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Primary mediastinic lymphoma

Clémentine Sarkozy, Thierry Molina, Hervé Ghesquières, Anne-Sophie Michallet, Jehan Dupuis, Diane Damotte, Franck Morsschauser, Marie Parrens, Laurent Martin, Peggy Dartigues, Aspasia Stamatoullas, Pierre Hirsch, Betina Fabiani, Krimo Bouabdallah, Maria Gomes da Silva, Marie Maerevoet, Camille Laurent, Bertrand Coiffier, Gilles Salles, Alexandra Traverse-Glehen
Mediastinal grey zone lymphoma, B cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B cell lymphoma, are not well described in the literature. We report the clinical characteristics and outcomes of a large retrospective series of 99 cases centrally reviewed by a panel of hematopathologists, with a consensus established for the diagnosis. Cases were defined as classical Hodgkin lymphoma-like morphology (64.6%) with primary mediastinal B cell lymphoma immunophenotype, primary mediastinal B cell lymphoma-like morphology (30...
October 6, 2016: Haematologica
Anna Biernacka, Konstantinos D Linos, Peter A DeLong, Arief A Suriawinata, Vijayalakshmi Padmanabhan, Xiaoying Liu
When confronted with a metastatic poorly differentiated tumor of unknown origin, the initial workup includes the standard panel of immunostains to rule out carcinoma, sarcoma, lymphoma, and the greatest mimicker in pathology - malignant melanoma. Although not specific, the S-100 protein is expressed in over 95% of malignant melanomas. Herein, we present a case of multiorgan metastatic malignancy with a dominant hilar and mediastinal mass in a current smoker; clinically, highly suggestive of widespread primary lung cancer...
2016: CytoJournal
Naoto Tomita
Diffuse large B-cell lymphoma (DLBCL) accounts for 30-40% of all lymphoma subtypes. More than half of DLBCLs are estimated to arise from extranodal sites, and are called extranodal DLBCLs. Generally, the outcomes of extranodal DLBCL are not different from those of nodal DLBCL. To our knowledge, this report is the first to discuss the concept and the cell of origin of extranodal DLBCL, with the significance of the presence/absence of each involvement site in determining the outcome. Second, the evidence of clinical manifestation and outcomes of several extranodal DLBCLs requiring treatments other than R-CHOP, which is the current standard therapy for this malignancy, are discussed...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Anna Kocurek, Bogdan Małkowski, Agnieszka Giza, Wojciech Jurczak
AIM OF THE STUDY: Determining the role of PET/CT imaging in the evaluation of treatment efficacy in primary mediastinal B-cell lymphoma (PMBCL). MATERIAL AND METHODS: Retrospective analysis of seven PMBCL patients, treated at the University Hospital in Krakow, with interim PET/CT after the third course of chemo-immunotherapy.The analysis was based on the calculation of exact tumour volume and metabolic activity, compared with initial values (directly after diagnosis)...
2016: Contemporary Oncology Współczesna Onkologia
Mohammad O Hussaini, Friederike H Kreisel, Anjum Hassan, TuDung T Nguyen, John L Frater
CD4 expression is rare in diffuse large B-cell lymphoma (DLBCL), with 4 previously reported cases. Its significance is uncertain. We report five patients with CD4(+) DLBCL and one CD4(+) primary mediastinal large B-cell lymphoma. Cases were identified by searching the electronic database of the department; each was reviewed. Average age was 56 years. Neoplastic cells expressed CD20 (5/6 tested cases). BCL2/BCL6 expression were seen in 3/3 tested cases, suggesting a germinal center origin. Additionally, expression of T-cell antigens CD2 and CD5 was noted in 2/2 and CD7 in 1/1 tested case...
September 26, 2016: World Journal of Methodology
C C Pinnix, Z Abou Yehia, W Qiao, S A Milgrom, G L Smith, M Akhtari, J Reddy, J R Gunther, E M Osborne, O R Mawlawi, L J Medeiros, N Garg, H H Chuang, S Neelapu, J R Westin, B Dabaja
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Larry Mansouri, Daniel Noerenberg, Emma Young, Elena Mylonas, Maysaa Abdulla, Mareike Frick, Fazila Asmar, Viktor Ljungström, Markus Schneider, Kenichi Yoshida, Aron Skaftason, Tatjana Pandzic, Blanca Gonzalez, Anna Tasidou, Nils Waldhueter, Alfredo Rivas-Delgado, Maria Angelopoulou, Marita Ziepert, Christopher Maximilian Arends, Lucile Couronné, Dido Lenze, Claudia D Baldus, Christian Bastard, Jessica Okosun, Jude Fitzgibbon, Bernd Dörken, Hans G Drexler, Damien Roos-Weil, Clemens A Schmitt, Helga Duverger Munch-Petersen, Thorsten Zenz, Martin-Leo Hansmann, Jonathan C Strefford, Gunilla Enblad, Olivier A Bernard, Elisabeth Ralfkiaer, Martin Erlanson, Penelope Korkolopoulou, Magnus Hultdin, Theodora Papadaki, Kirsten Grønbæk, Armando Lopez-Guillermo, Seishi Ogawa, Ralf Küppers, Kostas Stamatopoulos, Niki Stavroyianni, George Kanellis, Andreas Rosenwald, Elias Campo, Rose-Marie Amini, German Ott, Theodoros P Vassilakopoulos, Michael Hummel, Richard Rosenquist, Frederik Damm
We recently reported a truncating deletion in the NFKBIE gene, which encodes IκBϵ, a negative feedback regulator of NF-κB, in clinically aggressive chronic lymphocytic leukemia (CLL). Preliminary data indicate enrichment of NFKBIE aberrations in other lymphoid malignancies, hence we screened a large patient cohort (n=1460) diagnosed with different lymphoid neoplasms. While NFKBIE deletions were infrequent in follicular lymphoma, splenic marginal-zone lymphoma, and T-cell acute lymphoblastic leukemia (<2%), slightly higher frequencies were seen in diffuse large B-cell lymphoma, mantle cell lymphoma, and primary CNS lymphoma (3-4%)...
September 26, 2016: Blood
Ziad Hilal, Franziska Hartmann, Askin Dogan, Cem Cetin, Harald Krentel, Sven Schiermeier, Beate Schultheis, Clemens B Tempfer
BACKGROUND: Lymphoma of the uterine cervix (LUCX) is rare and may occur as a primary or secondary manifestation of this disease. Clinical and cytological presentations of LUCX vary and establishing diagnosis is often difficult. Surgery followed by radiation or chemotherapy is the mainstay of treatment. CASE REPORT: We present the case of a 73-year-old woman with recurrent pathological PAP smears of the cervix and a history of chronic lymphatic leukemia 15 years ago...
September 2016: Anticancer Research
Shigeki Shimizu, Yasushi Inoue, Tomoki Utsumi, Tomohisa Okuma, Tomoya Kawaguchi, Takahiko Kasai, Satoru Munakata, Masaru Shibano, Shinji Atagi
We herein report a case of primary marginal zone lymphoma (MZL) of the posterior mediastinum in an 84-year-old woman. Computed tomography of the chest showed a posterior mediastinal mass in the right thoracic paravertebral region with right pleural effusion. Pathological findings of a surgical biopsy from the posterior mediastinum, along with immunohistochemical and flow cytometric results, indicated MZL. The patient was treated with chemotherapy and radiation therapy for the mediastinal lesion and achieved complete remission...
2016: Internal Medicine
Haa-Na Song, Seok Jin Kim, Young Hyeh Ko, Won Seog Kim
BACKGROUND: Mediastinal gray zone lymphoma (MGZL) shares clinical characteristics with primary mediastinal B-cell lymphoma (PMBCL) and nodular sclerosing Hodgkin lymphoma (NSHL). However, MGZL is extremely rare, and an appropriate treatment for it has not yet been established. METHODS: We retrospectively analyzed 8 patients who were treated with systemic chemotherapy for MGZL between 2007 and 2014. RESULTS: The patients with MGZL were predominantly young and male (median age 26 years), and 62...
2016: Acta Haematologica
Laurence Brugières, Pauline Brice
Lymphomas are one of the commonest malignancies in adolescents and young adults (AYA) accounting respectively for 22% of all cancers in patients aged 15-24 years (16% for Hodgkin lymphoma (HL) and 6% for non-HL (NHL)). The distribution of NHL subtypes in this age group differs strikingly from the distribution in children and in older adults with 4 main subtypes accounting for the majority of the cases: diffuse large B-cell lymphoma (DLBCL) including primary mediastinal B-cell lymphoma, Burkitt lymphoma, lymphoblastic lymphoma or anaplastic large cell lymphoma...
2016: Progress in Tumor Research
T T Win, Z Kamaludin, A Husin
Primary mediastinal large B-cell lymphoma (PMLBL) is an uncommon non-Hodgkin lymphoma with a distinct clinicopathological entity in the WHO classification of lymphoid malignancies. It is known to originate from B-cells of the thymus. It mimics thymic neoplasms and other lymphomas clinically and histopathologically. We reported a 33-year-old obese man who presented with shortness of breath off and on for 4 years. Radiologically, there was a huge anterior mediastinal mass. Tru-cut biopsy was initially diagnosed as type-A thymoma...
August 2016: Malaysian Journal of Pathology
Luca Ceriani, Sally Barrington, Alberto Biggi, Bogdan Malkowski, Ur Metser, Annibale Versari, Maurizio Martelli, Andrew Davies, Peter W Johnson, Emanuele Zucca, Stéphane Chauvie
The International Extranodal Lymphoma Study Group (IELSG)-37 is a prospective randomized trial assessing the role of consolidation mediastinal radiotherapy after immunochemotherapy to patients with newly diagnosed primary mediastinal large B-cell lymphoma (PMBCL). It is a positron emission tomography (PET) response-guided study where patients obtaining a complete metabolic response on an end-of-therapy PET-computed tomography (CT) scan evaluated by a central review are randomized to receive radiotherapy or no further treatment...
August 22, 2016: Hematological Oncology
Jozef Malysz, Patrick Erdman, Jeremy Klapper, Junjia Zhu, Michael Creer, Michael G Bayerl
BACKGROUND: US Food and Drug Administration approval of brentuximab vedotin for treatment of CD30-positive relapsed/refractory lymphomas, including classical Hodgkin lymphoma and anaplastic large cell lymphoma, initiated significant interest in researching CD30 expression in other therapy-resistant or relapsed lymphomas. We evaluated CD30 expression in 116 cases of aggressive B-cell lymphomas diagnosed at Penn State Milton S. Hershey Medical Center between 2000 and 2012 with the purpose of assessing the benefit of treatment with brentuximab...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
S Rauthe, A Rosenwald
Lymphomas infiltrating the mediastinum are a challenge for the treating physician as well as for the pathological diagnostics. The clinical scenario is often an emergency situation, while the pathologist is usually confronted only with small biopsy samples. Classical Hodgkin's lymphoma is by far the most frequently occurring lymphoma in the mediastinum and predominantly the nodular sclerosis subtype. In small and very sclerotic specimens it can be difficult to morphologically detect Hodgkin and Reed-Sternberg cells and to identify the characteristic phenotype by immunohistochemistry...
September 2016: Der Pathologe
R J Rieker, A Marx, A Agaimy, P Ströbel
Mesenchymal neoplasms of the thymus and mediastinum account for only 2 % of neoplasms of the mediastinum and are therefore very rare. With very few exceptions the histology, immunohistochemistry and (based on current knowledge) molecular biology of mediastinal soft tissue tumors are not different from their counterparts in other organs. Characteristic features are more concerned with clinical epidemiological and therapeutic aspects as well as the multitude of possible differential diagnoses. With the exception of organ-specific tumors, such as gastrointestinal stromal tumors (GIST), virtually all entities encountered in peripheral soft tissues can also arise in the mediastinum...
September 2016: Der Pathologe
Vincent Camus, Aspasia Stamatoullas, Sylvain Mareschal, Pierre-Julien Viailly, Nasrin Sarafan-Vasseur, Elodie Bohers, Sydney Dubois, Jean Michel Picquenot, Philippe Ruminy, Catherine Maingonnat, Philippe Bertrand, Marie Cornic, Valérie Tallon-Simon, Stéphanie Becker, Liana Veresezan, Thierry Frebourg, Pierre Vera, Christian Bastard, Hervé Tilly, Fabrice Jardin
Classical Hodgkin lymphoma is one of the most common lymphomas and shares clinical and genetic features with primary mediastinal B-cell lymphoma. In this retrospective study, we analyzed the recurrent hotspot mutation of the exportin 1 (XPO1, p.E571K) gene, previously identified in primary mediastinal B-cell lymphoma, in biopsies and plasma circulating cell-free DNA from patients with classical Hodgkin lymphoma using a highly sensitive digital PCR technique. A total of 94 patients were included in the present study...
September 2016: Haematologica
Mary Gleeson, Eliza A Hawkes, David Cunningham, Nick Chadwick, Nicholas Counsell, Anthony Lawrie, Andrew Jack, Paul Smith, Paul Mouncey, Christopher Pocock, Kirit M Ardeshna, John Radford, Andrew McMillan, John Davies, Deborah Turner, Anton Kruger, Peter W M Johnson, Joanna Gambell, David Linch
We performed a subgroup analysis of the phase III UK National Cancer Research Institute R-CHOP-14 versus R-CHOP-21 (two- versus three-weekly rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) trial to evaluate the outcomes for 50 patients with World Health Organization 2008 classified primary mediastinal B-cell lymphoma identified from the trial database. At a median follow-up of 7·2 years the 5-year progression-free survival and overall survival was 79·8% and 83·8%, respectively. An exploratory analysis raised the possibility of a better outcome in those who received R-CHOP-14 and time intensification may still, in the rituximab era, merit testing in a randomised trial in this subgroup of patients...
August 1, 2016: British Journal of Haematology
Ya K Mangasarova, A U Magomedova, E S Nesterova, E M Volodicheva, V I Vorob'ev, S K Kravchenko
AIM: to evaluate the efficiency of the R-DA-EPOCH-21 + R-DHAP protocol and autologous hematopoietic stem cell transplantation (a BEAM conditioning mode) in first-line therapy for primary mediastinal large B-cell lymphoma (PMBCL). SUBJECTS AND METHODS: In 2013 to 2016, the investigation enrolled 57 patients with newly diagnosed PMBCL (according to the 2008 WHO criteria). The results were analyzed in 40 patients who had completed their treatment. RESULTS: All the 40 patients (14 men and 26 women) (median age, 27 years (19 to 67 years)) received 6 cycles of polychemotherapy (PCT) in accordance with the R-DA-EPOCH-21 regimen...
2016: Terapevticheskiĭ Arkhiv
Sergio Piña-Oviedo, Cesar A Moran
Primary mediastinal Classical Hodgkin lymphoma (CHL) is rare. Nodular sclerosis CHL (NS-CHL) is the most common subtype involving the anterior mediastinum and/or mediastinal lymph nodes. Primary thymic CHL is exceedingly rare. The disease typically affects young women and is asymptomatic in 30% to 50% of patients. Common symptoms include fatigue, chest pain, dyspnea and cough, but vary depending on the location and size of the tumor. B-symptoms develop in 30% of cases. By imaging, primary mediastinal CHL presents as mediastinal widening/mediastinal mass that does not invade adjacent organs but may compress vital structures as bulky disease...
September 2016: Advances in Anatomic Pathology
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