tumors of the skin and three nodular hidradenomas) using monoclonal antibodies to CD34, CD31, cytokeratin 14 (CK14), alpha-smooth muscle actin (ASMA) and high molecular weight caldesmon (HCD). We regarded CD34-positive, CD31-, CK14-, ASMA- and HCD-negative stromal cells to be CD34-positive stromal cells, and ASMA-positive, HCD-, CK14-, CD34- and CD31-negative stromal cells to be ASMA-positive stromal cells. CD34-positive stromal cells were detected in the tumor capsule of all 19 of the tumors examined. In nine of the 16 mixed tumors (56%) and all of the three nodular hidradenomas, ASMA-positive stromal cells were detected at the immediate inner side of the CD34-positive stromal cell layers. These results indicate that cellular components in the tumor capsules of mixed tumors of the skin and nodular hidradenomas are CD34-positive stromal cells and ASMA-positive stromal cells, and suggest that stromal cells of these two cell types are associated with tumor capsule formation of skin sweat gland neoplasms.

  9. Targeting angiogenesis-dependent calcified neoplasms using combined polymer therapeutics.

    Directory of Open Access Journals (Sweden)

    Ehud Segal

    Full Text Available There is an immense clinical need for novel therapeutics for the treatment of angiogenesis-dependent calcified neoplasms such as osteosarcomas and bone metastases. We developed a new therapeutic strategy to target bone metastases and calcified neoplasms using combined polymer-bound angiogenesis inhibitors. Using an advanced "living polymerization" technique, the reversible addition-fragmentation chain transfer (RAFT, we conjugated the aminobisphosphonate alendronate (ALN, and the potent anti-angiogenic agent TNP-470 with N-(2-hydroxypropylmethacrylamide (HPMA copolymer through a Glycine-Glycine-Proline-Norleucine linker, cleaved by cathepsin K, a cysteine protease overexpressed at resorption sites in bone tissues. In this approach, dual targeting is achieved. Passive accumulation is possible due to the increase in molecular weight following polymer conjugation of the drugs, thus extravasating from the tumor leaky vessels and not from normal healthy vessels. Active targeting to the calcified tissues is achieved by ALN's affinity to bone mineral.The anti-angiogenic and antitumor potency of HPMA copolymer-ALN-TNP-470 conjugate was evaluated both in vitro and in vivo. We show that free and conjugated ALN-TNP-470 have synergistic anti-angiogenic and antitumor activity by inhibiting proliferation, migration and capillary-like tube formation of endothelial and human osteosarcoma cells in vitro. Evaluation of anti-angiogenic, antitumor activity and body distribution of HPMA copolymer-ALN-TNP-470 conjugate was performed on severe combined immunodeficiency (SCID male mice inoculated with mCherry-labeled MG-63-Ras human osteosarcoma and by modified Miles permeability assay. Our targeted bi-specific conjugate reduced VEGF-induced vascular hyperpermeability by 92% and remarkably inhibited osteosarcoma growth in mice by 96%.This is the first report to describe a new concept of a narrowly-dispersed combined polymer therapeutic designed to target both tumor and

  10. Combined use of heat-shock protein 70 and glutamine synthetase is useful in the distinction of typical hepatocellular adenoma from atypical hepatocellular neoplasms and well-differentiated hepatocellular carcinoma

    Science.gov (United States)

    Nguyen, Thuy B; Roncalli, Massimo; Tommaso, Luca Di; Kakar, Sanjay

    2017-01-01

    Well-differentiated hepatocellular carcinoma can mimic high-grade dysplastic nodule in cirrhotic liver and hepatocellular adenoma in non-cirrhotic liver. This study evaluates the efficacy of combined use of heat-shock protein 70 (HSP70), glutamine synthetase (GS) and glypican-3 in this setting. Immunohistochemistry for these three markers was done in 17 typical hepatocellular adenoma, 15 high-grade dysplastic nodules, 20 atypical hepatocellular neoplasms (14 clinically atypical and 6 pathologically atypical), 14 very well-differentiated hepatocellular carcinoma, and 43 well-differentiated hepatocellular carcinoma. All three markers were negative in typical adenomas. HSP70 was positive in 10, 71, and 67% of atypical neoplasms, very well-differentiated and well-differentiated HCC, respectively, while GS was positive in 60, 50, and 60% of atypical neoplasms, very well-differentiated and well-differentiated hepatocellular carcinoma, respectively. Glypican-3 was negative in all atypical neoplasms and very well-differentiated hepatocellular carcinoma, and was positive in 27% of well-differentiated hepatocellular carcinoma. Positive staining with at least one marker (HSP70 and/or GS) was seen in 85% of very well-differentiated hepatocellular carcinoma, which was similar to well-differentiated hepatocellular carcinoma (78%, P = 0.4), and pathologically atypical cases (100%, P = 0.5), but significantly higher compared with clinically atypical cases (43%. P = 0.03) and none of typical adenomas (P hepatocellular carcinoma compared with atypical neoplasms (45 vs 10%, P = 0.004). Both these markers were also more often expressed in very well-differentiated hepatocellular carcinoma compared with atypical cases (38 vs 10%, P = 0.06). In conclusion, the combined use of GS and HSP70 can be useful in the diagnosis of very well-differentiated hepatocellular carcinoma. These stains can also help in the distinction of typical adenoma from atypical hepatocellular neoplasms. Glypican-3

  11. Combined use of heat-shock protein 70 and glutamine synthetase is useful in the distinction of typical hepatocellular adenoma from atypical hepatocellular neoplasms and well-differentiated hepatocellular carcinoma.

    Science.gov (United States)

    Nguyen, Thuy B; Roncalli, Massimo; Di Tommaso, Luca; Kakar, Sanjay

    2016-03-01

    Well-differentiated hepatocellular carcinoma can mimic high-grade dysplastic nodule in cirrhotic liver and hepatocellular adenoma in non-cirrhotic liver. This study evaluates the efficacy of combined use of heat-shock protein 70 (HSP70), glutamine synthetase (GS) and glypican-3 in this setting. Immunohistochemistry for these three markers was done in 17 typical hepatocellular adenoma, 15 high-grade dysplastic nodules, 20 atypical hepatocellular neoplasms (14 clinically atypical and 6 pathologically atypical), 14 very well-differentiated hepatocellular carcinoma, and 43 well-differentiated hepatocellular carcinoma. All three markers were negative in typical adenomas. HSP70 was positive in 10, 71, and 67% of atypical neoplasms, very well-differentiated and well-differentiated HCC, respectively, while GS was positive in 60, 50, and 60% of atypical neoplasms, very well-differentiated and well-differentiated hepatocellular carcinoma, respectively. Glypican-3 was negative in all atypical neoplasms and very well-differentiated hepatocellular carcinoma, and was positive in 27% of well-differentiated hepatocellular carcinoma. Positive staining with at least one marker (HSP70 and/or GS) was seen in 85% of very well-differentiated hepatocellular carcinoma, which was similar to well-differentiated hepatocellular carcinoma (78%, P=0.4), and pathologically atypical cases (100%, P=0.5), but significantly higher compared with clinically atypical cases (43%. P=0.03) and none of typical adenomas (Phepatocellular carcinoma compared with atypical neoplasms (45 vs 10%, P=0.004). Both these markers were also more often expressed in very well-differentiated hepatocellular carcinoma compared with atypical cases (38 vs 10%, P=0.06). In conclusion, the combined use of GS and HSP70 can be useful in the diagnosis of very well-differentiated hepatocellular carcinoma. These stains can also help in the distinction of typical adenoma from atypical hepatocellular neoplasms. Glypican-3 has low

  12. Disseminated Kaposi sarcoma in a HIV negative patient.

    Science.gov (United States)

    Lin, Guoshu; Wang, Hongyan; Fan, Xing; Li, Hui; Wang, Zaixing; Lin, Da; Yang, Sen; Zhang, Xuejun

    2015-01-01

    Kaposi sarcoma (KS) is a neoplasm of the endothelial cells. It often manifests with multiple vascular nodules on the skin and other organs. It is a systemic, malignant and multifactor disease and has a variable course. We describe an elderly Chinese man who had a rapidly growing maroon nodule on his right foot, both arms and cheekbones. KS in HIV-negative patients has only been reported sporadically.

  13. Leucemia mieloide crônica e outras doenças mieloproliferativas crônicas Chronic myeloid leukemia and other chronic myeloproliferative disorders

    Directory of Open Access Journals (Sweden)

    Vaneuza M. Funke

    2010-05-01

    HSCT have been restricted as a result of mortality related to this procedure and imatinib mesylate has become the new treatment of choice for this disease. In Brazil and possibly in other developing countries, socio-economic conditions make HSCT still feasible as first-line treatment in some situations. The HSCT remains indicated for Ph negative myeloproliferative disorders such as high risk myelofibrosis or patients with polycythemia vera or essential thrombocytosis that have evolved to myelofibrosis with high-risk features.

  14. Perforated colorectal neoplasms: Correlation of clinical, barium, and CT examinations

    International Nuclear Information System (INIS)

    Hulnick, D.H.; Megibow, A.J.; Gordon, R.; Balthazar, E.J.

    1986-01-01

    Findings on clinical, barium enema, and CT examinations in 24 patients with perforated colorectal neoplasms were retrospectively reviewed. In 12 patients with fever and leukocytosis, the clinical diagnoses were diverticulitis (n=8), appendicitis (n=2), pelvic inflammatory disease (n=2), and perforation of a known carcinoma (n=1). In 12 nontoxic patients, the clinical diagnoses were malignancy (n=8), obstruction (n=3) and an abdominal mass (n=1). In 18 barium enema examinations, the radiologic impression was uncomplicated carcinoma (n=9), perforated carcinoma (n=6), and diverticulitis (n=3). Twenty-two CT examinations revealed pericolic inflammation in all cases and macroperforation in 12. The primary tumor was evident in 14 patients, liver metastases were found in six, and ascites was present in three. CT findings influenced the management of patients initially suspected of having inflammatory conditions

  15. Autopsy findings of malignant neoplasms treated with radiation

    International Nuclear Information System (INIS)

    Okazaki, Atsushi; Ito, Jun; Tatezawa, Takashi; Nishimura, Toshinobu; Niibe, Hideo.

    1984-01-01

    Autopsy findings in 26 cases of malignant neoplasms treated with radiation were analysed and following results were obtained. 1. Causes of death except for neoplastic extension were 58% (15/26) and infection was the single predominant cause of death (73% : 11/15) with 50% (4/8) in lung cancer. 2. Distant metastases were found in 73% (19/26). In 7 cases, no obvious metastasis was found before and after autopsy. On the other hand, the patients with metastases over 2 organs before autopsy revealed metastases in 82% (9/11) to the other organs at autopsy. 3. Radiation therapy was effective and the primary tumor disappeared completely in 71% (10/14) with curative irradiation. In metastatic lesions, tumor cells were almost disappeared with dosage over 40 Gy. (author)

  16. Multiple primary malignant neoplasms in breast cancer patients in Israel

    International Nuclear Information System (INIS)

    Schenker, J.G.; Levinsky, R.; Ohel, G.

    1984-01-01

    The data of an epidemiologic study of multiple primary malignant neoplasms in breast cancer patients in Israel are presented. During the 18-year period of the study 12,302 cases of breast carcinoma were diagnosed, and, of these, 984 patients (8%) had multiple primary malignant tumors. Forty-seven of these patients developed two multiple primary cancers. A significantly higher than expected incidence of second primary cancers occurred at the following five sites: the opposite breast, salivary glands, uterine corpus, ovary, and thyroid. Cancers of the stomach and gallbladder were fewer than expected. Treatment of the breast cancer by irradiation was associated with an increased risk of subsequent cancers of lung and hematopoietic system. The prognosis was mainly influenced by the site and malignancy of the second primary cancer. The incidence of multiple primary malignancies justifies a high level of alertness to this possibility in the follow-up of breast cancer patients

  17. Localization of gonadotropin binding sites in human ovarian neoplasms

    International Nuclear Information System (INIS)

    Nakano, R.; Kitayama, S.; Yamoto, M.; Shima, K.; Ooshima, A.

    1989-01-01

    The binding of human luteinizing hormone and human follicle-stimulating hormone to ovarian tumor biopsy specimens from 29 patients was analyzed. The binding sites for human luteinizing hormone were demonstrated in one tumor of epithelial origin (mucinous cystadenoma) and in one of sex cord-stromal origin (theca cell tumor). The binding sites for human follicle-stimulating hormone were found in three tumors of epithelial origin (serous cystadenoma and mucinous cystadenoma) and in two of sex cord-stromal origin (theca cell tumor and theca-granulosa cell tumor). The surface-binding autoradiographic study revealed that the binding sites for gonadotropins were localized in the stromal tissue. The results suggest that gonadotropic hormones may play a role in the growth and differentiation of a certain type of human ovarian neoplasms

  18. Thyroid neoplasms after radiation therapy for adolescent acne vulgaris

    International Nuclear Information System (INIS)

    Paloyan, E.; Lawrence, A.M.

    1978-01-01

    There is a potential hazard of thyroid cancer after exposure to external irradiation for the treatment of adolescent acne vulgaris. We noted a 60% incidence of thyroid carcinoma among 20 patients with such a history, who were operated on for thyroid nodules during a five-year period. Eighty-three percent of the patients with carcinoma had either a follicular or a mixed papillary-follicular carcinoma; 17% had a papillary carcinoma; 33% had regional node metastases; none had evidence of distant metastases. The interval between radiation exposure and thyroidectomy ranged from nine to 41 years. This association of thyroid neoplasms and a prior history of radiation for acne vulgaris may be coincidental and therefore remains to be proved by retrospective surveys of large numbers of treated patients with appropriate controls

  19. CT diagnosis of hyperdense intracranial neoplasms. Review of the literature

    International Nuclear Information System (INIS)

    Ishikura, Reiichi; Ando, Kumiko; Tominaga, Satoru; Nakao, Norio; Ikeda, Jouta; Takemura, Yuriko; Morikawa, Tsutomu

    1999-01-01

    In contrast to typical astrocytic tumors that show hypodense areas on computed tomographic images, some intracranial tumors show hyperdense areas on CT images. The major reasons for hyperdensity on CT images are hypercellular lesions, intratumoral calcification, and intratumoral hemorrhage. Malignant lymphomas, germinomas, and medulloblastomas show homogenous hyperdensity on CT images because of their hypercellularity. Tumorous lesions such as subependymal giant cell astrocytomas, oligodendrogliomas, ependymomas, central neurocytomas, craniopharyngiomas, and meningiomas often present with hyperdense calcified lesions on CT images. Intratumoral hemorrhage also causes hyperdensity on CT images, and is often associated with metastatic brain tumors, glioblastomas, pituitary adenomas, and rarely with any of the other intracranial tumors. Although magnetic resonance imaging is now the major diagnostic tool for diseases of the central nervous system, the first imaging studies for patients with neurologic symptoms are still CT scans. Hyperdense areas on CT images are a clue to making an accurate diagnosis of intracranial neoplasms. (author)

  20. Aggressive management of peritoneal carcinomatosis from mucinous appendiceal neoplasms.

    Science.gov (United States)

    Austin, Frances; Mavanur, Arun; Sathaiah, Magesh; Steel, Jennifer; Lenzner, Diana; Ramalingam, Lekshmi; Holtzman, Matthew; Ahrendt, Steven; Pingpank, James; Zeh, Herbert J; Bartlett, David L; Choudry, Haroon A

    2012-05-01

    Peritoneal carcinomatosis (PC) in the setting of mucinous appendiceal neoplasms is characterized by the intraperitoneal accumulation of mucinous ascites and mucin-secreting epithelial cells that leads to progressive compression of intra-abdominal organs, morbidity, and eventual death. We assessed postoperative and oncologic outcomes after aggressive surgical management by experienced surgeons. We analyzed clinicopathologic, perioperative, and oncologic outcome data in 282 patients with PC from appendiceal adenocarcinomas between 2001 and 2010 from a prospective database. Kaplan–Meier survival curves and multivariate Cox-regression models were used to identify prognostic factors affecting oncologic outcomes. Adequate cytoreduction was achieved in 82% of patients (completeness of cytoreduction score (CC)-0: 49%; CC-1: 33%). Median simplified peritoneal cancer index (SPCI), operative time, and estimated blood loss were 14 (range, 0–21), 483.5 min (range, 46–1,402), and 800 ml (range, 0–14,000), respectively. Pathology assessment demonstrated high-grade tumors in 36% of patients and lymph node involvement in 23% of patients. Major postoperative morbidity occurred in 70 (25%) patients. Median overall survival was 6.72 years (95% confidence interval (CI), 4.17 years not reached), with 5 year overall survival probability of 52.7% (95% CI, 42.4, 62%). In a multivariate Cox-regression model, tumor grade, age, preoperative SPCI and chemo-naïve status at surgery were joint significant predictors of overall survival. Tumor grade, postoperative CC-score, prior chemotherapy, and preoperative SPCI were joint significant predictors of time to progression. Aggressive management of PC from mucinous appendiceal neoplasms, by experienced surgeons, to achieve complete cytoreduction provides long-term survival with low major morbidity.

  1. Active Surveillance for Renal Neoplasms with Oncocytic Features is Safe.

    Science.gov (United States)

    Richard, Patrick O; Jewett, Michael A S; Bhatt, Jaimin R; Evans, Andrew J; Timilsina, Narhari; Finelli, Antonio

    2016-03-01

    Oncocytomas are benign tumors often diagnosed incidentally on imaging. Small case series have suggested that the growth kinetics of oncocytomas are similar to those of malignant renal tumors. Biopsy material may be insufficient to exclude a diagnosis of chromophobe renal cell carcinoma. We evaluated and compared the growth rates of oncocytoma and chromophobe renal cell carcinoma to improve our understanding of their natural history. This was a single center, retrospective study of patients diagnosed with lesions suggestive of oncocytoma or chromophobe renal cell carcinoma between 2003 and 2014. The growth rates were estimated using a mixed effect linear model. Patient and lesion characteristics were tested using a similar model for association with growth rate. Of the 95 lesions (oncocytoma 81, chromophobe renal cell carcinoma 14) included in the analysis 98% were diagnosed on biopsy. The annual growth rate was 0.14 cm and 0.38 cm for oncocytoma (median followup 34 months) and chromophobe renal cell carcinoma (median followup 25 months), respectively (p=0.5). Baseline lesion size was significantly associated with growth (p renal cell carcinomas (67%) followed up to the 3-year mark had grown. Of these, 8 underwent surgery (6 in the chromophobe renal cell carcinoma group). The initial diagnosis was confirmed in all. Overall 5 patients died, all of nonrenal related causes. Although the majority of oncocytic renal neoplasms will grow with time, surveillance appears to remain safe. Patients opting for this strategy should be made aware that a diagnosis of oncocytoma following biopsy is associated with some degree of uncertainty due to the difficulty of differentiating them from other oncocytic renal neoplasms. Copyright © 2016 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  2. Percutaneous thermal ablation of renal neoplasms; Perkutane Thermoablation von Nierentumoren

    Energy Technology Data Exchange (ETDEWEB)

    Tacke, J. [Inst. fuer Diagnostische und Interventionelle Radiologie/Neuroradiologie, Klinikum Passau (Germany); Mahnken, A.H.; Guenther, R.W. [Klinik fuer Radiologische Diagnostik, Universitaetsklinikum Aachen (Germany)

    2005-12-15

    Due to modern examination techniques such as multidetector computed tomography and high-field magnetic resonance imaging, the detection rate of renal neoplasms is continually increasing. Even though tumors exceeding 4 cm in diameter rarely metastasize, all renal lesions that are possible neoplasms should be treated. Traditional treatment techniques include radical nephrectomy or nephron-sparing resection, which are increasingly performed laparoscopically. Modern thermal ablation techniques such as hyperthermal techniques like radiofrequency ablation RFA, laser induced thermal ablation LITT, focused ultrasound FUS and microwave therapy MW, as well as hypothermal techniques (cryotherapy) may be a useful treatment option for patients who are unfit for or refuse surgical resection. Cryotherapy is the oldest and best known thermal ablation technique and can be performed laparoscopically or percutaneously. Since subzero temperatures have no antistyptic effect, additional maneuvers must be performed to control bleeding. Percutaneous cryotherapy of renal tumors is a new and interesting method, but experience with it is still limited. Radiofrequency ablation is the most frequently used method. Modern probe design allows volumes between 2 and 5 cm in diameter to be ablated. Due to hyperthermal tract ablation, the procedure is deemed to be safe and has a low complication rate. Although there are no randomized comparative studies to open resection, the preliminary results for renal RFA are promising and show RFA to be superior to other thermal ablation techniques. Clinical success rates are over 90% for both, cryo- and radiofrequency ablation. Whereas laser induced thermal therapy is established in hepatic ablation, experience is minimal with respect to renal application. For lesions of more than 2 cm in diameter, additional cooling catheters are required. MR thermometry offers temperature control during ablation. Microwave ablation is characterized by small ablation volumes

  3. Spectrum of Intestinal Neoplasms: A study of 400 cases

    Directory of Open Access Journals (Sweden)

    Aminder Singh

    2015-02-01

    Full Text Available Objective: The present study is a five-year analysis of all the tumors of small and large intestine received in the Pathology Department of Dayanand Medical College and Hospital, Ludhiana. Methods: All the cases were grossly and microscopically examined and were staged according to Astler Coller Staging and classified and subtyped according to WHO classification. The important differences between the small and large intestine tumors were also analyzed. Results: There were 400 cases out of which 356 were in large intestine while 44 were in the small intestine. There were only 56 benign neoplasms while 344 were malignant tumors. Adenomas were the most common benign tumors while majority of malignant tumors were adenocarcinomas. Lymphomas, mesenchymal tumors, and carcinoid tumors were much more common in a small intestine as compared to large intestine. Majority of adenocarcinomas were located in the large intestine with most of them being moderately differentiated having Astler Coller Stage B II. Mucinous carcinomas had the worst prognosis as compared to adenocarcinomas. Anal canal had mainly squamous cell carcinomas. Conclusions: Tumors of large intestine were much commoner than of small intestine. There was a higher incidence of tumor in males with M:F ratio of 2.2:1. Mean age of presentation of benign tumor was younger, i.e., 32.6 years when compared to 54.5 years for malignant tumors. Tubular adenoma was the most common benign tumor and adenocarcinoma the commonest malignant neoplasm. [J Interdiscipl Histopathol 2015; 3(1.000: 19-23

  4. File list: InP.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

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  12. Comparison between MRI with MR cholangiopancreatography and endoscopic ultrasonography for differentiating malignant from benign mucinous neoplasms of the pancreas

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Jiyoung; Hong, Seong Sook; Kim, Hyun-joo [Seoul Hospital, Department of Radiology, Soonchunhyang University College of Medicine, Seoul (Korea, Republic of); Kim, Young Kon; Jeong, Woo Kyung [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Min, Ji Hye [Chungnam National University College of Medicine, Department of Radiology, Chungnam National University Hospital, Daejeon (Korea, Republic of)

    2018-01-15

    To compare diagnostic performance of magnetic resonance imaging (MRI) and endoscopic ultrasonography (EUS) for differentiating malignant from benign intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms (MCN) of the pancreas. This retrospective study included 55 patients with 47 surgically confirmed IPMNs (12 malignant, 35 benign) and eight MCNs (two malignant, six benign) who underwent contrast-enhanced pancreas MRI and EUS. Contrast enhancement was not routinely used at EUS examination. Two observers independently evaluated the MRIs, and another reviewed EUS images. They recorded their confidence for malignancy with each imaging modality. We calculated diagnostic performance using the area under the receiver operating characteristic curves (A{sub z}), and to determine the accuracy, sensitivity, specificity, and positive (PPV) and negative predictive (NPV) values. The A{sub z} values of MRI were higher than those of EUS (0.712 and 0.688 for MRI vs. 0.543 for EUS; p = 0.007). The diagnostic accuracies (74.5%), specificity (78.0% and 80.5%) and PPV (50.0%) of MRI in two observers were higher than those (56.4%, 58.5% and 29.2%, respectively) of EUS (p = 0.013-0.049). MRI showed better diagnostic performance than EUS for differentiating malignant from benign pancreatic IPMN and MCN. (orig.)

  13. A case series of clinically undiagnosed hematopoietic neoplasms discovered at autopsy.

    Science.gov (United States)

    Podduturi, Varsha; Guileyardo, Joseph M; Soto, Luis R; Krause, John R

    2015-06-01

    In the United States, autopsy rates have diminished to less than 5% during the last half of the 20th century and the beginning of the 21st century for a multitude of reasons. Many believe this results in unrecognized malignancies that could have explained a patient's death. We describe six deaths in which hematopoietic neoplasms were identified at autopsy but were not diagnosed clinically. The six undiagnosed hematopoietic malignancy cases discovered at autopsy include four men and two women ranging from 50 to 78 years of age. One patient was African American and five patients were white, all with multiple comorbidities. The tumors included diffuse large B-cell lymphoma, activated B-cell type, intravascular large B-cell lymphoma, ALK-negative anaplastic large cell lymphoma arising in a setting of human immunodeficiency virus, and a myeloid sarcoma. These cases illustrate the importance of the traditional postmortem examination in not only confirming clinical diagnoses but also identifying previously unknown diagnoses. Hematologic malignancies may present with nonspecific clinical manifestations, and this series of cases also emphasizes the necessity for widening the differential diagnosis in patients with unexplained lactic acidosis and hepatic failure to include hematopoietic malignancies since prompt treatment may be lifesaving. Copyright© by the American Society for Clinical Pathology.

  14. Improved Detection of Circulating Epithelial Cells in Patients with Intraductal Papillary Mucinous Neoplasms.

    Science.gov (United States)

    Franses, Joseph W; Basar, Omer; Kadayifci, Abdurrahman; Yuksel, Osman; Choz, Melissa; Kulkarni, Anupriya S; Tai, Eric; Vo, Kevin D; Arora, Kshitij S; Desai, Niyati; Licausi, Joseph A; Toner, Mehmet; Maheswaran, Shyamala; Haber, Daniel A; Ryan, David P; Brugge, William R; Ting, David T

    2018-01-01

    Recent work has demonstrated early shedding of circulating epithelial cells (CECs) from premalignant intraductal papillary mucinous neoplasms (IPMNs). However, the potential use of CECs as a "liquid biopsy" for patients with IPMNs has been limited by antigen dependence of CEC isolation devices and the lack of robust detection biomarkers across CEC phenotypes. We utilized a negative depletion microfluidic platform to purify CECs from contaminating leukocytes and coupled this platform with immunofluorescence, RNA in situ hybridization, and RNA sequencing (RNA-seq) detection and enumeration. Using established protein (EpCAM, cytokeratins) and novel noncoding RNA (HSATII, cytokeratins) biomarkers, we detected CECs in 88% of patients bearing IPMN lesions. RNA-seq analysis for MUC genes confirm the likely origin of these CECs from pancreatic lesions. Our findings increase the sensitivity of detection of these cells and therefore could have clinical implications for cancer risk stratification. This work describes a high-sensitivity platform for detection of epithelial cells shed from preneoplastic lesions at high risk of malignant transformation. Further research efforts are underway to define the transcriptional programs that might allow discrimination between circulating cells released from tumors that will become malignant and cells released from tumors that will not. After further refinement, this combination of technologies could be deployed for monitoring and early detection of patients at high risk for developing new or recurrent pancreatic malignancies. © AlphaMed Press 2017.

  15. NY-ESO-1 is a sensitive and specific immunohistochemical marker for myxoid and round cell liposarcomas among related mesenchymal myxoid neoplasms.

    Science.gov (United States)

    Hemminger, Jessica A; Iwenofu, O Hans

    2013-09-01

    positivity), and two cases (6%) showed weak (1+ intensity), patchy staining (20-30% tumor positivity). Immunoreactivity was predominantly cytoplasmic. All the other neoplasms evaluated were negative for NY-ESO-1. NY-ESO-1 appears to be a sensitive and a specific marker for myxoid and round cell liposarcoma among mesenchymal myxoid neoplasms. The assessment of NY-ESO-1 expression by immunohistochemistry in the appropriate setting provides a cheaper, faster, and more accessible confirmatory test.

  16. Diagnostic and therapeutic implications of genetic heterogeneity in myeloid neoplasms uncovered by comprehensive mutational analysis

    Directory of Open Access Journals (Sweden)

    Sarah M. Choi

    2017-01-01

    Full Text Available While growing use of comprehensive mutational analysis has led to the discovery of innumerable genetic alterations associated with various myeloid neoplasms, the under-recognized phenomenon of genetic heterogeneity within such neoplasms creates a potential for diagnostic confusion. Here, we describe two cases where expanded mutational testing led to amendment of an initial diagnosis of chronic myelogenous leukemia with subsequent altered treatment of each patient. We demonstrate the power of comprehensive testing in ensuring appropriate classification of genetically heterogeneous neoplasms, and emphasize thoughtful analysis of molecular and genetic data as an essential component of diagnosis and management.

  17. Pitfalls in the staging of cancer of the major salivary gland neoplasms.

    Science.gov (United States)

    Friedman, Elliott R; Saindane, Amit M

    2013-02-01

    The major salivary glands consist of the parotid, submandibular, and sublingual glands. Most neoplasms in other subsites in the head and neck are squamous cell carcinoma, but tumors of the salivary glands may be benign or malignant. Surgical treatment differs if the lesion is benign, and therefore preoperative fine needle aspiration is important in salivary neoplasms. The role of imaging is to attempt to determine histology, predict likelihood of a lesion being malignant, and report an imaging stage. This article reviews the various histologies, imaging features, and staging of major salivary gland neoplasms. Copyright © 2013 Elsevier Inc. All rights reserved.

  18. Branch duct-type intraductal papillary mucinous neoplasm presenting as paraneoplastic small plaque para-psoriasis

    Directory of Open Access Journals (Sweden)

    Cyriac A Philips

    2018-01-01

    Full Text Available To present and discuss a novel association between branch duct-type intraductal papillary mucinous neoplasm and paraneoplastic parapsoriasis. We present the case of a middle-aged male presenting with skin lesions that were suggestive of parapsoriasis, resistant to treatment, and in whom a diagnosis of branch-type intraductal papillary mucinous neoplasm of the pancreas was eventually made. A curative Whipple's surgery led to complete resolution of the skin lesions within 3 weeks. Paraneoplastic parapsoriasis in association with intraductal papillary mucinous pancreatic neoplasm has never been reported before.

  19. Branch Duct-type Intraductal Papillary Mucinous Neoplasm Presenting as Paraneoplastic Small Plaque Para-psoriasis

    Science.gov (United States)

    Philips, Cyriac A.; Augustine, Philip; Kumar, Lijesh; Joseph, George; Mahadevan, Pushpa

    2018-01-01

    To present and discuss a novel association between branch duct-type intraductal papillary mucinous neoplasm and paraneoplastic parapsoriasis. We present the case of a middle-aged male presenting with skin lesions that were suggestive of parapsoriasis, resistant to treatment, and in whom a diagnosis of branch-type intraductal papillary mucinous neoplasm of the pancreas was eventually made. A curative Whipple's surgery led to complete resolution of the skin lesions within 3 weeks. Paraneoplastic parapsoriasis in association with intraductal papillary mucinous pancreatic neoplasm has never been reported before. PMID:29441297

  20. CK13 in craniopharyngioma versus related odontogenic neoplasms and human enamel organ.

    Science.gov (United States)

    el-Sissy, N A; Rashad, N A

    1999-05-01

    The monoclonal antibody NCL-CK13 was studied in specimens of craniopharyngioma, ameloblastoma and calcifying odontogenic cyst neoplasms and the mandible and maxillae of normal human fetuses. There was a decrease in NCL-CK13 as the dental lamina developed, with a complete loss in the enamel organ. The neoplastic epithelia of the neoplasms revealed a clear phenotypic and immunohistochemical reactive relationship to the stratified embroyonic mucosa, away from the enamel organ. This suggests that these neoplasms might have their histogenesis from early stage epithelium, the oral part of the dental lamina or its remnants.

  1. [Bladder neoplasm in a patient with panarteritis nodosa treated with cyclophosphamide].

    Science.gov (United States)

    Albanell, J; Gallego, O S; Bellmunt, J; Vicente, P; Morales, S; Solé, L A

    1992-05-01

    Cyclophosphamide is used both in the treatment of malignant and non-malignant diseases. Urinary neoplasms secondary to its use have been described. We discuss the case of a patient with panarteritis nodosa treated with cyclophosphamide during 63 months, with a total dose of 210 grams, and that showed a bladder neoplasm 8 years after beginning of the treatment. In patients receiving a total dose of cyclophosphamide over 85 grams, a follow-up of ten years minimum should be performed aimed to the early detection of secondary neoplasms.

  2. Branch Duct-type Intraductal Papillary Mucinous Neoplasm Presenting as Paraneoplastic Small Plaque Para-psoriasis.

    Science.gov (United States)

    Philips, Cyriac A; Augustine, Philip; Kumar, Lijesh; Joseph, George; Mahadevan, Pushpa

    2018-01-01

    To present and discuss a novel association between branch duct-type intraductal papillary mucinous neoplasm and paraneoplastic parapsoriasis. We present the case of a middle-aged male presenting with skin lesions that were suggestive of parapsoriasis, resistant to treatment, and in whom a diagnosis of branch-type intraductal papillary mucinous neoplasm of the pancreas was eventually made. A curative Whipple's surgery led to complete resolution of the skin lesions within 3 weeks. Paraneoplastic parapsoriasis in association with intraductal papillary mucinous pancreatic neoplasm has never been reported before.

  3. Changing patterns of radiosensitivity of hematopoietic progenitors from chronically irradiated dogs prone either to aplastic anemia or to myeloproliferative disease.

    Science.gov (United States)

    Seed, T M; Kaspar, L V

    1990-01-01

    Hematopoietic patterns have been assessed in chronic 60Co gamma irradiated dogs during preclinical phases of evolving aplastic anemia (AA) or myeloproliferative disease (MPD), principally myeloid leukemia. Within the AA-prone dog, a singular phase of progressive decline in blood levels of granulocytes and monocytes was noted along with a similar reduction in marrow progenitors committed to granulocyte/monocyte differentiation (CFU-GM). Measured radioresistance of the preAA CFU-GM in vitro, relative to control CFU-GM from nonirradiated animals, revealed only slightly increased resistance to gamma rays, but significantly increased resistance to fission neutrons. Within the MPD-prone dogs, four preclinical phases (i.e. suppression, partial recovery, accommodation, and preleukemic transition) preceding development of overt MPD were evidenced by the monitored change in blood granulocyte/monocyte counts and marrow progenitor levels. Analysis of radioresistance of preMPD CFU-GM revealed marked changes with time of exposure and, in turn, with preclinical phase transitions. Gamma ray resistance increased in the initial phases of exposure, with maximal levels occurring during the middle phase of exposure (accommodation, phase III) followed by a tailing off of resistance at later times. Resistance to fission neutrons by preMPD CFU-GM was observed as well, but somewhat later in the exposure course and at a much lower, more consistent level. These differential patterns of radioresistance expressed by marrow CFU-GM of chronically irradiated MPD-prone dogs to gamma rays and fission neutrons gave rise to preclinical phase-specific 'relative biological effectiveness' (RBE) values. From these observations, we conclude that: (i) CFU-GM of MPD-prone dogs acquire and maintain marked radioresistance to low linear energy transfer (LET) gamma rays, but only marginally elevated radioresistance to high-LET fission neutrons during the course of chronic gamma ray exposure; and (ii) CFU-GM of

  4. Pediatric adrenocortical neoplasms: can imaging reliably discriminate adenomas from carcinomas?

    International Nuclear Information System (INIS)

    Flynt, Kelsey A.; Dillman, Jonathan R.; Smith, Ethan A.; Strouse, Peter J.; Davenport, Matthew S.; Caoili, Elaine M.; Else, Tobias

    2015-01-01

    There is a paucity of literature describing and comparing the imaging features of adrenocortical adenomas and carcinomas in children and adolescents. To document the CT and MRI features of adrenocortical neoplasms in a pediatric population and to determine whether imaging findings (other than metastatic disease) can distinguish adenomas from carcinomas. We searched institutional medical records to identify pediatric patients with adrenocortical neoplasms. Pre-treatment CT and MRI examinations were reviewed by two radiologists in consensus, and pertinent imaging findings were documented. We also recorded relevant histopathological, demographic, clinical follow-up and survival data. We used the Student's t-test and Wilcoxon rank sum test to compare parametric and nonparametric continuous data, and the Fisher exact test to compare proportions. We used receiver operating characteristic (ROC) curve analyses to evaluate the diagnostic performances of tumor diameter and volume for discriminating carcinoma from adenoma. A P-value ≤0.05 was considered statistically significant. Among the adrenocortical lesions, 9 were adenomas, 15 were carcinomas, and 1 was of uncertain malignant potential. There were no differences in mean age, gender or sidedness between adenomas and carcinomas. Carcinomas were significantly larger than adenomas based on mean estimated volume (581 ml, range 16-2,101 vs. 54 ml, range 3-197 ml; P-value = 0.003; ROC area under the curve = 0.92) and mean maximum transverse plane diameter (9.9 cm, range 3.0-14.9 vs. 4.4 cm, range 1.9-8.2 cm; P-value = 0.0001; ROC area under the curve = 0.92). Carcinomas also were more heterogeneous than adenomas on post-contrast imaging (13/14 vs. 2/9; odds ratio [OR] = 45.5; P-value = 0.001). Six of 13 carcinomas and 1 of 8 adenomas contained calcification at CT (OR = 6.0; P-value = 0.17). Seven of 15 children with carcinomas exhibited metastatic disease at diagnosis, and three had inferior vena cava invasion. Median

  5. Pediatric adrenocortical neoplasms: can imaging reliably discriminate adenomas from carcinomas?

    Energy Technology Data Exchange (ETDEWEB)

    Flynt, Kelsey A.; Dillman, Jonathan R.; Smith, Ethan A.; Strouse, Peter J. [University of Michigan Health System, Section of Pediatric Radiology, C. S. Mott Children' s Hospital, Department of Radiology, Ann Arbor, MI (United States); Davenport, Matthew S.; Caoili, Elaine M. [University of Michigan Health System, Division of Abdominal Imaging, Department of Radiology, Ann Arbor, MI (United States); Else, Tobias [University of Michigan Health System, Division of Metabolism, Endocrinology and Diabetes, Department of Internal Medicine, Ann Arbor, MI (United States)

    2015-08-15

    There is a paucity of literature describing and comparing the imaging features of adrenocortical adenomas and carcinomas in children and adolescents. To document the CT and MRI features of adrenocortical neoplasms in a pediatric population and to determine whether imaging findings (other than metastatic disease) can distinguish adenomas from carcinomas. We searched institutional medical records to identify pediatric patients with adrenocortical neoplasms. Pre-treatment CT and MRI examinations were reviewed by two radiologists in consensus, and pertinent imaging findings were documented. We also recorded relevant histopathological, demographic, clinical follow-up and survival data. We used the Student's t-test and Wilcoxon rank sum test to compare parametric and nonparametric continuous data, and the Fisher exact test to compare proportions. We used receiver operating characteristic (ROC) curve analyses to evaluate the diagnostic performances of tumor diameter and volume for discriminating carcinoma from adenoma. A P-value ≤0.05 was considered statistically significant. Among the adrenocortical lesions, 9 were adenomas, 15 were carcinomas, and 1 was of uncertain malignant potential. There were no differences in mean age, gender or sidedness between adenomas and carcinomas. Carcinomas were significantly larger than adenomas based on mean estimated volume (581 ml, range 16-2,101 vs. 54 ml, range 3-197 ml; P-value = 0.003; ROC area under the curve = 0.92) and mean maximum transverse plane diameter (9.9 cm, range 3.0-14.9 vs. 4.4 cm, range 1.9-8.2 cm; P-value = 0.0001; ROC area under the curve = 0.92). Carcinomas also were more heterogeneous than adenomas on post-contrast imaging (13/14 vs. 2/9; odds ratio [OR] = 45.5; P-value = 0.001). Six of 13 carcinomas and 1 of 8 adenomas contained calcification at CT (OR = 6.0; P-value = 0.17). Seven of 15 children with carcinomas exhibited metastatic disease at diagnosis, and three had inferior vena cava invasion. Median

  6. Laparoscopic Low Anterior Resection and Eversion Technique Combined With a Nondog Ear Anastomosis for Mid- and Distal Rectal Neoplasms: A Preliminary and Feasibility Study.

    Science.gov (United States)

    Zhuo, Changhua; Liang, Lei; Ying, Mingang; Li, Qingguo; Li, Dawei; Li, Yiwei; Peng, Junjie; Huang, Liyong; Cai, Sanjun; Li, Xinxiang

    2015-12-01

    The transanal eversion and prolapsing technique is a well-established procedure, and can ensure an adequate distal margin for patients with low rectal neoplasms. Potential leakage risks, however, are associated with bilateral dog ear formation, which results from traditional double-stapling anastomosis. The authors determined the feasibility of combining these techniques with a commercial stapling set to achieve a nondog ear (end-to-end) anastomosis for patients with mid- and distal rectal neoplasms. Patients with early-stage (c/ycT1-2N0), mid- to distal rectal neoplasms and good anal sphincter function were included in this study. Laparoscopic low anterior resection was performed with a standard total mesorectal excision technique downward to the pelvic floor as low as possible. The bowel was resected proximal to the lesion with an endoscopic linear stapler. An anvil was inserted extracorporeally into the proximal colon via an extended working pore. The distal rectum coupled with the lesion was prolapsed and everted out of the anus. The neoplasm was resected with a sufficient margin above the dentate line under direct sight. A transrectal anastomosis without dog ears was performed intracorporeally to reconstitute the continuity of the bowel. Eleven cases, 6 male and 5 female patients, were included in this study. The mean operative time was 191 (129-292) minutes. The mean blood loss was 110 (30-300) mL. The median distal margin distance from the lower edge of the lesion to the dentate line was 1.5 (0.5-2.5) cm. All the resection margins were negative. Most patients experienced uneventful postoperative recoveries. No patient had anastomotic leak. Most patients had an acceptable stool frequency after loop ileostomy closure. Our preliminary data demonstrated the safety and feasibility of achieving a sound anastomosis without risking potential anastomotic leakage because of dog ear formation.

  7. Pterygium: degeneration, exuberant wound healing or benign neoplasm?

    Directory of Open Access Journals (Sweden)

    Nurbuanto Tradjutrisno

    2016-02-01

    Full Text Available Pterygium is a condition characterized by the encroachment of a fleshy triangle of conjunctival tissue into the cornea. Despite various studies, the pathogenesis of pterygium remains unclear. Chronic exposure to sun is the only factor of which the role has been clearly documented by epidemiologic and in vitro studies. Recent studies have provided data such as loss of heterozygosity and microsatellite instability, decreased apoptosis, increased growth factors, increased p53 expression, telomerase activity, and presence of oncogenic viruses, which support the concept that pterygia can be considered a neoplastic condition. Some of results are contradictory and some features were only found in a proportion of pterygia; this may be due to differences in methodology and study population, but may also be indicative of multiple causative factors. This implies that the pterygium should be viewed as a final common outcome of various etiological factors, such as geography and ethnicity, with ultraviolet radiation-induced damage as the main factor. Thus the pterygium remains an enigma, having the features of an exuberant growth but also those of a benign neoplasm. In this connection there is a need for a unifying theory capable of explaining all of the characteristics of the pterygium.

  8. Pterygium: degeneration, exuberant wound healing or benign neoplasm?

    Directory of Open Access Journals (Sweden)

    Nurbuanto Tradjutrisno

    2009-12-01

    Full Text Available Pterygium is a condition characterized by the encroachment of a fleshy triangle of conjunctival tissue into the cornea. Despite various studies, the pathogenesis of pterygium remains unclear. Chronic exposure to sun is the only factor of which the role has been clearly documented by epidemiologic and in vitro studies. Recent studies have provided data such as loss of heterozygosity and microsatellite instability, decreased apoptosis, increased growth factors, increased p53 expression, telomerase activity, and presence of oncogenic viruses, which support the concept that pterygia can be considered a neoplastic condition. Some of results are contradictory and some features were only found in a proportion of pterygia; this may be due to differences in methodology and study population, but may also be indicative of multiple causative factors. This implies that the pterygium should be viewed as a final common outcome of various etiological factors, such as geography and ethnicity, with ultraviolet radiation-induced damage as the main factor. Thus the pterygium remains an enigma, having the features of an exuberant growth but also those of a benign neoplasm. In this connection there is a need for a unifying theory capable of explaining all of the characteristics of the pterygium.

  9. Current trend of malignant neoplasms among atomic bomb survivors

    International Nuclear Information System (INIS)

    Hamada, Tadao

    1984-01-01

    A survey was made on 7,589 admitted patients and 1,965 autopsy cases. The overall incidence of malignant neoplasms tended to decrease in the group exposed to atomic bomb within 2 km in autopsy cases and to increase in admitted patients. The incidence of pulmonary cancer tended to increase in both autopsy cases and admitted patients. The incidence of gastric cancer tended to increase up to 1975, and thereafter tended to decrease. The incidence of liver cancer tended to increase in both autopsy cases and admitted patients, which was marked in males. The incidence of leukemia was high in the group exposed to atomic bomb within 2 km in autopsy cases, and in the group within 1 km and the group which entered the city after the explosion in admitted patients. The incidence of malignant lymphoma tended to decrease, and the incidence of carcinoma of the colon tended to gradually increase in both autopsy cases and admitted patients. The incidence of multiple carcinomas tended to increase in both atomic bomb exposed group and non-exposed group, being higher in atomic bomb group than in non-exposed group. The incidence of breast cancer became constant since 1970. The incidence of carcinoma of the thyroid gland tended to decrease, although it was high in the group exposed near the explosion. (Namekawa, K.)

  10. Upper lip malignant neoplasms. A study of 59 cases.

    Science.gov (United States)

    Luna-Ortiz, Kuauhyama; Güemes-Meza, Agustín; Villavicencio-Valencia, Verónica; Mosqueda-Taylor, Adalberto

    2012-05-01

    To present the demographic data, clinico-pathologic features and therapeutic outcome of a series of upper lip malignancies. Retrospective study at a single Cancer Institution in Mexico City during a 14-year period. There were 59 cases, (30 males and 29 females); age range: 14 to 106 years (mean: 73 yr.). Antecedents of ultraviolet light and tobacco exposure were found in 20 (33.9%) and 16 cases (27%) respectively. There were 35 squamous cell carcinomas (59.3%), 19 basal cell carcinomas (32.2%) and one case each (1.7%) of adenocarcinoma NOS, adenoid cystic carcinoma, angiosarcoma, Merkel cell carcinoma and melanoma. There were 14 cases in stage I (23.7%), 14 in stage II (23.7%), 3 in stage III (5.1%) 14 in stage IV (23.7%) and 14 were not classified (23.7%). There were no significant differences with respect to the overall survival curve and the disease-free survival curve among surgical treatment and radiotherapy. In addition, there was not statistically significant difference in the overall survival and disease-free survival among squamous cell carcinoma and basal cell carcinoma cases with respect to the type of treatment. Upper lip malignant neoplasms are infrequent lesions. The present series describes the main clinico-pathological features in a hospital-based population in Mexico city and demonstrates some differences with respect to those found in the lower lip.

  11. Salivary Gland Neoplasms: Does Morphological Diversity Reflect Tumor Heterogeneity?

    Science.gov (United States)

    Rito, Miguel; Fonseca, Isabel

    2017-09-21

    Salivary gland tumor classification encompasses a vast list of benign and malignant neoplasms. Their morphological diversity is recognized not only between different entities but also within individual tumors. Tumor categories as described by the World Health Organization reflect, in part, a true genetic heterogeneity (e.g., translocations involving CRTC1 and CRTC3-MAML2 genes in mucoepidermoid carcinoma and MYB-NFIB fusion in adenoid cystic carcinoma). Carcinoma ex pleomorphic adenoma shows diversity in its histological appearance, but recurrent rearrangements on PLAG1 and HMGA2 are common to its benign precursor. More recently, new categories have been defined, like secretory carcinoma with the t(12;15) (p13;q25) ETV6-NTRK3 translocation and clear-cell carcinoma with EWSR1-ATF1 fusion. Recent studies on cribriform adenocarcinoma of minor salivary gland origin and epithelial-myoepithelial carcinoma point to a correlation with their morphological features. All of these advances show that the search of a histogenetic and genetic basis for salivary gland tumors is helping to clarify morphological categories and unraveling new ones. Nevertheless, currently morphology is still the hallmark of tumor classification and the gold standard. The therapeutic options for advanced tumors remain very limited but the discovery of translocation-generated gene fusions and increased knowledge of the genomic information of salivary gland tumors is creating opportunities for the development of specific targeted therapies. © 2017 S. Karger AG, Basel.

  12. Mucinous cystic neoplasm of the pancreas in a male patient

    Directory of Open Access Journals (Sweden)

    Kazuhiro Yoshida

    2011-04-01

    Full Text Available Mucinous cystic neoplasms (MCNs make up a morphologic family of similar appearing tumors arising in the ovary and various extraovarian organs such as pancreas, hepatobiliary tract and mesentery. MCNs of the pancreas occur almost exclusively in women. Here, we report a rare case of MCN in a male patient. A 39-year-old man was admitted to our hospital with the chief complaint of back pain. Abdominal computed tomography revealed a multilocular cyctic mass 6.3 cm in diameter in the pancreatic tail. In addition, the outer wall and septae with calcification were demonstrated in the cystic lesion. On magnetic resonance imaging , the cystic fluid had low intensity on T1-weighted imaging and high intensity on T2-weighted imaging. Endoscopic retrograde cholangio-pancreatography (ERCP showed neither communication between the cystic lesion and the main pancreatic duct nor encasement of the main pancreatic duct. Endoscopic ultrasonography revealed neither solid component nor thickness of the septae in the cystic lesion. Consequently, we performed distal pancreatectomy with splenectomy under the diagnosis of cystic neoplasia of the pancreas. Histopathologically, the cystic lesion showed two distinct component: an inner epithelial layer and an outer densely cellular ovarian-type stromal layer. Based on these findings, the cystic lesion was diagnosed as MCN.

  13. Meningioma as second malignant neoplasm after oncological treatment during childhood

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, H.L.; Gebhardt, U. [Klinikum Oldenburg (Germany). Dept. of Pediatric Hematology and Oncology; Warmuth-Metz, M. [University Hospital Wuerzburg (Germany). Dept. of Neuroradiology; Pietsch, T. [Bonn Univ. (Germany). Dept. of Neuropathology; Soerensen, N. [Evangelisches Krankenhaus, Oldenburg (Germany). Dept. of Neurosurgery; Kortmann, R.D. [University Hospital Leipzig (Germany). Dept. of Radiooncology

    2012-05-15

    A total of 38 patients (18 female/20 male) with childhood meningioma were recruited from the German registry HIT-Endo (1989-2009). In 5 cases meningioma occurred as second malignant neoplasm (SMN). Histologies were confirmed by reference assessment in all cases (SMN: 2 WHO I, 1 WHO II, 2 WHO III). The SMNs were diagnosed at a median age of 12.4 years with a median latency of 10.2 years after primary malignancy (PMN; 4 brain tumors, 1 lymphoblastic leukemia; median age at diagnosis 2.7 years). Meningioma occurred as SMN in the irradiated field of PMN (range 12-54 Gy). The outcome after treatment of SMN meningioma (surgery/irradiation) was favorable in terms of psychosocial status and functional capacity in 4 of 5 patients (1 death). We conclude that survivors of childhood cancer who were exposed to radiation therapy at young age harbor the risk of developing meningioma as a SMN at a particularly short latency period in case of high dose exposure. (orig.)

  14. Pancreatic carcinosarcoma mimics malignant intraductal papillary mucinous neoplasm

    Science.gov (United States)

    Li, Bing-Qi; Liu, Qiao-Fei; Chang, Xiao-Yan; Hu, Ya; Chen, Jie; Guo, Jun-Chao

    2017-01-01

    Abstract Rationale: Carcinosarcoma, an extremely rare pancreatic primary tumor, is characterized by coexistence of both carcinomatous and sarcomatous components. Due to its rarity, the clinical manifestation and imaging features have not been recognized. An accurate diagnostic method has not been available and a widely accepted guidelines instructing treatment has not been established. Patient concerns: We present an uncommon case of pancreatic carcinosarcoma (PCS) which has been preoperatively diagnosed as pancreatic malignant intraductal papillary mucinous neoplasm. A radical resection, including total pancreatectomy (TP) and splenectomy, was performed. Diagnosis: The diagnosis of PCS was confirmed by postoperative pathology. Interventions: A radical resection, including TP and splenectomy, was performed. The patient was followed up by abdominal contrast-enhanced computed tomography scan and blood tumor marker examination. Outcomes: The patient is still alive and self-sufficient 7 months after the surgery. No evidence of tumor recurrence is found during follow-up. Lessons: Although, until recently, there are no widely accepted guidelines instructing treatment for PCS, a radical resection is still a possible way. All the pancreatic neoplastic patients with high surgical risk should be transferred to a specialized high-volume pancreatic center to get precise preoperative evaluation, fine operation technique, and careful postoperative management. PMID:28591030

  15. Parathyroid carcinoma: an unusual presentation of a rare neoplasm

    Directory of Open Access Journals (Sweden)

    Shruti, Sharma

    2017-12-01

    Full Text Available Parathyroid carcinoma is an extremely rare malignant endocrine neoplasm that is very challenging in its diagnosis as well as its treatment. Clinically the disease is detected earlier in patients who present with hyperparathyroidism with signs of profound hypercalcemia. Differentiation between benign and malignant disease of the parathyroid is challenging both for the clinician and for the pathologist. Complete surgical resection at the time of first operation offers the best chance of cure. Even after radical excision which is the standard management, local recurrence and metastases are frequent. The disease usually has a slow indolent course and most patients suffer from complications of hypercalcemia rather than tumor invasion or metastasis.We report a case of a 31-year-old woman who presented with renal colic. Various hematological, biochemical and radiological investigations were performed and a slightly enlarged right parathyroid was found. A clinical diagnosis of parathyroid adenoma was made and a right parathyroidectomy was done. Intraoperatively the surgeon had no suspicion of malignancy but microscopically the lesion was malignant and a final diagnosis of parathyroid carcinoma was rendered based on the criteria of invasion. Since there is no gold standard, a multidisciplinary approach, including the entire clinical, biochemical, radiological and pathological profile of the disease aids in an accurate diagnosis. Here we are reporting a case of a functional parathyroid carcinoma presenting in a relatively young patient with all the biochemical and radiological investigations and findings pointing towards a benign parathyroid disease.

  16. Mesothelial neoplasms presenting as, and mimicking, ovarian cancer.

    Science.gov (United States)

    Mani, Haresh; Merino, Maria J

    2010-11-01

    Mesotheliomas of the abdominal cavity are rare tumors that primarily involve the peritoneum, mesentery, and omentum. The involvement of the viscera is usually secondary to bulky and extensive serosal disease. We describe 7 cases of mesothelioma in which the initial manifestation was that of an ovarian mass. All patients underwent surgery with a primary diagnosis of ovarian cancer. Clinical histories, gross features, and histology slides were reviewed. Immunostains were performed on all cases and electron microscopy was performed in 2 cases. The patients ranged in age from 22 to 52 years and the lesions ranged in size from 3.8 to 9 cm. Of the 7 cases, 4 were predominantly cystic and 3 were solid. Histologically, all cystic tumors were multicystic mesothelioma, whereas the 3 solid tumors were diffuse malignant mesotheliomas. One patient had a borderline mucinous tumor with the mesothelioma occurring as a mural nodule, an association not described earlier. The oldest patient in this series had a diffuse malignant mesothelioma of the peritoneum with predominant ovarian surface involvement. Mesothelial neoplasms can present as ovarian masses in young women. Awareness of this presentation is important to establish appropriate management.

  17. Allergy and the risk of selected digestive and laryngeal neoplasms.

    Science.gov (United States)

    Bosetti, C; Talamini, R; Franceschi, S; Negri, E; Giacosa, A; La Vecchia, C

    2004-06-01

    The relation between allergy and cancer has been investigated within an integrated series of case-control studies of digestive tract and laryngeal neoplasms conducted in Italy since the early 1990s. These included 598 patients with incident, histologically confirmed cancer of the oral cavity and pharynx, 304 of the oesophagus, 1225 of the colon, 728 of the rectum, 460 of the larynx and 4999 controls, selected among patients admitted to the same network of hospitals as cases for acute, non-neoplastic diseases. Inverse associations with history of allergy were found for all cancer sites examined (odds ratio=0.44 for oral cavity and pharynx, 0.80 for oesophagus, 0.76 for colon, 0.54 for rectum and 0.33 for larynx). The associations were consistent in strata of age and sex, and when subjects with a first diagnosis of allergy 5 or more years before cancer diagnosis or hospital admission were considered. The present study therefore provides further evidence for a possible protective effect of prior history of allergy on cancer risk.

  18. Morphological clues to the appropriate recognition of hereditary renal neoplasms.

    Science.gov (United States)

    Moch, Holger; Ohashi, Riuko; Gandhi, Jatin S; Amin, Mahul B

    2018-02-14

    An important emerging role of the surgical pathologist besides the traditional tasks of establishment of the diagnosis and documentation of prognostic and predictive factors, is to recognize the possibility of a hereditary condition in cases where the histology is suggestive for a familial cancer syndrome. In recent years, the knowledge regarding all of the above roles, including the role of recognition of familial cancer, has particularly expanded in renal neoplasms with the close scrutiny to morphology, molecular correlates and clinical features of the different sub-types of renal cell carcinoma. Awareness of these clinically distinctive sub-types and their associated histologic clues will prompt the pathologist for further immunohistochemical or molecular work up, to look for clinical information to support the suspected diagnosis of familial cancer, to alert managing physician/s to look for stigmata of history of familial cancer, which will permit triaging patients and their families for appropriate genetic counseling. This review provides a comprehensive review of the known sub-types of renal cell carcinoma that have a predilection to occur in the setting of hereditary disease; examples include renal cancers occurring in the background of von Hippel Lindau disease, hereditary leiomyomatosis and renal cell carcinoma syndrome, tuberous sclerosis, Birt Hogg Dube syndrome and succinate dehydrogenase deficiency. Herein we focus on diagnostic clues for renal tumors occurring in a non-pediatric setting that should prompt their correct recognition and reiterate the importance of the correct diagnosis. Copyright © 2018 Elsevier Inc. All rights reserved.

  19. Analysis of errors during medical and computerized diagnostics of spherical lung neoplasms

    International Nuclear Information System (INIS)

    Pozmogov, A.I.; Petruk, D.A.

    1985-01-01

    Reasons for errors in medical and computerized diagnostics of spherical lung neoplasms are studied based on material of 212 case records and clinicoroentgenological data; it should promote improvement of their diagnostics

  20. Prevalence, Diagnosis and Management of Pancreatic Cystic Neoplasms: Current Status and Future Directions

    Science.gov (United States)

    Farrell, James J.

    2015-01-01

    Cystic neoplasms of the pancreas are found with increasing prevalence, especially in elderly asymptomatic individuals. Although the overall risk of malignancy is very low, the presence of these pancreatic cysts is associated with a large degree of anxiety and further medical investigation due to concerns about malignancy. This review discusses the different cystic neoplasms of the pancreas and reports diagnostic strategies based on clinical features and imaging data. Surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines, is also discussed, with special reference to intraductal papillary mucinous neoplasm (IPMN; particularly the branch duct variant), which is the lesion most frequently identified incidentally. IPMN pathology, its risk for development into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk are discussed. Finally, surgical treatment, strategies for surveillance of pancreatic cysts, and possible future directions are discussed. PMID:26343068

  1. An extremely rare neoplasm, histiocytic sarcoma: A report of two cases with an aggressive clinical course

    Directory of Open Access Journals (Sweden)

    Erkan Kayikcioglu

    2017-07-01

    Conclusions: HS is an extremely rare malignant neoplasm of the monocytic/macrophage lineage, with no standardized chemotherapy regimen for multisystemic disease. Metastatic patients have a more aggressive clinical course than those with unifocal disease.

  2. Microsatellite Status of Primary Colorectal Cancer Predicts the Incidence of Postoperative Colorectal Neoplasms.

    Science.gov (United States)

    Takiyama, Aki; Tanaka, Toshiaki; Yamamoto, Yoko; Hata, Keisuke; Ishihara, Soichiro; Nozawa, Hiroaki; Kawai, Kazushige; Kiyomatsu, Tomomichi; Nishikawa, Takeshi; Otani, Kensuke; Sasaki, Kazuhito; Watanabe, Toshiaki

    2017-10-01

    Few studies have evaluated the risk of postoperative colorectal neoplasms stratified by the nature of primary colorectal cancer (CRC). In this study, we revealed it on the basis of the microsatellite (MS) status of primary CRC. We retrospectively reviewed 338 patients with CRC and calculated the risk of neoplasms during postoperative surveillance colonoscopy in association with the MS status of primary CRC. A propensity score method was applied. We identified a higher incidence of metachronous rectal neoplasms after the resection of MS stable CRC than MS instable CRC (adjusted HR 5.74, p=0.04). We also observed a higher incidence of colorectal tubular adenoma in patients with MSS CRC (adjusted hazard ratio 7.09, pcolorectal cancer influenced the risk of postoperative colorectal neoplasms. Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  3. Diverticular disease of the vermiform appendix: a diagnostic clue to underlying appendiceal neoplasm.

    Science.gov (United States)

    Dupre, Marc P; Jadavji, Irfan; Matshes, Evan; Urbanski, Stefan J

    2008-12-01

    Acquired diverticula of the vermiform appendix are rare and arise as a result of different pathogenetic mechanisms. One of the etiologies includes proximally located, often unsuspected small neoplasms. Although the association of appendiceal diverticulosis and neoplasia is known, it remains underemphasized in the teaching and practice of surgical pathology. To investigate the frequency of appendiceal neoplasms with acquired diverticulosis, we conducted a retrospective analysis of all appendectomy specimens received in our institution for a 55-month period (January 2002-July 2006). A total of 1361 appendectomy specimens were identified. Diverticulosis was diagnosed in 23 (1.7%) of all cases. Eleven (48%) appendectomy specimens with diverticulosis also harbored an appendiceal neoplasm. The association of appendiceal neoplasms with diverticulosis was statistically significant (P appendix in this setting does not create a significant impact on the workload within the pathologic laboratory.

  4. Renal Function Outcomes for Multifocal Renal Neoplasms Managed by Radiofrequency Ablation

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Pushpender, E-mail: pugupta@wakehealth.edu; Allen, Brian C., E-mail: bcallen2@wakehealth.edu; Chen, Michael Y., E-mail: mchen@wakehealth.edu; Childs, David D., E-mail: dchilds@wakehealth.edu; Kota, Gopi, E-mail: gkota@wakehealth.edu; Zagoria, Ronald J., E-mail: rzagoria@wakehealth.edu [Wake Forest University School of Medicine, Department of Radiology (United States)

    2013-10-15

    Purpose: To evaluate renal function changes related to radiofrequency ablation (RFA) for the treatment of multifocal renal neoplasms. Methods: This is an institutional review board-approved, Health Insurance Portability and Accountability Act compliant retrospective study of all patients treated with computed tomography guided RFA for multifocal renal neoplasms at one institution. Fifty-seven subjects, mean age 70 (range 37-88) years, underwent RFA of 169 renal neoplasms (average size 2.0 cm). Subjects had between 2 and 8 (mean 2.96) neoplasms ablated. Estimated glomerular filtration rate (eGFR) was measured before and after RFA. Complications related to RFA were recorded. Results: eGFR decreased on average of 4.4 % per tumor treated and 6.7 % per ablation session (average 1.76 tumors treated per session). For subjects with the largest neoplasm measuring >3 cm, eGFR decreased an average of 14.5 % during the course of their treatment. If the largest neoplasm measured 2-3 cm, eGFR decreased an average of 7.7 %, and if the largest neoplasm measured <2 cm, eGFR decreased an average of 3.8 %. Subjects with reduced baseline renal function were more likely to have a greater decline in eGFR after RFA. There was a minor complication rate of 6.3 % (6 of 96 sessions), none of which required treatment, and a major complication rate of 4.2 % (4 of 96 sessions). Conclusion: RFA for the treatment of multifocal renal neoplasms results in mild decline of renal function.

  5. Branch duct-type intraductal papillary mucinous neoplasm presenting as paraneoplastic small plaque para-psoriasis

    OpenAIRE

    Cyriac A Philips; Philip Augustine; Lijesh Kumar; George Joseph; Pushpa Mahadevan

    2018-01-01

    To present and discuss a novel association between branch duct-type intraductal papillary mucinous neoplasm and paraneoplastic parapsoriasis. We present the case of a middle-aged male presenting with skin lesions that were suggestive of parapsoriasis, resistant to treatment, and in whom a diagnosis of branch-type intraductal papillary mucinous neoplasm of the pancreas was eventually made. A curative Whipple's surgery led to complete resolution of the skin lesions within 3 weeks. Paraneoplasti...

  6. Hemosiderin laden macrophages and hemosiderin within follicular cells distinguish benign follicular lesions from follicular neoplasms

    Directory of Open Access Journals (Sweden)

    Jaffar Reema

    2009-01-01

    Full Text Available Background: Published criteria to distinguish benign colloid nodules from follicular neoplasms emphasize only three interdependent features: size of follicles, amount of colloid, and cellularity. There is a need for the validation of other independent criteria. Methods: This study quantified the significance of cystic change, defined as presence of macrophages, and the presence of hemosiderin in either the macrophages or follicular cells. The cohort consisted of 165 patients with fine needle aspiration (FNA and histologic follow-up of either goiter (101, follicular adenoma (47, or follicular carcinoma (17. Papillary thyroid carcinomas and Hürthle cell neoplasms were excluded from the cohort, because these categories are known to show cystic change and hemosiderin. FNAs were reviewed blindly with the most cellular slide scored for the presence of macrophages and/or hemosiderin. Results: Hemosiderin within macrophages were seen in 67% (68 of 101 of the goiters and only 6% (four of 64 of follicular neoplasms ( P < .0001. All four follicular neoplasms with hemosiderin in macrophages were adenomas. Three of these four had equivocal features of a benign colloid nodule histologically. None of the 17 follicular carcinomas had hemosiderin in macrophages ( P < .12. Macrophages without hemosiderin also strongly distinguished goiters from neoplasms (83% vs 17% but appears less useful as a criterion since macrophages were present within 3 of 17 follicular carcinomas. Hemosiderin within follicular epithelial cells was present in 18% (18 of 101 of goiters, whereas none of the 64 follicular neoplasms had intraepithelial hemosiderin ( P < .0003. Conclusions: If papillary thyroid carcinoma and Hürthle cell neoplasm are ruled out, our findings indicate that the presence of hemosiderin virtually excludes a clinically significant follicular neoplasm.

  7. Familial Myelodysplastic/Acute Leukemia Syndromes—Myeloid Neoplasms with Germline Predisposition

    Directory of Open Access Journals (Sweden)

    Renata Lyrio Rafael Baptista

    2017-09-01

    Full Text Available Although most cases of myeloid neoplasms are sporadic, a small subset has been associated with germline mutations. The 2016 revision of the World Health Organization classification included these cases in a myeloid neoplasm group with a predisposing germline mutational background. These patients must have a different management and their families should get genetic counseling. Cases identification and outline of the major known syndromes characteristics will be discussed in this text.

  8. Familial Myelodysplastic/Acute Leukemia Syndromes—Myeloid Neoplasms with Germline Predisposition

    OpenAIRE

    Baptista, Renata Lyrio Rafael; dos Santos, Anna Cláudia Evangelista; Gutiyama, Luciana Mayumi; Solza, Cristiana; Zalcberg, Ilana Renault

    2017-01-01

    Although most cases of myeloid neoplasms are sporadic, a small subset has been associated with germline mutations. The 2016 revision of the World Health Organization classification included these cases in a myeloid neoplasm group with a predisposing germline mutational background. These patients must have a different management and their families should get genetic counseling. Cases identification and outline of the major known syndromes characteristics will be discussed in this text.

  9. Hemosiderin laden macrophages and hemosiderin within follicular cells distinguish benign follicular lesions from follicular neoplasms.

    Science.gov (United States)

    Jaffar, Reema; Mohanty, Sambit K; Khan, Ashraf; Fischer, Andrew H

    2009-01-19

    Published criteria to distinguish benign colloid nodules from follicular neoplasms emphasize only three interdependent features: size of follicles, amount of colloid, and cellularity. There is a need for the validation of other independent criteria. This study quantified the significance of cystic change, defined as presence of macrophages, and the presence of hemosiderin in either the macrophages or follicular cells. The cohort consisted of 165 patients with fine needle aspiration (FNA) and histologic follow-up of either goiter (101), follicular adenoma (47), or follicular carcinoma (17). Papillary thyroid carcinomas and Hürthle cell neoplasms were excluded from the cohort, because these categories are known to show cystic change and hemosiderin. FNAs were reviewed blindly with the most cellular slide scored for the presence of macrophages and/or hemosiderin. Hemosiderin within macrophages were seen in 67% (68 of 101) of the goiters and only 6% (four of 64) of follicular neoplasms (Phemosiderin in macrophages were adenomas. Three of these four had equivocal features of a benign colloid nodule histologically. None of the 17 follicular carcinomas had hemosiderin in macrophages (Phemosiderin also strongly distinguished goiters from neoplasms (83% vs 17%) but appears less useful as a criterion since macrophages were present within 3 of 17 follicular carcinomas. Hemosiderin within follicular epithelial cells was present in 18% (18 of 101) of goiters, whereas none of the 64 follicular neoplasms had intraepithelial hemosiderin (Phemosiderin virtually excludes a clinically significant follicular neoplasm.

  10. Malignant nerve-sheath neoplasms in neurofibromatosis: distinction from benign tumors by using imaging techniques

    International Nuclear Information System (INIS)

    Levine, E.; Huntrakoon, M.; Wetzel, L.H.

    1987-01-01

    Malignant peripheral nerve-sheath neoplasms frequently complicate neurofibromatosis causing pain, enlarging masses, or neurologic deficits. However, similar findings sometimes also occur with benign nerve neoplasms. Our study was done retrospectively to determine if imaging techniques can differentiate malignant from benign nerve tumors in neurofibromatosis. Eight patients with symptomatic neoplasms (three benign, five malignant) were studied by CT in eight, MR in six, and 67 Ga-citrate scintigraphy in seven. Uptake of 67 Ga occurred in all five malignant lesions but not in two benign neoplasms studied. On CT or MR, all eight lesions, including three benign neoplasms, showed inhomogeneities. Of five lesions with irregular, infiltrative margins on CT or MR, four were malignant and one was benign. Of three lesions with smooth margins, one was malignant and two were benign. One malignant neoplasm caused irregular bone destruction. Accordingly, CT and MR could not generally distinguish malignant from benign lesions with certainty. However, both CT and MR provided structural delineation to help surgical planning for both types of lesion. 67 Ga scintigraphy appears promising as a screening technique to identify lesions with malignant degeneration in patients with neurofibromatosis. Any area of abnormal radiogallium uptake suggests malignancy warranting further evaluation by CT or MR. Biopsy of any questionable lesion is essential

  11. Alemtuzumab and Glucocorticoids in Treating Newly Diagnosed Acute Graft-Versus-Host Disease in Patients Who Have Undergone a Donor Stem Cell Transplant

    Science.gov (United States)

    2010-05-12

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Graft Versus Host Disease; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Diseases; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  12. The Clinical Significance of IDH Mutations in Essential Thrombocythemia and Primary Myelofibrosis

    OpenAIRE

    Yonal-Hindilerden, Ipek; Daglar-Aday, Aynur; Hindilerden, Fehmi; Akadam-Teker, Basak; Yilmaz, Ceylan; Nalcaci, Meliha; Yavuz, Akif Selim; Sargin, Deniz

    2015-01-01

    Background Limited data exist regarding impact of IDH mutations in Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPNs). Prognostic significance of IDH mutations was asessed in 184 Ph-negative MPN patients - 107 essential thrombocythemia (ET) and 77 primary myelofibrosis (PMF). Methods High-resolution melting (HRM) analysis was used to detect IDH1 and IDH2 mutations. Results PMF and ET patients showed no significant difference for prevalence of IDH mutations. Mutant IDH (IDH1...

  13. Thermal and epithermal neutrons in the treatment of neoplasms

    International Nuclear Information System (INIS)

    Sweet, W.H.; Wellum, G.; Tolpin, E.; Messer, J.R.

    1975-01-01

    Progress is reported in the preparation and testing of boron compounds for use as targets in the neutron capture therapy of neoplasms. The synthesis and structural confirmation of the disulfide, B 1 H 11 SSB 12 H 11 -4 is reported as well as a new structural model for its mono-oxidized derivative, B 12 H 11 SOSB 12 H 11 -4 , which showed useful tumor:blood concentration ratios in rats after three I. V. injections. The material, B 12 H 11 (SH) 2 -2 , was isolated and identified from impure Cs 2 B 12 H 11 SH. The former anion was shown to be responsible, in its oxidized form, for the excellent tumor uptake results shown by impure B 12 H 11 SH -2 in the past. As a consequence of this work, a borane polymer was synthesized which has shown extremely encouraging tumor uptake properties. The synthesis of this polymer depended on our production and characterization of the first stable thiyl radical at room temperature, B 12 H 11 -S: -2 . The retesting of certain phosphate boranes is reported. And a reagent, namely acetyl sulfenyl chloride, lSCOCH 3 , has been found which will produce B 12 H 11 SH -2 in a laboratory synthesis. The ability of sulfur derivatives of B 12 H 11 SH -2 to bind to proteins was examined, as was the blood distribution of two of these interesting boranes. The amount of non-protein bound borane was related to the rate of blood clearance of the borane in rats. Progress is also reported on dog irradiation experiments. (U.S.)

  14. Second Malignant Neoplasms and Cardiovascular Disease Following Radiotherapy

    Science.gov (United States)

    Ng, Andrea K.; Allan, James M.; Pui, Ching-Hon; Kennedy, Ann R.; Xu, X. George; Purdy, James A.; Applegate, Kimberly; Yahalom, Joachim; Constine, Louis S.; Gilbert, Ethel S.; Boice, John D.

    2012-01-01

    Second malignant neoplasms (SMNs) and cardiovascular disease (CVD) are among the most serious and life-threatening late adverse effects experienced by the growing number of cancer survivors worldwide and are due in part to radiotherapy. The National Council on Radiation Protection and Measurements (NCRP) convened an expert scientific committee to critically and comprehensively review associations between radiotherapy and SMNs and CVD, taking into account radiobiology; genomics; treatment (ie, radiotherapy with or without chemotherapy and other therapies); type of radiation; and quantitative considerations (ie, dose–response relationships). Major conclusions of the NCRP include: 1) the relevance of older technologies for current risk assessment when organ-specific absorbed dose and the appropriate relative biological effectiveness are taken into account and 2) the identification of critical research needs with regard to newer radiation modalities, dose–response relationships, and genetic susceptibility. Recommendation for research priorities and infrastructural requirements include 1) long-term large-scale follow-up of extant cancer survivors and prospectively treated patients to characterize risks of SMNs and CVD in terms of radiation dose and type; 2) biological sample collection to integrate epidemiological studies with molecular and genetic evaluations; 3) investigation of interactions between radiotherapy and other potential confounding factors, such as age, sex, race, tobacco and alcohol use, dietary intake, energy balance, and other cofactors, as well as genetic susceptibility; 4) focusing on adolescent and young adult cancer survivors, given the sparse research in this population; and 5) construction of comprehensive risk prediction models for SMNs and CVD to permit the development of follow-up guidelines and prevention and intervention strategies. PMID:22312134

  15. Endocrine control of canine mammary neoplasms: serum reproductive hormone levels and tissue expression of steroid hormone, prolactin and growth hormone receptors.

    Science.gov (United States)

    Spoerri, Michèle; Guscetti, Franco; Hartnack, Sonja; Boos, Alois; Oei, Christine; Balogh, Orsolya; Nowaczyk, Renata M; Michel, Erika; Reichler, Iris M; Kowalewski, Mariusz P

    2015-09-15

    Neoplasms of the mammary gland are among the most common diseases in female domestic dogs (Canis familiaris). It is assumed that reproductive hormones influence tumorigenesis in this species, although the precise role of the endocrine milieu and reproductive state is subject to continuing discussion. In line with this, a recent systematic review of available data on the development of mammary neoplasms revealed weak evidence for risk reduction after neutering and an effect of age at neutering. Investigation of several hormone receptors has revealed decreased expression of estrogen receptor-alpha (ERα, ESR1), progesterone (P4) receptor (PGR), prolactin (PRL) receptor (PRLR) and growth hormone receptor (GHR) associated with neoplastic differentiation of mammary tissues. In other studies, increased levels of estrogens, progesterone and prolactin were found in serum and/or tissue homogenates of dogs with malignant neoplasms. However, the association between these entities within one animal population was never previously examined. Therefore, this study investigated the association between circulating serum concentrations of estradiol-17β, progesterone and prolactin, and gene expression of ERα (ESR1), ERβ (ESR2), PGR, PRLR, PRL and GHR, with respect to reproductive state (spayed vs. intact) and cycle stage (anestrus vs. diestrus). Additionally, the expression of E-cadherin (CDH-1) was evaluated as a possible indicator of metastatic potential. For all receptors, the lowest gene expression was found in malignant tumors compared to normal tissues of affected dogs. Steroid levels were not influenced by their corresponding receptor expression in mammary neoplasms, but increased PRL levels were negatively associated with low PRLR gene expression in malignant tumors. The expression of CDH-1 was influenced by tumor malignancy and cycle stage, i.e., the highest gene expression was found in benign mammary tumors in diestrous dogs compared to normal and malignant mammary

  16. A new combined criterion to better predict malignant lesions in patients with pancreatic cystic neoplasms

    Science.gov (United States)

    Lan, Chungen; Li, Xin; Wang, Xiuchao; Hao, Jihui; Ren, He

    2018-01-01

    Objective: Cystic lesions of the pancreas have been increasingly recognized. Some lesions exhibit benign behavior, while others have unequivocal malignant potential. Thus, accurate identification of malignancy in patients diagnosed with pancreatic cystic neoplasms (PCNs) remains a major challenge. The aim of this study was to define a combined criterion to better predict malignant lesions in patients with PCNs. Methods: We retrospectively analyzed 165 patients who underwent resection of PCNs from October 2011 to May 2017. The relationship among malignancy and serum carbohydrate antigen 19-9 (CA19-9), preoperative neutrophil-to-lymphocyte ratio (NLR), and the presence of enhanced solid component on imaging was analyzed. Results: NLR before surgery in patients with malignant PCNs (2.81±2.14) was significantly higher than that in patients diagnosed with pancreatic neuroendocrine tumor (1.90±0.69, P=0.013) or healthy volunteers (1.40±0.48; P1.976 and presence of enhanced solid component were independent predictors of PCN malignancy. A combined criterion meeting any two or more of the three elements including CA19-9 ≥39 U/mL, NLR >1.976, and presence of enhanced solid component on computed tomography imaging is an indicator with a high positive predictive value of 80.5% and a high negative predictive value of 87.9%, and thus, represents a highly accurate test (86.1%). Conclusions: The new combined criterion is an effective predictor of tumor malignancy in patients with PCNs. PMID:29545970

  17. Progesterone arrested cell cycle progression through progesterone receptor isoform A in pancreatic neuroendocrine neoplasm.

    Science.gov (United States)

    Yazdani, Samaneh; Kasajima, Atsuko; Onodera, Yoshiaki; McNamara, Keely May; Ise, Kazue; Nakamura, Yasuhiro; Tachibana, Tomoyoshi; Motoi, Fuyuhiko; Unno, Michiaki; Sasano, Hironobu

    2018-04-01

    In pancreatic neuroendocrine neoplasms (Pan-NEN) progesterone signaling has been shown to have both inhibitory and stimulatory effects on cell proliferation. The ability of progesterone to inhibit tumor proliferation is of particular interest and is suggested to be mediated through the less abundantly expressed progesterone receptor (PR) isoform A (PRA). To date the mechanistic processes underlying this inhibition of proliferation remain unclear. To examine the mechanism of PRA actions, the human Pan-NEN cell line QGP-1, that endogenously expresses PR isoform B (PRB) without PRA, was transfected with PRA. PRA transfection suppressed the majority of cell cycle related genes increased by progesterone including cyclin A2 (CCNA2), cyclin B1 (CCNB1), cyclin-dependent kinase 1 (CDK1) and cyclin-dependent kinase 2 (CDK2). Importantly, following progesterone administration cell cycle distribution was shifted to S and G2/M phases in the naïve cell line but in PRA-transfected cells, this effect was suppressed. To see if these mechanistic insights were confirmed in patient samples PRA, PRB, CCNA2, CCNB, CDK1 and CDK2 immunoreactivities were assessed in Pan-NEN cases. Higher levels of cell cycle markers were associated with higher WHO grade tumors and correlations between the markers suggested formation of cyclin/CDK activated complexes in S and G2/M phases. PRA expression was associated with inverse correlation of all cell cycle markers. Collectively, these results indicate that progesterone signals through PRA negatively regulates cell cycle progression through suppressing S and G2/M phases and downregulation of cell cycle phases specific cyclins/CDKs. Copyright © 2018 Elsevier Ltd. All rights reserved.

  18. Thread sign in biliary intraductal papillary mucinous neoplasm: a novel specific finding for MRI

    International Nuclear Information System (INIS)

    Hong, Gil-Sun; Byun, Jae Ho; Kim, Jin Hee; Kim, Hyoung Jung; Lee, Seung Soo; Lee, Moon-Gyu; Hong, Seung-Mo

    2016-01-01

    To evaluate thread sign of biliary intraductal papillary mucinous neoplasm (B-IPMN) on magnetic resonance imaging (MRI). Thread sign was defined as intraductal linear or curvilinear hypointense striations. Two radiologists independently evaluated the presence and location of thread sign on MR cholangiography (thin-slice, thick-slab and 3D MRC) and axial MR images (T2 TSE, T2 HASTE and DWI) in patients with B-IPMN (n = 38) and in matched control groups with benign (n = 36) or malignant (n = 35) biliary diseases. Sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV) of thread sign for diagnosing B-IPMN were evaluated. Thread sign was observed only in patients with B-IPMN on MRC (44.7-52.6 % [17/38-20/38], P < 0.001) and axial MR images (31.6 % [12/38], P < 0.001), except in one patient with recurrent pyogenic cholangitis on MRC (2.8 %, 1/36). The sensitivity, specificity, accuracy, PPV and NPV of thread sign for diagnosing B-IPMN on MRC were 0.53, 0.99, 0.83, 0.95 and 0.80, respectively (reader 1) and 0.45, 1.0, 0.81, 1.0 and 0.77, respectively (reader 2). Thread sign was detected mainly at the extrahepatic bile duct (52.6 %, 20/38). B-IPMN can manifest thread sign, a novel specific MR finding, mainly at the extrahepatic bile duct on MRI, especially on MRC. (orig.)

  19. The impact of thyroid nodule size on the risk of malignancy in follicular neoplasms.

    Science.gov (United States)

    Ibrahim, Yasin; Mohamed, Salah Eldin H; Deniwar, Ahmed; Al-Qurayshi, Zaid H; Khan, Amna N; Moroz, Krzysztof; Friedlander, Paul; Kandil, Emad

    2015-03-01

    Studies have shown that the risk of malignancy in follicular neoplasms is as high as 30%. Often, surgery is recommended for such lesions, not for therapeutic purposes but as a diagnostic method, leading to increased hospital costs and related morbidities. Recent studies have suggested that tumor size predicts malignant potential of these follicular neoplasms. Our aim was to identify the impact of nodule size on the risk of malignancy for such lesions. A retrospective medical chart review was undertaken for patients who underwent thyroid surgery at a single academic North American Institution. A total of 120 follicular lesions, follicular neoplasms (Bethesda category IV) or follicular lesions of undetermined significance (Bethesda category III) in 110 patients undergoing thyroid surgery were evaluated. Nodule size as measured by ultrasound, fine-needle aspiration cytological results, and final histopathology reports were reviewed. Analysis was performed by classification according to nodule size: malignant on final pathological examination. The malignancy rate in nodulesmalignancy rate for follicular neoplasms. Hence, we recommend against routine total thyroidectomy for patients with follicular neoplasms based on the size criteria. Copyright© 2015 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  20. Factors associated with phyllodes tumor of the breast after core needle biopsy identifies fibroepithelial neoplasm.

    Science.gov (United States)

    Gould, Daniel J; Salmans, Jessica A; Lassinger, Brian K; Contreras, Alejandro; Gutierrez, Carolina; Bonefas, Elizabeth; Liscum, Kathleen R; Silberfein, Eric J

    2012-11-01

    Phyllodes tumors represent less than 1% of all breast neoplasms and can mimic fibroadenoma on core needle biopsy (CNB). The treatment of fibroepithelial (FE) neoplasms identified on CNB is controversial. We sought to identify factors that were associated with phyllodes tumors after CNB suggested FE neoplasm. A retrospective database was queried for all patients diagnosed with FE neoplasm on CNB at Ben Taub General Hospital over a 10-y period. One hundred twenty-three patients were identified and demographic, clinical, and outcome data were analyzed. Of the 123 patients, 46 (37%) were found to have fibroadenomatous features and 59 (48%) were found to have FE features. All went on to have surgical excision. Forty (38%) contained phyllodes tumors, and 65 (62%) found no phyllodes tumor on final pathology. There were significant differences in the median size of the masses (4 cm versus 2.4 cm P phyllodes tumors and the group that did not on preoperative imaging. Further evaluation did not show any significant differences on preoperative imaging between benign and borderline/malignant phyllodes tumors. Hispanic ethnicity correlated with a higher chance of phyllodes tumor after CNB (P phyllodes tumor, surgical excision remains the standard of care; however, patients with suspicious FE neoplasms represent a treatment dilemma as many will prove to be benign. Preoperative size and the density of the mass on imaging and ethnicity were associated with phyllodes tumors on final pathology. Copyright © 2012 Elsevier Inc. All rights reserved.

  1. Simultaneous Occurrence of Different Follicular Neoplasms within the Same Thyroid Gland

    Directory of Open Access Journals (Sweden)

    Şefika Burçak Polat

    2016-06-01

    Full Text Available Purpose: Neoplasms of the thyroid gland are classified according to the cells they originate from and commonly develop from cells of follicular origin. The most common differentiated thyroid cancers (DTC are papillary and follicular carcinomas. Coexistence of two different histological types of primary follicular thyroid neoplasm is a rare condition. There are previous reports of concomitant medullary and papillary thyroid cancers. However, there is scarce data about the simultaneous occurrence of the two different histological types of primary follicular thyroid tumors and this is the first study on that subject. Material and Method: From January 2007 to September 2014, our institutional database was reviewed for patients who underwent thyroid surgery for various indications. Medical records and cytopathology reports of those patients were examined retrospectively. Simultaneous neoplasms of follicular origin were noted. Results: A total of 3.700 patients were operated. Histopathological examination revealed a benign pattern in 2.686 (73% patients and a malignant pattern in 1.014 (27% patients. Among the patients with the diagnosis of DTC, only 20 (1.9% had a concomitant neoplasm within the same thyroid gland. Discussion: Such simultaneous tumors may be a part of a familial tumor syndrome or an unidentified novel gene mutation playing role in the pathogenesis of more than one type of tumor. Based on the current evidence, the synchronous occurrence of those neoplasms in a given patient is likely coincidental in the literature. Further studies on larger patient population with standardized genetic characterization are needed.

  2. Low grade Schwann cell neoplasms with leptomeningeal dissemination: clinicopathologic and autopsy findings

    Science.gov (United States)

    Rodriguez, Erika F.; Blakeley, Jaishri; Langmead, Shannon; Olivi, Alessandro; Tufaro, Anthony; Tabbarah, Abeer; Berkenblit, Gail; Sacks, Justin M.; Newsome, Scott D.; Montgomery, Elizabeth; Rodriguez, Fausto J.

    2016-01-01

    Leptomeningeal dissemination of low grade Schwann cell neoplasms is an exceptionally rare occurrence, and has not been well documented in the literature. We encountered two cases of leptomeningeal dissemination of low grade Schwann cell neoplasms. Patient 1 was a 63-year-old woman with NF1 and a progressive low grade MPNST developing from a diffuse/plexiform orbital neurofibroma that arose in childhood. The neoplasm demonstrated local and leptomeningeal dissemination intracranially leading to the patient’s death. There was partial loss of H3K27 tri-methylation, p16 and collagen IV. Patient 2 was a 60-year-old man without NF1 who presented with cranial nerve symptoms and a disseminated neoplasm with a Schwann cell phenotype. The neoplasm stabilized after irradiation and chemotherapy but the patient died of medical complications. Autopsy findings documented disseminated leptomeningeal disease in the intracranial and spinal compartment. H3K27M tri-methylation was preserved. The clinicopathologic and autopsy findings are studied and presented, and the literature is reviewed. PMID:27666764

  3. Helicobacter pylori infection is an independent risk factor of early and advanced colorectal neoplasm.

    Science.gov (United States)

    Kim, Tae Jun; Kim, Eun Ran; Chang, Dong Kyung; Kim, Young-Ho; Baek, Sun-Young; Kim, Kyunga; Hong, Sung Noh

    2017-06-01

    The role of Helicobacter pylori (H. pylori) in the development of colorectal neoplasm remains controversial. We examined the association between H. pylori infection and colorectal neoplasm in a large sample of healthy participants who underwent screening colonoscopy. A cross-sectional study of 8916 men, who participated in a regular health-screening examination that included an H. pylori-specific immunoglobulin G antibody test and colonoscopy, was conducted to evaluate the association between H. pylori and colorectal neoplasm. Multivariable analyses adjusted for age, body mass index, smoking status, alcohol intake, regular exercise, regular aspirin use, and family history of colorectal cancer showed that the odds ratio (OR) (95% confidence interval [CI]) for any adenoma and advanced neoplasm was 1.32 (1.07-1.61) and 1.90 (1.05-3.56) in participants with H. pylori infection and without H. pylori infection, respectively. The association persisted after further adjustment for inflammatory markers or metabolic variables including fasting blood glucose, triglycerides, high-density lipoprotein-cholesterol, and low-density lipoprotein-cholesterol. Regarding the location, a positive association was confined to cases with proximal adenomas and was observed similarly in all the evaluated subgroups. In a large-scale study, carefully controlled for confounding factors, involving asymptomatic participants without a history of colonoscopy, H. pylori infection was significantly associated with the risk of any colorectal adenoma and advanced colorectal neoplasm. Prospective studies are necessary to determine whether H. pylori eradication can reduce this risk. © 2017 John Wiley & Sons Ltd.

  4. Classification tree analysis of second neoplasms in survivors of childhood cancer

    International Nuclear Information System (INIS)

    Jazbec, Janez; Todorovski, Ljupčo; Jereb, Berta

    2007-01-01

    Reports on childhood cancer survivors estimated cumulative probability of developing secondary neoplasms vary from 3,3% to 25% at 25 years from diagnosis, and the risk of developing another cancer to several times greater than in the general population. In our retrospective study, we have used the classification tree multivariate method on a group of 849 first cancer survivors, to identify childhood cancer patients with the greatest risk for development of secondary neoplasms. In observed group of patients, 34 develop secondary neoplasm after treatment of primary cancer. Analysis of parameters present at the treatment of first cancer, exposed two groups of patients at the special risk for secondary neoplasm. First are female patients treated for Hodgkin's disease at the age between 10 and 15 years, whose treatment included radiotherapy. Second group at special risk were male patients with acute lymphoblastic leukemia who were treated at the age between 4,6 and 6,6 years of age. The risk groups identified in our study are similar to the results of studies that used more conventional approaches. Usefulness of our approach in study of occurrence of second neoplasms should be confirmed in larger sample study, but user friendly presentation of results makes it attractive for further studies

  5. The synchronous prevalence of colorectal neoplasms in patients with stomach cancer.

    Science.gov (United States)

    Lee, Sang Su; Jung, Woon Tae; Kim, Cha Young; Ha, Chang Yoon; Min, Hyun Ju; Kim, Hyun Jin; Kim, Tae Hyo

    2011-10-01

    The association between stomach cancer and colorectal cancer is controversial. The purpose of this study was to determine the synchronous prevalence of colorectal neoplasms in patients with stomach cancer. A total of 123 patients with stomach cancer (86 male) and 246 consecutive, age- and sex-matched persons without stomach cancer were analyzed from July 2005 to June 2010. All of them underwent colonoscopy within 6 months after undergoing gastroscopy. The prevalence of colorectal neoplasms was significantly higher in the stomach cancer group (35.8%) than in the control group (17.9%) (P stomach cancer (odds ratio [OR], 3.10; 95% confidence interval [CI], 1.71 to 5.63). In particular, the difference in the prevalence of colorectal neoplasms was more prominent in the patients above 50 years old (OR, 3.54; 95% CI, 1.80 to 6.98). The results showed that the synchronous prevalence of colorectal neoplasms was higher in patients with stomach cancer than in those without stomach cancer. Therefore, patients with stomach cancer should be regarded as a high-risk group for colorectal neoplasms, and colonoscopy should be recommended for screening.

  6. Significance of acquired diverticular disease of the vermiform appendix: a marker of regional neoplasms?

    Science.gov (United States)

    Kallenbach, Klaus; Hjorth, Sofie Vetli; Engel, Ulla; Schlesinger, Nis Hallundbæk; Holck, Susanne

    2012-07-01

    To assess the prevalence of acquired diverticulum of the appendix (DA), including incipient forms and its possible significance as a marker of local/regional neoplasms. The pathology database at Hvidovre Hospital was searched for appendix specimens, received between 2001 and 2010, coded for DA or for a space-occupying lesion. Slides were reviewed to determine DA status and the nature of lesions possibly causing DA. Among 4413 appendix specimens, DA were identified in 39 (0.9%, CI 0.6% to 1.2%) cases, 17 (43.6%, 28.0% to 59.2%) of which additionally harboured an appendiceal neoplasm/neoplastic precursor, whereas this figure was 1.2% (CI 0.9% to 1.6%) for non-DA specimens (p<0.0001). Six of the 39 DA specimens comprised incipient DA, three of which coexisted with appendiceal neoplasms. In addition, local/regional non-neoplastic lesions (six cases) and colorectal carcinomas (four cases) coexisted with DA. DA has significance as a putative marker of local/regional neoplasms. Therefore, a DA specimen proved significantly more likely to harbour a neoplastic growth than a non-DA counterpart. Submission for microscopy of the entire DA specimen, whether transmural or only incipient, and a comment in the pathology report on the occasional concurrence of local/regional neoplasms in this setting seem appropriate. The observation of DA may thus provide a valuable contribution in the diagnostic process.

  7. The 2016 revision of the World Health Organization classification of lymphoid neoplasms

    Science.gov (United States)

    Campo, Elias; Pileri, Stefano A.; Harris, Nancy Lee; Stein, Harald; Siebert, Reiner; Advani, Ranjana; Ghielmini, Michele; Salles, Gilles A.; Zelenetz, Andrew D.; Jaffe, Elaine S.

    2016-01-01

    A revision of the nearly 8-year-old World Health Organization classification of the lymphoid neoplasms and the accompanying monograph is being published. It reflects a consensus among hematopathologists, geneticists, and clinicians regarding both updates to current entities as well as the addition of a limited number of new provisional entities. The revision clarifies the diagnosis and management of lesions at the very early stages of lymphomagenesis, refines the diagnostic criteria for some entities, details the expanding genetic/molecular landscape of numerous lymphoid neoplasms and their clinical correlates, and refers to investigations leading to more targeted therapeutic strategies. The major changes are reviewed with an emphasis on the most important advances in our understanding that impact our diagnostic approach, clinical expectations, and therapeutic strategies for the lymphoid neoplasms. PMID:26980727

  8. Serous Cystadenoma of the Pancreas Presenting as a Third Primary Neoplasm

    Directory of Open Access Journals (Sweden)

    Aydın Şeref Köksal

    2003-01-01

    Full Text Available Serous cystadenomas are the most common cystic neoplasms of the pancreas. They may occur solely or coexist with other neoplasms. A 10 cm mass involving the body of the pancreas was observed in the computed tomography of a 61-year-old man with a previous history of bladder and prostate carcinoma. Ultrasonography and computed tomography of the mass demonstrated multiple small cysts associated with a central calcified scar. A distal pancreatectomy was performed. Pathological examination confirmed the diagnosis of serous microcystic adenoma. This is the first report of a serous cystadenoma of the pancreas with two metachronous neoplasms. This feature should be kept in mind during the diagnosis and evaluation of patients with serous cystadenoma.

  9. Neoplasms of the apocrine sweat glands in 44 dogs and 10 cats.

    Science.gov (United States)

    Kalaher, K M; Anderson, W I; Scott, D W

    1990-10-20

    Neoplasms of the apocrine sweat glands accounted for 2.0 per cent and 3.6 per cent, respectively, of all canine and feline skin neoplasms diagnosed during a period of three years. They occurred in dogs from six to 17 years of age of both sexes, and golden retrievers appeared to be predisposed; they occurred in cats from six to 17 years of age and there were no breed or sex predilections. In both species, the neoplasms were usually solitary and occurred anywhere on the body; they were nearly always carcinomas and histologically were usually of the solid type. There were no clinical measurements that made it possible to distinguish reliably between benign and malignant lesions. No distant metastases were recorded, even though 22.5 per cent of the canine carcinomas had invaded the lymphatic system.

  10. [Mucinous neoplasms of the vermiform appendix, Pseudomyxoma peritonei, and the new WHO classification].

    Science.gov (United States)

    Reu, S; Neumann, J; Kirchner, T

    2012-02-01

    Mucinous neoplasms of the appendix are rare tumors, some of them characterized by an enigmatic discrepancy between a benign morphologic appearance and an aggressive biologic potential, associated with a poor prognosis and high mortality. The clinical picture of pseudomyxoma peritonei is, with few exceptions, caused by mucinous appendiceal neoplasms and differs in many aspects from usual peritoneal carcinomatosis. The controversy regarding terminology, diagnostic criteria, classification and therapy of these tumors has lasted for decades. The revised edition of the World Health Organization Classification of Tumors of the Digestive System proposes a uniform reporting system for mucinous appendiceal neoplasms and the peritoneal disease associated with it, thereby creating a comparable basis for pathological diagnosis, clinical therapy and further scientific studies.

  11. A STUDY OF P53 EXPRESSION IN UROTHELIAL NEOPLASMS OF URINARY BLADDER

    Directory of Open Access Journals (Sweden)

    G. Sathish Kumar

    2017-07-01

    Full Text Available BACKGROUND Urothelial Cell Carcinoma (UCC of urinary bladder is the seventh commonest cancer wordwide.1 At initial diagnosis, 30% of UCC display solid and invasive growth patterns and are locally advanced or metastatic at the time of diagnosis. 70% of tumours are noninvasive papillary UCC confined to the epithelium and subepithelial connective tissue,2 which can be managed by endoscopic resection. A significant number of post-resected cases, progress for recurrence of tumour and infiltration to muscle layers. Invasive bladder cancer has high morbidity and uniform mortality when it is metastatic. There are no effective tools to predict aggressiveness of tumour, so that these cases can be managed more successfully. Mutated Tp53/p53 is the genetic abnormality most frequently associated with UCC and related to cell transformation, malignancy and high recurrence rates.2 MATERIALS AND METHODS This is a descriptive study conducted in the departments of urology and pathology and during the period of March 2014 to February 2015. All consecutive cystoscopic biopsies, Trans urethral resection of bladder tumour (TURBT and radical cystectomy specimens histopathologically diagnosed as UCC were included in the study. p53 expression was assessed by immunohistochemistry. Positive and negative controls were used. Bivariate analysis was done using Chi-square test in all cases. RESULTS A total of 80 cases were analysed. Significant association of p53 expression was found in higher grades of tumour. Also, noted relation of p53 mutation with tumour size, multifocality, multiplicity, muscle invasion and tumour stage, which were statistically not significant. CONCLUSION Bladder tumour grade shows significant association to p53 expression. Papillary neoplasm of low malignant potential (PUNLMP tumours are negative for p53, and in the present study, there was significant difference in p53 over expression low-grade papillary UCC compared with PUNLMP. 90% of low

  12. Dietary patterns and risk of advanced colorectal neoplasms: A large population based screening study in Germany.

    Science.gov (United States)

    Erben, Vanessa; Carr, Prudence R; Holleczek, Bernd; Stegmaier, Christa; Hoffmeister, Michael; Brenner, Hermann

    2018-06-01

    Specific components of the diet such as red and processed meat have been associated with the risk of developing colorectal cancer. However, evidence on the association of dietary patterns with colorectal neoplasms is sparse. The aim of this study was to analyze the association of dietary patterns with prevalence of advanced colorectal neoplasms among older adults in Germany. A cross-sectional study was conducted among participants of screening colonoscopy in Saarland, Germany, who were enrolled in the KolosSal study (Effektivität der Früherkennungs-Koloskopie: eine Saarland-weite Studie) from 2005 to 2013. Information on diet and lifestyle factors was obtained through questionnaires and colonoscopy results were extracted from physicians' reports. Associations of a priori defined dietary patterns (vegetarian or adapted versions of the Healthy Eating Index [HEI] and the Dietary Approaches to Stop Hypertension [DASH] index) with the risk of advanced colorectal neoplasms were assessed by multiple logistic regression analyses with comprehensive adjustment for potential confounders. A total of 14,309 participants were included (1561 with advanced colorectal neoplasms). Healthier eating behavior was associated with lower prevalence of advanced colorectal neoplasms in a dose-response manner. Adjusted odds ratios (95% confidence intervals) comparing the highest with the lowest categories of adapted HEI and DASH were 0.61 (0.50, 0.76) and 0.70 (0.55, 0.89), respectively. No significant associations were observed for a vegetarian eating pattern (adjusted OR 0.80 (0.55, 1.17)). Healthy dietary patterns, as described by a high HEI or DASH score, but not a vegetarian diet alone, are associated with reduced risk of advanced colorectal neoplasms. Copyright © 2018 Elsevier Inc. All rights reserved.

  13. Use of color Doppler ultrasonography for the prediction of malignancy in follicular thyroid neoplasms: systematic review and meta-analysis.

    Science.gov (United States)

    Iared, Wagner; Shigueoka, David Carlos; Cristófoli, Júlio Cesar; Andriolo, Regis; Atallah, Alvaro Nagib; Ajzen, Sérgio Aron; Valente, Orsine

    2010-03-01

    The purpose of this systematic review was to obtain summary estimates of the diagnostic accuracy of color Doppler ultrasonography (CDU) in predicting malignancy in thyroid follicular neoplasms (FNs). We searched Medical Subject Headings together with the search terms "follicular," "thyroid," and "Doppler" in the MEDLINE, Web of Science, and Excerpta Medica databases as well as the Latin American and Caribbean Health Sciences Literature database, after which we performed manual searches of the reference lists to locate additional studies. There were no language restrictions. We included studies that assessed the diagnostic accuracy of CDU in identifying malignancy in thyroid FNs. The assessments of the quality and extraction of data were performed by 3 independent reviewers. We included 4 studies, which collectively evaluated 457 thyroid FNs, 67 of which had been classified as malignant based on the evaluation of surgical biopsy samples. Moderate, rich, predominant, or exclusive internal flow on CDU of thyroid FNs was considered indicative of malignancy. The overall sensitivity of CDU was 85% (95% confidence interval [CI], 74%-93%), with an overall specificity of 86% (95% CI, 82%-89%). The overall prevalence was 14.7%, and the positive and negative predictive values were 51% and 97%, respectively. The positive likelihood ratio was 6.07, and the negative likelihood ratio was 0.18. Predominant internal flow seen on CDU is associated with malignancy of thyroid FNs. Absence of internal flow or predominantly peripheral flow indicates a low probability of thyroid FN malignancy.

  14. Diffuse large B-cell lymphoma of the kidney: A rare neoplasm

    Directory of Open Access Journals (Sweden)

    Ram Narayan Das

    2013-01-01

    Full Text Available Primary renal lymphoma is a rare neoplasm, but it should be kept in mind in the differential diagnosis of renal neoplasms. A middle aged man presented with symptoms of weight loss, anorexia and fullness of the abdomen after meals. On clinical and radiological examination, a renal mass was revealed and operated upon. A diagnosis of primary high grade renal lymphoma was made on histopathological examination and immunohistochemically it was further classified as diffuse large B-cell lymphoma. Unfortunately, the patient died after 5 months of diagnosis in spite of three cycles of chemotherapy following surgery. The pathological details of rare tumor are presented here.

  15. Malignant Nonfunctioning Neuroendocrine Neoplasm of the Pancreas in a 10-Year-Old Child

    Directory of Open Access Journals (Sweden)

    Ahmed Marwan

    2009-04-01

    Full Text Available Malignant neoplasms of the pancreas are extremely rare in children and only represent a small percentage of pediatric cancer-related deaths. The paucity of cases reported in the literature, in addition to the lack of understanding of biologic behavior, has led to a lack of consensus concerning optimal management strategy. Presentation differs compared to adult counterparts and generally prognosis is improved even when lymph node metastases occur. Here we review the literature and report the case of a 10-year-old autistic female with a malignant nonfunctioning pancreatic endocrine neoplasm of the head of the pancreas successfully extirpated via pancreaticoduodenectomy.

  16. Myeloid neoplasm with prominent eosinophilia and PDGFRA rearrangement treated with imatinib mesylate

    DEFF Research Database (Denmark)

    Rathe, Mathias; Kielsgaard Kristensen, Thomas; Møller, Michael Boe

    2010-01-01

    The FIP1L1-PDGFRA fusion gene is the most frequent genetic aberration in myeloid neoplasms associated with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1. Affected patients in adult populations are very sensitive to imatinib therapy. Pediatric cases are rare and so far only one case...... of FIP1L1-PDGFRA positive disease has been reported. We report a 2-year-old female with a myeloid neoplasm associated with eosinophilia and rearrangement of PDGFRA. Treatment with imatinib resulted in complete and durable clinical, hematological, and molecular remission within 3 months after starting...

  17. Myeloid Neoplasms with Germline Predisposition: A New Provisional Entity Within the World Health Organization Classification.

    Science.gov (United States)

    Czuchlewski, David R; Peterson, LoAnn C

    2016-03-01

    The forthcoming update of the World Health Organization (WHO) classification of hematopoietic neoplasms will feature "Myeloid Neoplasms with Germline Predisposition" as a new provisional diagnostic entity. This designation will be applied to some cases of acute myeloid leukemia and myelodysplastic syndrome arising in the setting of constitutional mutations that render patients susceptible to the development of myeloid malignancies. For the diagnostic pathologist, recognizing these cases and confirming the diagnosis will demand a sophisticated grasp of clinical genetics and molecular techniques. This article presents a concise review of this new provisional WHO entity, including strategies for clinical practice. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Bone sarcoma as a second malignant neoplasm in children: influence of radiation and genetic predisposition

    International Nuclear Information System (INIS)

    Meadows, A.T.; Strong, L.C.; Li, F.P.; D'angio, G.J.; Schweisguth, O.; Freeman, A.I.; Jenkin, R.D.T.; Morris-Jones, P.; Nesbit, M.E.

    1980-01-01

    Osteosarcoma or chondrosarcoma developed as a second malignant neoplasm (SMN) in 40 of 188 individuals with SMN whose first neoplasm occurred in childhood. A genetic susceptibility to cancer was found in 23; the SMN developed in an irradiated field in 32; both factors were present in 16; neither in one. When a genetic predisposition was present, radiation shortened the interval to SMN. The intervals between tumors and the age at which the bone sarcomas developed in relation to genetic disease and therapy were analyzed by a two-mutation hypothesis

  19. Immunomodifying effect of VCG vaccine in treatment of urinary bladder neoplasm

    International Nuclear Information System (INIS)

    Neprina, G.S.; Panteleeva, E.S.; Vatin, O.E.; Karyakin, O.B.; Kurasova, V.G.; Filatov, P.P.; Dunchik, V.N.

    1989-01-01

    It is shown that immunotherapy realization using VCG vaccine after completion of PCT (polychemotherapy) course in patients suffering from later stages of urinary bladder neoplasm, allowed one to maximally connect stages of chemo- and radiation therapy at the expense of sufficient increase of the quantity of main groups of immunocompetent cells. Introduction of incometacin to immunocorrection scheme allowed one to remove disbalance in immunoregulating lymphocyte system which testifies to advisability of combined applicaion of VCG vaccine and indometacin in complex treatment of cerinary bladder neoplasms. 5 refs

  20. Activating mutations of the GNAQ gene : a frequent event in primary melanocytic neoplasms of the central nervous system

    NARCIS (Netherlands)

    Kusters-Vandevelde, Heidi V. N.; Klaasen, Annelies; Kusters, Benno; Groenen, Patricia J. T. A.; van Engen-van Grunsven, Ilse A. C. H.; van Dijk, Marcory R. C. F.; Reifenberger, Guido; Wesseling, Pieter; Blokx, Willeke A. M.

    Primary melanocytic neoplasms of the central nervous system (CNS) are uncommon neoplasms derived from melanocytes that normally can be found in the leptomeninges. They cover a spectrum of malignancy grades ranging from low-grade melanocytomas to lesions of intermediate malignancy and overtly

  1. Surgical management of neoplasms of the ampulla of Vater: Local resection or pancreatoduodenectomy and prognostic factors for survival

    NARCIS (Netherlands)

    de Castro, S. M. M.; van Heek, N. T.; Kuhlmann, K. F. D.; Busch, O. R. C.; Offerhaus, G. J. A.; van Gulik, T. M.; Obertop, H.; Gouma, D. J.

    2004-01-01

    yBackground. Neoplasms of the ampulla of Vater have a better 5-year survival than pancreatic and bile duct neoplasms after resection. This study was Performed to analyze the outcome after local resection and pancreatoduodenectomy (PD) and to identify predictive factors for survival. Methods. We used

  2. Sebaceous neoplasms and the immunoprofile of mismatch-repair proteins as a screening target for syndromic cases

    DEFF Research Database (Denmark)

    Boennelycke, Marie; Thomsen, Birthe M; Holck, Susanne

    2015-01-01

    , MLH1/PMS2 loss in 3, MSH6 loss only in 2 cases) and 1 (16.7%) SC (MLH1/PMS2 loss). All 4 MLH1 deficient cases were p16-positive. CONCLUSION: A substantial proportion of sebaceous neoplasms were MMR-protein deficient and thus likely MTS candidates. Given the low prevalence of sebaceous neoplasms...

  3. Multimodal MRI in the characterization of glial neoplasms: the combined role of single-voxel MR spectroscopy, diffusion imaging and echo-planar perfusion imaging

    International Nuclear Information System (INIS)

    Zonari, Paolo; Baraldi, Patrizia; Crisi, Girolamo

    2007-01-01

    Diffusion-weighted imaging (DWI), perfusion-weighted imaging (PWI) and MR spectroscopy (MRS) provide useful data for tumor evaluation. To assess the contribution of these multimodal techniques in grading glial neoplasms, we compared the value of DWI, PWI and MRS in the evaluation of histologically proven high- and low-grade gliomas in a population of 105 patients. Independently for each modality, the following variables were used to compare the tumors: minimum apparent diffusion coefficient (ADC) and maximum relative cerebral blood volume (rCBV) normalized values between tumor and healthy tissue, maximum Cho/Cr ratio and minimum NAA/Cr ratio in tumor, and scored lactate and lipid values in tumor. The Mann-Whitney and Wilcoxon tests were employed to compare DWI, PWI and MRS between tumor types. Logistic regression analysis was used to determine which parameters best increased the diagnostic accuracy in terms of sensitivity, specificity, and positive and negative predictive values. ROC curves were determined for parameters with high sensitivity and specificity to identify threshold values to separate high- from low-grade lesions. Statistically significant differences were found for rCBV tumor/normal tissue ratio, and NAA/Cr ratio in tumor and Cho/Cr ratio in tumor between low- and high-grade tumors. The best performing single parameter for group classification was the normalized rCBV value; including all parameters, statistical significance was reached by rCBV tumor/normal tissue ratio, NAA/Cr tumor ratio and lactate. From the ROC curves, a high probability for a neoplasm to be a high-grade lesion was associated with a rCBV tumor/normal tissue ratio of >1.16 and NAA/Cr tumor ratio of <0.44. Combining PWI and MRS with conventional MR imaging increases the accuracy of the attribution of malignancy to glial neoplasms. The best performing parameter was found to be the perfusion level. (orig.)

  4. Multimodal MRI in the characterization of glial neoplasms: the combined role of single-voxel MR spectroscopy, diffusion imaging and echo-planar perfusion imaging

    Energy Technology Data Exchange (ETDEWEB)

    Zonari, Paolo [Ospedale ' ' B. Ramazzini' ' , AUSL Modena, Neuroradiologia, Dipartimento Integrato di Neuroscienze, Carpi, Modena (Italy); Baraldi, Patrizia [Universita degli Studi di Modena e Reggio Emilia, Dipartimento di Scienze Biomediche, Sezione di Fisiologia, Modena (Italy); Crisi, Girolamo [Azienda Ospedaliero-Universitaria di Parma, Dipartimento ad Attivita Integrata di Neuroscienze, Parma (Italy)

    2007-10-15

    Diffusion-weighted imaging (DWI), perfusion-weighted imaging (PWI) and MR spectroscopy (MRS) provide useful data for tumor evaluation. To assess the contribution of these multimodal techniques in grading glial neoplasms, we compared the value of DWI, PWI and MRS in the evaluation of histologically proven high- and low-grade gliomas in a population of 105 patients. Independently for each modality, the following variables were used to compare the tumors: minimum apparent diffusion coefficient (ADC) and maximum relative cerebral blood volume (rCBV) normalized values between tumor and healthy tissue, maximum Cho/Cr ratio and minimum NAA/Cr ratio in tumor, and scored lactate and lipid values in tumor. The Mann-Whitney and Wilcoxon tests were employed to compare DWI, PWI and MRS between tumor types. Logistic regression analysis was used to determine which parameters best increased the diagnostic accuracy in terms of sensitivity, specificity, and positive and negative predictive values. ROC curves were determined for parameters with high sensitivity and specificity to identify threshold values to separate high- from low-grade lesions. Statistically significant differences were found for rCBV tumor/normal tissue ratio, and NAA/Cr ratio in tumor and Cho/Cr ratio in tumor between low- and high-grade tumors. The best performing single parameter for group classification was the normalized rCBV value; including all parameters, statistical significance was reached by rCBV tumor/normal tissue ratio, NAA/Cr tumor ratio and lactate. From the ROC curves, a high probability for a neoplasm to be a high-grade lesion was associated with a rCBV tumor/normal tissue ratio of >1.16 and NAA/Cr tumor ratio of <0.44. Combining PWI and MRS with conventional MR imaging increases the accuracy of the attribution of malignancy to glial neoplasms. The best performing parameter was found to be the perfusion level. (orig.)

  5. Identification of Two Distinct Molecular Subtypes of Non-Invasive Follicular Neoplasm with Papillary-Like Nuclear Features by Digital RNA Counting.

    Science.gov (United States)

    Giannini, Riccardo; Ugolini, Clara; Poma, Anello Marcello; Urpì, Maria; Niccoli, Cristina; Elisei, Rossella; Chiarugi, Massimo; Vitti, Paolo; Miccoli, Paolo; Basolo, Fulvio

    2017-10-01

    The follicular variant (FV) of papillary thyroid cancer (PTC) is one of the most common variants of PTC. Clinically, non-infiltrative FVPTC is considered a low-risk variant of PTC, and the non-invasive encapsulated forms of FVPTC represent a group of thyroid tumors with a particularly good prognosis. Consequently, these neoplasms have been very recently reclassified as non-invasive follicular neoplasms with papillary-like nuclear features (NIFTP). From a molecular standpoint, NIFTP appears to be similar to follicular neoplasms. However, only limited data are currently available regarding their gene expression profile. The aim of this study was to identify specific molecular signatures of 26 NIFTPs compared to those of 19 follicular adenomas (FAs) and 18 infiltrative FVPTCs (IFVPTCs). A nanoString custom assay was used to perform mRNA expression analysis. All cases were also genotyped for BRAF, N-, H-, and K-RAS mutations. Samples were grouped on the basis of gene expression profiles by Pearson's correlation and non-negative matrix factorization clustering analysis. Finally, the uncorrelated shrunken centroid machine-learning algorithm was used to classify the samples. The results revealed distinct expression profiles of FAs and IFVPTCs. NIFTP samples can exhibit different expression profiles, more similar to FAs (FA-like) or to IFVPTCs (IFVPTC-like), and these different expression profiles largely depend on the presence of different mutations (RAS or BRAF). In conclusion, although further validation of the model is required by using a larger group of prospective cases, these data reinforce the hypothesis that IFVPTC-like NIFTPs might represent precursors of IFVPTC.

  6. Interleukin-12 in Treating Patients With Hematologic Cancers or Solid Tumors

    Science.gov (United States)

    2014-09-09

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Kidney Cancer; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  7. Interleukin-12 Followed by Interferon Alfa in Treating Patients With Advanced Cancer

    Science.gov (United States)

    2013-01-31

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  8. Biological Therapy Following Chemotherapy and Peripheral Stem Cell Transplantation in Treating Patients With Cancer

    Science.gov (United States)

    2013-03-25

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Kidney Cancer; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Neuroblastoma; Ovarian Cancer; Sarcoma; Testicular Germ Cell Tumor

  9. Second malignant neoplasms in childhood cancer survivors in a tertiary paediatric oncology centre in Hong Kong, China.

    Science.gov (United States)

    Sun, Wai-Fun; Cheng, Frankie Wai-Tsoi; Lee, Vincent; Leung, Wing-Kwan; Shing, Ming-Kong; Yuen, Patrick Man-Pan; Li, Chi-Kong

    2011-11-01

    Childhood cancer survivors were at risk of development of second malignant neoplasms. The aim of this study is to evaluate the incidence, risk factors and outcome of second malignant neoplasms in childhood cancer survivors in a tertiary paediatric oncology centre in Hong Kong, China. We performed a retrospective review of patients with childhood cancer treated in Children's Cancer Centre in Prince of Wales Hospital, Hong Kong, China between May 1984 and June 2009. Case records of patients who developed second malignant neoplasms were reviewed. Totally 1374 new cases aged less than 21-year old were treated in our centre in this 25-year study period. Twelve cases developed second malignant neoplasms with 10-year and 20-year cumulative incidence of 1.3% (95% confidence interval 0.3% - 2.3%) and 2.9% (95% confidence interval 1.1% - 4.7%) respectively. Another 4 cases were referred to us from other centres for the management of second malignant neoplasms. In this cohort of 16 children with second malignant neoplasms, the most frequent second malignant neoplasms were acute leukemia or myelodysplastic syndrome (n = 6) and central nervous system tumor (n = 4). Median interval between diagnosis of primary and second malignant neoplasms was 7.4 years (range 2.1 - 13.3 years). Eight patients developed second solid tumor within the previous irradiated field. Radiotherapy significantly increased the risk of development of second solid tumor in patients with acute lymphoblastic leukemia (P = 0.027). Seven out of 16 patients who developed second malignant neoplasms had a family history of cancer among the first or second-degree relatives. Nine patients died of progression of second malignant neoplasms, mainly resulted from second central nervous system tumor and osteosarcoma. Cumulative incidence of second cancer in our centre was comparable to western countries. Radiotherapy was associated with second solid tumour among patients with acute lymphoblastic leukemia. Patients who

  10. CT investigation of lung neoplasm changes after cluster electrode radio frequency ablation

    International Nuclear Information System (INIS)

    Pan Haiying; Xu Shandan; Wang Yaocheng; Liang Guomin; Wu Qiuzhen; Huang Jin

    2001-01-01

    Objective; To evaluate short-term therapeutic effect of lung neoplasm after cluster electrode radio frequency ablation. Methods: CT scans were performed in 68 cases with lung neoplasm to investigate the alterations of tumor size and density pre- and post-procedure. Results: Seventy lesions in 68 cases with lung neoplasm were observed by CT pre-procedure and 30 min after the procedure, while the follow-up was taken place after 60 d. In mono-target group, size of the lesion was increased 30 min after the procedure and decreased after 60 d, the sizes were (4.15 +- 0.97) cm, (5.54 +- 1.37) cm and (2.79 +- 0.68) cm, respectively. There were significant differences between them (P 0.05). The density of the lesions were lessened both 30 min and 60 d after the procedure. In mono-target group, the average density of lesions in different times were (49.6 +- 6.2) Hu, (40.5 +- 14.4) Hu and (35.2 +- 3.2) Hu, respectively, while they were (46.7 +- 5.3) Hu, (37.4 +- 11.8) Hu, (35.1 +- 2.3) Hu, respectively, in multi-target. There were significant differences (P<0.05). Conclusions: CT scan is an useful method to observe size and density of lung neoplasm after the procedure of cluster electrode radio frequency ablation providing information of objective parameters and prediction of prognosis

  11. The Disruption of Geniculocalcarine Tract in Occipital Neoplasm: A Diffusion Tensor Imaging Study

    Directory of Open Access Journals (Sweden)

    Yan Zhang

    2016-01-01

    Full Text Available Aim. Investigate the disruption of geniculocalcarine tract (GCT in different occipital neoplasm by diffusion tensor imaging (DTI. Methods. Thirty-two subjects (44.1 ± 3.6 years who had single occipital neoplasm (9 gliomas, 6 meningiomas, and 17 metastatic tumors with ipsilateral GCT involved and thirty healthy subjects (39.2 ± 3.3 years underwent conventional sequences scanning and diffusion tensor imaging by a 1.5T MR scanner. The diffusion-sensitive gradient direction is 13. Compare the fractional anisotropy (FA and mean diffusivity (MD values of healthy GCT with the corresponding values of GCT in peritumoral edema area. Perform diffusion tensor tractography (DTT on GCT by the line propagation technique in all subjects. Results. The FA values of GCT in peritumoral edema area decreased (P=0.001 while the MD values increased (P=0.002 when compared with healthy subjects. There was no difference in the FA values across tumor types (P=0.114 while the MD values of GCT in the metastatic tumor group were higher than the other groups (P=0.001. GCTs were infiltrated in all the 9 gliomas cases, with displacement in 2 cases and disruption in 7 cases. GCTs were displaced in 6 meningiomas cases. GCTs were displaced in all the 7 metastatic cases, with disruption in 7 cases. Conclusions. DTI represents valid markers for evaluating GCT’s disruption in occipital neoplasm. The disruption of GCT varies according to the properties of neoplasm.

  12. [Thymic epithelial neoplasms: updates on diagnosis, staging, biology and management in France].

    Science.gov (United States)

    Hadoux, Julien; Girard, Nicolas; Besse, Benjamin

    2012-11-01

    Thymic epithelial neoplasms are rare malignancies with about 250 new incident cases in France every year. The WHO histologic classification distinguishes thymoma and thymic carcinoma which are tumors with different biological and clinical behaviors and outcomes. The Masaoka-Koga staging system is considered as a reference and is also of prognosis value. Diagnosis, multimodal treatment and follow-up of thymic epithelial neoplasms require a multidisciplinary approach where surgery is the cornerstone treatment. A national expert center coordinates thymic epithelial neoplasms management with 12 other regional expert centers through the French organization named RYTHMIC (www.rythmic.org). Patient's files have to be discussed at regional or national multidisciplinary staff. A group of expert pathologists will centrally review tumors when the diagnosis or classification is a matter of controversy. Among its objectives, RYHTMIC has to promote medical education, patient's information and research. This review focuses on RYTHMIC guidelines and data regarding multimodal management and targeted therapies in epithelial thymic neoplasms.

  13. Orthovolt X-radiation and gamma-therapy pf brain neoplasms

    International Nuclear Information System (INIS)

    Pil', B.N.; Badmaev, K.N.

    1982-01-01

    Actuallity of application of X-ray and gamma-therapy of brain neoplasms is shown. Various irradiation methods are described. Data on life span, observation maximum terms, survival rate of patients with brain tumors, who underwent orthovolt X-ray and gamma-therapy are presented

  14. Histologically benign but clinically malignant neoplasms in the thorax: CT–pathological overview

    International Nuclear Information System (INIS)

    Kim, E.Y.; Kim, T.S.; Han, J.; Kim, H.; Choi, Y.S.

    2012-01-01

    The purpose of this article is to review the computed tomography (CT) and histopathological features of uncommon primary neoplasms of the thorax that can manifest clinically malignant features (multiplicity of pulmonary nodules, an invasive nature, and metastases or recurrence after surgery) with little evidence of histological malignancy.

  15. Evaluation of selected interleukins in patients with different gastric neoplasms: a preliminary report

    Science.gov (United States)

    Madej-Michniewicz, Anna; Budkowska, Marta; Sałata, Daria; Dołęgowska, Barbara; Starzyńska, Teresa; Błogowski, Wojciech

    2015-01-01

    Abnormal interactions between cytokines may be an overlooked mechanism linking the development of different types of gastric neoplasms. In this study a comprehensive analysis of the systemic levels of interleukins (IL-1,IL-6, IL-8,IL-10 and IL-12) was performed in 75 patients with different gastric neoplasms (cancer, gastrointestinal stromal tumors, neuroendocrine neoplasms, lymphomas) and 40 healthy volunteers. Patients with gastric cancer (GC) have significantly higher IL-6 levels, and lower IL-8 and IL-10 concentrations, in comparison to controls and patients with other gastric neoplasms. Analogous results were observed in terms of IL-6/IL-8 and IL-6/IL-10 ratios, whose values were also higher in GC patients. In GC patients no associations were detected between the systemic levels/values of interleukins (ratios) and TNM staging. IL-6, IL-10, IL-6/IL-8 and IL-6/IL-10 ratios appeared to hold diagnostic potential in confirming/excluding the presence of GC. Their sensitivity/specificity in GC detection/exclusion was approximately 54–72%. In conclusion, disturbed systemic biochemical balance in multiple interleukins exists at the earliest stages of and appears to be specific to GC. The interleukin ratios proposed here seem to be more promising indicators of GC in humans than direct systemic levels of interleukins, and probably possess the potential to be applied as a supporting factor for techniques routinely used. PMID:26486258

  16. The 2016 revision of the World Health Organization classification of lymphoid neoplasms | Center for Cancer Research

    Science.gov (United States)

    A revision of the nearly 8-year-old World Health Organization classification of the lymphoid neoplasms and the accompanying monograph is being published. It reflects a consensus among hematopathologists, geneticists, and clinicians regarding both updates to current entities as well as the addition of a limited number of new provisional entities.

  17. The impact of ruxolitinib treatment on inflammation-mediated comorbidities in myelofibrosis and related neoplasms

    DEFF Research Database (Denmark)

    Bjørn, Mads Emil; Hasselbalch, Hans Carl

    2015-01-01

    The inflammation-mediated comorbidities in myelofibrosis (MF) and related neoplasms (MPNs) likely reflect the concurrent immune deregulation and systemic inflammatory nature of the MPNs, emphasizing the link between chronic systemic inflammation, immune deregulation, and the malignant clone. JAK1......-2 inhibitors in MF-patients reduce constitutional symptoms and splenomegaly, but also taget autoimmune and inflammation-mediated comorbidities....

  18. Surgical Management of Penile and Preputial Neoplasms in Equine with Special Reference to Partial Phallectomy

    Directory of Open Access Journals (Sweden)

    Awad Rizk

    2013-01-01

    Full Text Available Penile and preputial neoplasia in horses occurs infrequently and represents diagnostic and therapeutic challenges. The present study was carried out on a total number of 21 equids (14 stallions and 7 donkeys suffered from different penile and preputial neoplasia. Diagnosis of neoplasms was based up on history of the case, clinical examination as well as histopathological evaluation. Animals with penile and preputial neoplasms were underwent local excision and partial phallectomy with a slightly modified version of the techniques described by William’s. The diagnosed neoplasms were penile and preputial squamous cell carcinomas (SCCs; ; sarcoid (; a-fibrosarcoma; and a melanoma. Local excision was curative in all cases except 5 stallions with SCCs. These stallions had extensive damage of the glans penis, free part of the penis and the inner lamina of the internal fold of the prepuce, and they underwent a partial phallectomy with successful outcome. Follow-up information was obtained by visit and telephone inquiries. In conclusion, penile and preputial neoplasms are commonly encountered in elderly male horses and SCCs are the most common type affecting male external genitalia. Partial phallectomy is effective for management of equine neoplasia if they are confined to the glans and body of the penis and there is no proximal spread or involvement to regional lymph nodes.

  19. Synthetic display of three-dimensional CT and MPR for gastric neoplasm

    International Nuclear Information System (INIS)

    Ogura, Toshihiro; Maruyama, Masakazu

    1998-01-01

    We attempted to obtain synthesized three dimensional (3D) and MPR (Multi Planar Reconstruction) helical CT scans (3D-MPR-CT) of gastric neoplasm by using the air as a contrast medium, and we assessed the usefulness of 3D-MPR-CT gastroendoscopy in the diagnosis of gastric neoplasm. Five minutes before the scan, 20 mg Scopolamine Butylbromide (Buscopan) was injected intramuscularly to minimize gastric peristalsis. An effervescent agent (bubble-make granules) was fed to extend the stomach wall. Non-ionic contrast material (100 mL) was power injected immediately before the scan start. Axial images were obtained with an intersection gap of 5-mm, a 5-mm/sec table speed, and 1-mm reconstruction intervals. 3D-MPR-CT images were reconstructed from these images. In abdominal study, 3D-MPR-CT images enabled the visualization of neoplasm and its adjacent structures in versatile directions, including a view similar to endoscopic observation, proximal aspect of narrowing by tumor and also could get the information about invasive depth of gastric neoplasm. Reports on some clinical cases and the advantages and disadvantages of 3D-MPR-CT gastroendoscopy were discussed. (author)

  20. Synthetic display of three-dimensional CT and MPR for gastric neoplasm

    Energy Technology Data Exchange (ETDEWEB)

    Ogura, Toshihiro; Maruyama, Masakazu [Cancer Inst. Hospital, Tokyo (Japan)

    1998-03-01

    We attempted to obtain synthesized three dimensional (3D) and MPR (Multi Planar Reconstruction) helical CT scans (3D-MPR-CT) of gastric neoplasm by using the air as a contrast medium, and we assessed the usefulness of 3D-MPR-CT gastroendoscopy in the diagnosis of gastric neoplasm. Five minutes before the scan, 20 mg Scopolamine Butylbromide (Buscopan) was injected intramuscularly to minimize gastric peristalsis. An effervescent agent (bubble-make granules) was fed to extend the stomach wall. Non-ionic contrast material (100 mL) was power injected immediately before the scan start. Axial images were obtained with an intersection gap of 5-mm, a 5-mm/sec table speed, and 1-mm reconstruction intervals. 3D-MPR-CT images were reconstructed from these images. In abdominal study, 3D-MPR-CT images enabled the visualization of neoplasm and its adjacent structures in versatile directions, including a view similar to endoscopic observation, proximal aspect of narrowing by tumor and also could get the information about invasive depth of gastric neoplasm. Reports on some clinical cases and the advantages and disadvantages of 3D-MPR-CT gastroendoscopy were discussed. (author)

  1. Ion bombardment targeting neoplasms. The particle accelerator substituting the surgical knife

    International Nuclear Information System (INIS)

    Mueller, B.

    1997-01-01

    The particle accelerator can do the work of the surgical knife. Protons, neutrons, and heavy ions are of interest not only to physicists. The doctors are increasingly using the atomic ammunition from the large accelerators as a weapon for combatting neoplasms. (Orig./CB) [de

  2. Smell and taste in patients with neoplasms tumors of palatine tonsils treated by radiation

    International Nuclear Information System (INIS)

    Korytowska, A.; Szmeja, Z.; Wojtowicz, J.G.

    1993-01-01

    The smell and taste investigations were made in group of 47 subjects with neoplasms of palatine tonsils surgical treated with radiation and in group of 7 subjects treated only by radiation. The investigation presents mostly quantitative disturbance of smell in both groups but with temporary character. (author)

  3. Molecular approaches identify a cryptic MECOM rearrangement in a child with a rapidly progressive myeloid neoplasm.

    Science.gov (United States)

    Capela de Matos, Roberto R; Othman, Moneeb A K; Ferreira, Gerson M; Costa, Elaine S; Melo, Joana B; Carreira, Isabel M; de Souza, Mariana T; Lopes, Bruno A; Emerenciano, Mariana; Land, Marcelo G P; Liehr, Thomas; Ribeiro, Raul C; Silva, Maria Luiza M

    2018-02-01

    Myeloid neoplasms are a heterogeneous group of hematologic disorders with divergent patterns of cell differentiation and proliferation, as well as divergent clinical courses. Rare recurrent genetic abnormalities related to this group of cancers are associated with poor outcomes. One such abnormality is the MECOM gene rearrangement that typically occurs in cases with chromosome 7 abnormalities. MECOM encodes a transcription factor that plays an essential role in cell proliferation and maintenance and also in epigenetic regulation. Aberrant expression of this gene is associated with reduced survival. Hence, its detailed characterization provides biological and clinical information relevant to the management of pediatric myeloid neoplasms. In this work, we describe a rare karyotype harboring three copies of MECOM with overexpression of the gene in a child with a very aggressive myeloid neoplasm. Cytogenetic studies defined the karyotype as 46,XX,der(7)t(3;7)(q26.2;q21.2). Array comparative genomic hybridization (aCGH) revealed a gain of 26.04 Mb in the 3q26.2-3qter region and a loss of 66.6 Mb in the 7q21.2-7qter region. RT-qPCR analysis detected elevated expression of the MECOM and CDK6 genes (458.5-fold and 35.2-fold, respectively). Overall, we show the importance of performing detailed molecular cytogenetic analysis of MECOM to enable appropriate management of high-risk pediatric myeloid neoplasms. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Nordic guidelines 2014 for diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms

    DEFF Research Database (Denmark)

    Janson, Eva Tiensuu; Sorbye, Halfdan; Welin, Staffan

    2014-01-01

    BACKGROUND: The diagnostic work-up and treatment of patients with neuroendocrine neoplasms (NENs) has undergone major recent advances and new methods are currently introduced into the clinic. An update of the WHO classification has resulted in a new nomenclature dividing NENs into neuroendocrine...

  5. Risk of second malignant neoplasm and mortality in patients with rheumatoid arthritis treated with biological DMARDs

    DEFF Research Database (Denmark)

    Dreyer, Lene; Cordtz, René L; Hansen, Inger Marie J

    2018-01-01

    OBJECTIVE: To study the risk of a second malignant neoplasm (SMN) and mortality in patients with rheumatoid arthritis (RA) with a history of a primary cancer diagnosis and treated with biological disease-modifying antirheumatic drugs (bDMARD). METHODS: Among patients with RA (n=15 286) registered...

  6. Malignant melanoma as second malignant neoplasm in long-term childhood cancer survivors: A systematic review

    NARCIS (Netherlands)

    Braam, Katja I.; Overbeek, Annelies; Kaspers, Gertjan J. L.; Ronckers, Cecile M.; Schouten-van Meeteren, Annette Y. N.; van Dulmen-den Broeder, Eline; Veening, Margreet A.

    2012-01-01

    This systematic review provides information on malignant melanoma as second malignant neoplasm (SMN) after childhood cancer and evaluates its risk factors. Study reports describing incidences of SMN and malignant melanoma as SMN in a population of childhood cancer survivors (CCS) were included. Of

  7. [Cystic lymphangioma of the transverse mesocolon simulating neoplasm of the pancreatic tail].

    Science.gov (United States)

    Zago, A; Tosi, D; Portuese, D; Rosa, G

    1997-01-01

    Mesenteric cysts are uncommon lesions interesting surgeons above all for frequently difficulties in the preoperatory diagnosis. The role of radiology is to demonstrate the nature of these abdominal masses, but only with surgery we can establish a definitive histologic diagnosis. The authors report a case of a lynphangioma of the transverso mesocolon, miming a pancreatic neoplasm.

  8. Factors influencing comfort level in head and neck neoplasm patients receiving radiotherapy

    Directory of Open Access Journals (Sweden)

    Fang Cheng

    2014-12-01

    Conclusion: Encouraging utilization of social support systems and a positive coping mode is important for increasing comfort level in head and neck neoplasm patients during radiotherapy. Nurses should pay particular attention to those patients during later stages of radiotherapy or chemotherapy, with diabetes, without family accompaniment, and with lower education level.

  9. Radioisotopic methods for the study of bone sarcoma and soft tissue neoplasms

    Energy Technology Data Exchange (ETDEWEB)

    Gongora, R.

    1988-01-01

    Radioisotopic methods are widely applied to investigations of bone sarcoma and soft tissue neoplasms. We have at our disposal molecules with osseous, tumoral or vascular tropism. Their use, as single agents or combination, is helpful in positive and differential diagnosis and provides nosological informations. They are also useful in treatment monitoring and in long-term follow-up.

  10. Nodular fasciitis: A pseudomalignant clonal neoplasm characterized by USP gene rearrangements and spontaneous regression

    LENUS (Irish Health Repository)

    Hennebry, Jennifer

    2017-01-01

    Introduction: Nodular fasciitis (NF) is a rapidly growing, self-limited, myofibroblastic neoplasm that typically arises in subcutaneous tissues of young adults and regresses spontaneously. Nodular fasciitis mimics sarcoma on clinical, radiological, and histological grounds and is usually, diagnosed following excision.\\r\

  11. [Identification of human papilloma viruses (HPV) in inflammatory states and ear neoplasms].

    Science.gov (United States)

    Rydzewski, Bogdan; Goździcka-Józefiak, Anna; Sokalski, Jerzy; Matusiak, Monika; Durzyński, Lukasz

    2007-01-01

    Human Papilloma Virus has a strong relation to oropharyngeal mucosa and is considered to be responsible for a wide range of upper respiratory tract pathologies, like laryngeal papilloma. There's a hypothesis, that it plays a significant role in middle ear chronic inflammations and neoplasm's. MATERIAL AND METHODIC. The examination was carried on a group of 53 patients, 39 of which was suffering from granulation tissue chronic otitis media, 7-cholesteatomatous otitis media, 6--middle ear malignant neoplasm, and 1 middle and/or external ear benign neoplasm. The control group consisted of 5 patients operated on: otosclerosis--4 cases and post-traumatic tympanic membrane perforation--1 case. The material was postoperative tissue, like polyps, inflammatory granulation tissue, cholesteatoma masses and malignant neoplasm's tissue. In the whole group of 53 examined cases, HPV DNA was confirmed in 22 cases (41.5%), in that group oncogenic types 16 or 18 in 12 cases (22.6%), and in 14 cases (26.4%) types 6 or 11. In a group of chronic granulomatous otitis media DNA characteristic for Papilloma was identified in 12 cases (25.6%), in it in 9 cases DNA HPV type 6 or 11 was confirmed, and in 7 cases type 16 or 18. Among cholesteatomatous chronic otitis media HPV DNA types 6 or 11 was identified in 70%. In every case of middle ear malignant neoplasm a presence of high-risk DNA Papilloma types 16 or 18 was confirmed. In any case of control group HPV DNA was detected. The results has been compared with other authors examinations and it is claimed that they confirm the observation, that Human Papilloma Viruses may be a factor, that might play an important role in pathology of chronic otitis media and ear neoplasm's. It is concluded, that differences in percentages of HPV presence in chronic inflammations (70%) and ear neoplasm's may be explained by viral co-infection during bacterial c. o. m. Viral infection probably evolves carcinogenesis, which leads to a neoplastic growth.

  12. A STUDY ON EYELID AND CONJUNCTIVAL NEOPLASM IN A TERTIARY CARE CENTRE IN EASTERN INDIA

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    Soumya Swarup Chattopadhyay

    2017-09-01

    Full Text Available BACKGROUND Eyelid and conjunctival tumours (benign and malignant are very common in a tropical country like India causing not only cosmetic deformity, but also endangering patients’ life. The aim of our study is to determine the sociodemographic factors and clinicopathological assessment in order to early diagnosis and treatment. MATERIALS AND METHODS The present study is an observational study involving the cases with primary eyelid and/or conjunctival tumours. After thorough clinical history including detailed demographic details and clinical examination, the cases are admitted in the hospital. After undergoing thorough preoperative evaluation, FNAB or partial excision biopsy or total excision biopsy of the tumour mass is done by single competent ophthalmic surgeon and sent for histopathological analysis. The cases were classified into two major groups, eyelid neoplasm group and conjunctival neoplasm group. Each major group was subdivided into benign neoplasm and malignant neoplasm groups. The demographic details, clinical history, examination findings and results of the histopathological analysis is documented, appropriately tabulated and reported and statistical calculations done. RESULTS In this prospective observational study, 133 eyes of 133 patients (M:F=70:63 with primary eyelid and conjunctival tumour attending OPD and oculoplasty clinic at Regional Institute of Ophthalmology, Kolkata, were recruited into the study. There were a total of 103 (77.44% eyelid neoplasms and 30 (22.55% patients were having conjunctival neoplasms. Out of the 103 eyelids, neoplasms diagnosed 42.72% (n=44 of them were histopathologically diagnosed as benign and 57.28% (n=59 as malignant. Of the 59 cases diagnosed as malignant, four most common eyelid malignant neoplasms were sebaceous cell carcinoma (n=22, 37%, basal cell carcinoma (n=15, 25%, squamous cell carcinoma (n=10, 17% and malignant melanoma (n=7, 12%. Among the 30 conjunctival neoplasms

  13. SOX10 immunohistochemistry in sweat ductal/glandular neoplasms.

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    Cassarino, David S; Su, Albert; Robbins, Bruce A; Altree-Tacha, David; Ra, Seong

    2017-06-01

    SOX10 is a newer Schwannian and melanocytic marker that has generated great interest for its relative sensitivity and specificity in the diagnosis of neural crest-derived tumors. Previous studies with SOX10 have shown positive immunohistochemical expression in cutaneous eccrine glands and negative expression in eccrine ducts, apocrine glands and hair follicles. Thus, we hypothesized that some sweat gland tumors of presumed eccrine origin would be positive for SOX10, whereas apocrine-derived sweat gland tumors would not. A mouse monoclonal anti-SOX10 (clone BC34: Biocare Medical; Concord, California) immunohistochemical antibody was performed on various sweat gland tumors and basal cell carcinoma. SOX10 showed positivity in spiradenomas (13/13), cylindromas (9/10), hidradenoma papilliferum (10/10), syringocystadenoma papilliferum (8/10), apocrine adenomas (8/10), and negativity in poromas (0/12), syringomas (0/10), and basal cell carcinomas (0/13). There was mixed staining of hidradenomas (6/15). SOX10 immunohistochemistry may be of utility in distinguishing some of the varying adnexal tumors from each other, and from basal cell carcinoma (BCC), but given the staining of both apocrine and eccrine tumors, does not seem to provide information as to their origins as either eccrine or apocrine tumors. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  14. Factor V G1691A (Leiden and prothrombin G20210A gene mutation status, and thrombosis in patients with chronic myeloproliferative disorders

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    Nur Soyer

    2011-12-01

    Full Text Available Objective: The aim of this study was to examine Factor V G1691A (Leiden (FVL and prothrombin G20210A (PT gene mutation status, and their relationship with thrombosis in patients with chronic myeloproliferative disorders (CMPDs.Materials and Methods: The study included 160 patients with a CMPD that were regularly followed-up between 1993 and 2009. FVL and PT mutation status was established based on blood samples analyzed via PCR using specific primers.Results: The frequency of FVL and PT mutation was 12.5% and 4.4%, respectively. In total, 27 episodes of thrombosis occurred in 24 (15% of the patients, and there wasn’t an association between the observed thrombotic events, and FVL or PT mutations. Hepatic vein thrombosis was noted in 3 patients that had FVL mutation, of which 1 also had PT mutation.Conclusion: We did not observe a relationship between thrombosis, and FVL or PT mutations in CMPD patients; however, 3 of the patients that had hepatic vein thrombosis also had FVL mutation. Larger studies are needed to more clearly determine if all CMPD patients with hepatic vein thrombosis need be investigated for FVL and PT mutation.

  15. Characterization of the SUMO-binding activity of the myeloproliferative and mental retardation (MYM-type zinc fingers in ZNF261 and ZNF198.

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    Catherine M Guzzo

    Full Text Available SUMO-binding proteins interact with SUMO modified proteins to mediate a wide range of functional consequences. Here, we report the identification of a new SUMO-binding protein, ZNF261. Four human proteins including ZNF261, ZNF198, ZNF262, and ZNF258 contain a stretch of tandem zinc fingers called myeloproliferative and mental retardation (MYM-type zinc fingers. We demonstrated that MYM-type zinc fingers from ZNF261 and ZNF198 are necessary and sufficient for SUMO-binding and that individual MYM-type zinc fingers function as SUMO-interacting motifs (SIMs. Our binding studies revealed that the MYM-type zinc fingers from ZNF261 and ZNF198 interact with the same surface on SUMO-2 recognized by the archetypal consensus SIM. We also present evidence that MYM-type zinc fingers in ZNF261 contain zinc, but that zinc is not required for SUMO-binding. Immunofluorescence microscopy studies using truncated fragments of ZNF198 revealed that MYM-type zinc fingers of ZNF198 are necessary for localization to PML-nuclear bodies (PML-NBs. In summary, our studies have identified and characterized the SUMO-binding activity of the MYM-type zinc fingers in ZNF261 and ZNF198.

  16. Blast cell deficiency of CD11a as a marker of acute megakaryoblastic leukemia and transient myeloproliferative disease in children with and without Down syndrome.

    Science.gov (United States)

    Boztug, Heidrun; Schumich, Angela; Pötschger, Ulrike; Mühlegger, Nora; Kolenova, Alexandra; Reinhardt, Katarina; Dworzak, Michael

    2013-01-01

    The classification of acute myeloid leukemia (AML) FAB subtype M7 relies on immunophenotypic assessment. CD41 is expressed throughout all stages of maturation of megakaryocytes and has therefore been described as a specific blast cell marker in AML M7 as well as in transient myeloproliferative disease (TMD) of patients with Down syndrome (DS). However, technical difficulties underlie the need for new markers for these entities. We evaluated the expression of human lymphocyte function-associated antigen 1 (CD11a) in a large cohort of pediatric AML and TMD patients (n = 91) of the Austrian AML-BFM 98 and 2004 studies. We found a consistent deficiency of CD11a as assessed by mean fluorescence intensity in all patients with non-DS AML M7 (n = 8) and M6 (n = 1), all cases of classical DS-AML (n = 12) as well as TMD (n = 15) that was statistically significant in comparison to non-DS AML M0-M5 patients (n = 55; P leukemias (M4/5) and normal monocytes typically showed a high CD11a expression, FAB types M1/2 and normal neutrophils an intermediate expression level, while all M3 leukemias were rather low in CD11a expression. We conclude, that deficiency of CD11a expression should be added to the diagnostic criteria of AML-M7, classical DS-AML and TMD. Copyright © 2013 International Clinical Cytometry Society.

  17. Conditional Expression of E2A-HLF Induces B-Cell Precursor Death and Myeloproliferative-Like Disease in Knock-In Mice.

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    Jesús Duque-Afonso

    Full Text Available Chromosomal translocations are driver mutations of human cancers, particularly leukemias. They define disease subtypes and are used as prognostic markers, for minimal residual disease monitoring and therapeutic targets. Due to their low incidence, several translocations and their biological consequences remain poorly characterized. To address this, we engineered mouse strains that conditionally express E2A-HLF, a fusion oncogene from the translocation t(17;19 associated with 1% of pediatric B-cell precursor ALL. Conditional oncogene activation and expression were directed to the B-cell compartment by the Cre driver promoters CD19 or Mb1 (Igα, CD79a, or to the hematopoietic stem cell compartment by the Mx1 promoter. E2A-HLF expression in B-cell progenitors induced hyposplenia and lymphopenia, whereas expression in hematopoietic stem/progenitor cells was embryonic lethal. Increased cell death was detected in E2A-HLF expressing cells, suggesting the need for cooperating genetic events that suppress cell death for B-cell oncogenic transformation. E2A-HLF/Mb1.Cre aged mice developed a fatal myeloproliferative-like disorder with low frequency characterized by leukocytosis, anemia, hepatosplenomegaly and organ-infiltration by mature myelocytes. In conclusion, we have developed conditional E2A-HLF knock-in mice, which provide an experimental platform to study cooperating genetic events and further elucidate translational biology in cross-species comparative studies.

  18. Goblet cell carcinoid neoplasm of the appendix: Clinical and CT features

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    Lee, K.S., E-mail: kyungmouklee@alum.mit.edu [Department of Radiology Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Tang, L.H., E-mail: tangl@mskc.org [Department of Pathology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Shia, J., E-mail: shiaj@mskcc.org [Department of Pathology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Paty, P.B., E-mail: patyp@mskcc.org [Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Weiser, M.R., E-mail: weiser1@mskcc.org [Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Guillem, J.G., E-mail: guillemj@mskcc.org [Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Temple, L.K., E-mail: temple@mskcc.org [Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Nash, G.M., E-mail: nashg@mskcc.org [Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Reidy, D., E-mail: reidyd@mskcc.org [Department of Medicine, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Saltz, L., E-mail: saltzl@mskcc.org [Department of Medicine, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States); Gollub, M.J., E-mail: gollubm@mskcc.org [Department of Radiology Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065 (United States)

    2013-01-15

    Purpose: To describe the clinical and CT imaging features of goblet cell carcinoid (GCC) neoplasm of the appendix. Methods and materials: A computer search of pathology and radiology records over a 19-year period at our two institutions was performed using the search string “goblet”. In the patients with appendiceal GCC neoplasms who had abdominopelvic CT, imaging findings were categorized, blinded to gross and surgical description, as: “Appendicitis”, “Prominent appendix without peri-appendiceal infiltration”, “Mass” or “Normal appendix”. The CT appearance was correlated with an accepted pathological classification of: low grade GCC, signet ring cell adenocarcinoma ex, and poorly differentiated adenocarcinoma ex GCC group. Results: Twenty-seven patients (age range, 28–80 years; mean age, 52 years; 15 female, 12 male) with pathology-proven appendiceal GCC neoplasm had CT scans that were reviewed. Patients presented with acute appendicitis (n = 12), abdominal pain not typical for appendicitis (n = 14) and incidental finding (n = 1). CT imaging showed 9 Appendicitis, 9 Prominent appendices without peri-appendiceal infiltration, 7 Masses and 2 Normal appendices. Appendicitis (8/9) usually correlated with typical low grade GCC on pathology. In contrast, the majority of Masses and Prominent Appendices without peri-appendiceal infiltration were pathologically confirmed to be signet ring cell adenocarcinoma ex GCC. Poorly differentiated adenocarcinoma ex GCC was seen in only a small minority of patients. Hyperattenuation of the appendiceal neoplasm was seen in a majority of cases. Conclusions: GCC neoplasm of the appendix should be considered in the differential diagnosis in patients with primary appendiceal malignancy. Our cases demonstrated close correlation between our predefined CT pattern and the pathological classification.

  19. Peptide receptor radionuclide therapy as neoadjuvant therapy for resectable or potentially resectable pancreatic neuroendocrine neoplasms.

    Science.gov (United States)

    Partelli, Stefano; Bertani, Emilio; Bartolomei, Mirco; Perali, Carolina; Muffatti, Francesca; Grana, Chiara Maria; Schiavo Lena, Marco; Doglioni, Claudio; Crippa, Stefano; Fazio, Nicola; Zamboni, Giuseppe; Falconi, Massimo

    2018-04-01

    Peptide receptor radionuclide therapy is a valid therapeutic option for pancreatic neuroendocrine neoplasms. The aim of this study was to describe an initial experience with the use of peptide receptor radionuclide therapy as a neoadjuvant agent for resectable or potentially resectable pancreatic neuroendocrine neoplasms. The postoperative outcomes of 23 patients with resectable or potentially resectable pancreatic neuroendocrine neoplasms at high risk of recurrence who underwent neoadjuvant peptide receptor radionuclide therapy (peptide receptor radionuclide therapy group) were compared with 23 patients who underwent upfront surgical operation (upfront surgery group). Patients were matched for tumor size, grade, and stage. Median follow-up was 61 months. The size (median greatest width) of the primary pancreatic neuroendocrine neoplasms decreased after neoadjuvant peptide receptor radionuclide therapy (59 to 50 mm; P=.047). There were no differences in intraoperative and postoperative outcomes and there were no operative deaths, but the risk of developing a pancreatic fistula tended to be less in the peptide receptor radionuclide therapy group when compared to the upfront surgery group (0/23 vs 4/23; P radionuclide therapy group (n= 9/23 vs 17/23; P.2) differed between groups, but progression-free survival in the 31 patients who had an R0 resection seemed to be greater in the 15 patients in the peptide receptor radionuclide therapy group versus 16 patients the upfront group (median progression-free survival not reached vs 36 months; Pradionuclide therapy for resectable or potentially resectable pancreatic neuroendocrine neoplasms in patients with high-risk features of recurrence seems to be beneficial, but well-designed and much larger prospective trials are needed to confirm the safety and the oncologic value of this approach. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Incidental Gynecologic Tract Neoplasms in Women Undergoing Anterior Pelvic Exenteration for Urothelial Carcinoma.

    Science.gov (United States)

    Tran, Lily; Antic, Tatjana; Lastra, Ricardo R

    2017-12-18

    Urothelial carcinoma (UC) invasive into the muscularis propria or tumors unresponsive to treatment are indications for cystectomy. In females, with the goal of achieving complete cancer eradication and for concerns of UC extension into the adjacent pelvic organs, this may also warrant resection of the gynecologic organs. This study is aimed to assess the prevalence of unanticipated gynecologic neoplasms in anterior pelvic exenteration specimens. A retrospective review of pathology reports to identify women undergoing anterior pelvic exenteration for UC was performed (N=221), and incidentally discovered gynecologic tract neoplasms were recorded. Concomitant malignant or premalignant lesions of the gynecologic tract were identified in 8 patients (3.6%). These included endometrial adenocarcinoma [endometrioid type, International Federation of Gynecology and Obstetrics grade 1 (n=2, 0.9%)], cervical high-grade squamous intraepithelial lesion (n=2, 0.9%), Sertoli-Leydig cell tumor of intermediate differentiation (n=1, 0.5%), endometrioid adenocarcinoma of the ovary (n=1, 0.5%), and high-grade serous carcinoma of the ovary (n=1, 0.5%) and fallopian tube (n=1, 0.5%). Benign uterine neoplasms included leiomyomas (n=81, 37%), adenomyoma (n=3, 1.4%), and adenomatoid tumors (n=2, 0.9%). Benign ovarian neoplasms included serous cystadenoma (n=7, 3%), serous cystadenofibroma (n=4, 2%), benign Brenner tumor (n=5, 2.3%), mature teratoma (n=4, 2%), stromal luteoma (n=2, 0.9%), mucinous cystadenoma (n=1, 0.5%), thecoma (n=1, 0.5%), and endometrioid cystadenoma (n=1, 0.5%). Involvement of the gynecologic tract by UC was identified in 11 patients (5%). Spread of UC to the reproductive organs is rare in anterior pelvic exenteration specimens. Coexisting neoplasms of the gynecologic tract are occasionally identified, therefore careful evaluation of these organs is necessary.