title: Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association.
abstract: Mediastinal gray zone lymphoma, B-cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma, have not been 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 (30.3%) with classical Hodgkin lymphoma or composite (5.1%) (synchronous occurrence of classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma). The median age was 32 years (13-83 years); 55% were women. Thirteen of 81 evaluable cases (16%) were Epstein-Barr virus-positive. Twenty-eight percent of patients presented primary refractory disease (progression under first-line treatment or relapse within one year). The 3-year event-free and overall survival rates were 63% and 80%, respectively. Patients treated with a standard regimen (RCHOP/ABVD) had worse event-free survival (P=0.003) and overall survival (P=0.02) than those treated with a dose-intensive chemotherapy (high-dose RCHOP/escalated BEACOPP). Rituximab added to chemotherapy was not associated with better event-free survival (P=0.55) or overall survival (P=0.88). Radiotherapy for patients in complete remission had no impact on event-free survival. In multivariate prognostic analysis, ECOG-PS and anemia were the strongest factors associated with a shorter event-free survival and overall survival, respectively. In conclusion, this report describes the largest series of mediastinal gray zone lymphoma. Our data suggest that a dose-intensive treatment might improve the outcome of this rare and aggressive disease.
authors.affiliation: Hospices Civils de Lyon, Centre Hospitalier Lyon-Sud, Department of Hematology, Pierre Bénite cedex, France clementine.sarkozy@chu-lyon.fr.|INSERM1052, CNRS 5286, Université Claude Bernard, Faculté de Médecine Lyon-Sud Charles Mérieux Lyon-1, Pierre Bénite cedex, France.
authors.forename: Clémentine
authors.initials: C
authors.lastname: Sarkozy
authors.affiliation: Department of Pathology, Hôpital Universitaire Necker Enfants Malades, AP-HP, Université Paris Descartes, Paris, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Molina
authors.affiliation: Hospices Civils de Lyon, Centre Hospitalier Lyon-Sud, Department of Hematology, Pierre Bénite cedex, France.|INSERM1052, CNRS 5286, Université Claude Bernard, Faculté de Médecine Lyon-Sud Charles Mérieux Lyon-1, Pierre Bénite cedex, France.
authors.forename: Hervé
authors.initials: H
authors.lastname: Ghesquières
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Michallet
authors.affiliation: Department of Lymphoid Malignancies, Henri Mondor University Hospital, Créteil, France.
authors.forename: Jehan
authors.initials: J
authors.lastname: Dupuis
authors.affiliation: Department of Pathology, Hôpitaux Universitaire Paris Centre, Inserm U1138, Cordelier Research Center, Paris, France.
authors.forename: Diane
authors.initials: D
authors.lastname: Damotte
authors.affiliation: Department of Hematology, Centre Hospitalier Régional Universitaire de Lilles, France.
authors.forename: Franck
authors.initials: F
authors.lastname: Morsschauser
authors.affiliation: Department of Pathology, INSERM U1053, CHU de Bordeaux, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Parrens
authors.affiliation: Department of Pathology, CHU de Dijon, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Martin
authors.affiliation: Department of Pathology, Institut Gustave Roussy, Villejuif, France.
authors.forename: Peggy
authors.initials: P
authors.lastname: Dartigues
authors.affiliation: Department of Hematology, U918Centre Henri Becquerel, Rouen, France.
authors.forename: Aspasia
authors.initials: A
authors.lastname: Stamatoullas
authors.affiliation: AP-HP, Hôpital Saint Antoine, Hematology Department, 75012, Paris, France.|Sorbonne Universités, UPMC Université Paris 06, UMR_S 938, CDR Saint-Antoine, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Hirsch
authors.affiliation: Department of Pathology, Hôpital Saint Antoine, AP-HP, Paris, France.
authors.forename: Bettina
authors.initials: B
authors.lastname: Fabiani
authors.affiliation: Department of Hematology and Cellular Therapy, CHU Hau-Lévèque, Bordeaux, France.
authors.forename: Krimo
authors.initials: K
authors.lastname: Bouabdallah
authors.affiliation: Department of Hematology, Instituto Português de Oncologia de Lisboa, Portugual.
authors.forename: Maria Gomes
authors.initials: MG
authors.lastname: da Silva
authors.affiliation: Institut Bordet, Université Libre de Bruxelles, Belgium.
authors.forename: Marie
authors.initials: M
authors.lastname: Maerevoet
authors.affiliation: Department of Pathology, CHU de Toulouse, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Laurent
authors.affiliation: Hospices Civils de Lyon, Centre Hospitalier Lyon-Sud, Department of Hematology, Pierre Bénite cedex, France.|INSERM1052, CNRS 5286, Université Claude Bernard, Faculté de Médecine Lyon-Sud Charles Mérieux Lyon-1, Pierre Bénite cedex, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
authors.affiliation: Hospices Civils de Lyon, Centre Hospitalier Lyon-Sud, Department of Hematology, Pierre Bénite cedex, France.|INSERM1052, CNRS 5286, Université Claude Bernard, Faculté de Médecine Lyon-Sud Charles Mérieux Lyon-1, Pierre Bénite cedex, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Salles
authors.affiliation: INSERM1052, CNRS 5286, Université Claude Bernard, Faculté de Médecine Lyon-Sud Charles Mérieux Lyon-1, Pierre Bénite cedex, France.|Hospices Civiles de Lyon, Centre Hospitalier Lyon-Sud, Pathology Department, 69495 Pierre Bénite cedex, France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Traverse-Glehen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D008297:Male; D008479:Mediastinal Neoplasms / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor