U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Mediastinal gray zone lymphoma

MedGen UID:
233679
•Concept ID:
C1334657
•
Neoplastic Process
Synonyms: Mediastinal B-Cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-Cell Lymphoma and Classic Hodgkin Lymphoma; mediastinal B-cell lymphoma, unclassifiable, with features Intermediate between diffuse large B-cell lymphoma and Classic Hodgkin lymphoma; Mediastinal B-Cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma; mediastinal B-cell lymphoma, unclassifiable, with features Intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma; Mediastinal Gray Zone Lymphoma; mediastinal gray zone lymphoma; mediastinal Gray zone lymphoma; Mediastinal Grey Zone Lymphoma; mediastinal Grey zone lymphoma
 
Monarch Initiative: MONDO:0004020

Definition

A mediastinal lymphoma with molecular, morphologic, immunophenotypic, and clinical features of both mediastinal (thymic) large B-cell lymphoma and classic Hodgkin lymphoma. The identification of this group of lymphomas, along with recent gene expression profiling results (PDL2 gene expression in both mediastinal (thymic) large B-cell lymphoma tissues and Hodgkin lymphoma cell lines), further supports the hypothesis that mediastinal (thymic) large B-cell lymphomas and classic Hodgkin lymphomas are related entities. [from NCI]

Professional guidelines

PubMed

Hodgkin Lymphoma and Its Differential Diagnosis: New Twists on an Old Challenge.
Sohani AR
Surg Pathol Clin 2023 Jun;16(2):287-346. Epub 2023 Mar 30 doi: 10.1016/j.path.2023.02.002. PMID: 37149361
Primary Mediastinal (Thymic) Large B-Cell Lymphoma: Fidelity of Diagnosis Using WHO Criteria.
Fairchild A, McCall CM, Oyekunle T, Niedzwiecki D, Champ C, McKinney M, Kelsey CR
Clin Lymphoma Myeloma Leuk 2021 May;21(5):e464-e469. Epub 2020 Dec 30 doi: 10.1016/j.clml.2020.12.015. PMID: 33487576
Management of Primary Mediastinal B-Cell Lymphoma and Gray Zone Lymphoma.
Dunleavy K
Oncology (Williston Park) 2017 Jun 15;31(6):499-501. PMID: 28620906

Recent clinical studies

Etiology

Primary Mediastinal (Thymic) Large B-Cell Lymphoma: Fidelity of Diagnosis Using WHO Criteria.
Fairchild A, McCall CM, Oyekunle T, Niedzwiecki D, Champ C, McKinney M, Kelsey CR
Clin Lymphoma Myeloma Leuk 2021 May;21(5):e464-e469. Epub 2020 Dec 30 doi: 10.1016/j.clml.2020.12.015. PMID: 33487576
The utility of CD83, fascin and CD23 in the differential diagnosis of primary mediastinal large B-cell lymphoma versus classic Hodgkin lymphoma.
Aladily TN, Mansour A, Alsughayer A, Sughayer M, Medeiros LJ
Ann Diagn Pathol 2019 Jun;40:72-76. Epub 2019 Apr 23 doi: 10.1016/j.anndiagpath.2019.04.009. PMID: 31075666
Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association.
Sarkozy C, Molina T, Ghesquières H, Michallet AS, Dupuis J, Damotte D, Morsschauser F, Parrens M, Martin L, Dartigues P, Stamatoullas A, Hirsch P, Fabiani B, Bouabdallah K, da Silva MG, Maerevoet M, Laurent C, Coiffier B, Salles G, Traverse-Glehen A
Haematologica 2017 Jan;102(1):150-159. Epub 2016 Oct 6 doi: 10.3324/haematol.2016.152256. PMID: 27758822Free PMC Article
Clinicopathological analysis of mediastinal large B-cell lymphoma and classical Hodgkin lymphoma of the mediastinum.
Yamamoto W, Nakamura N, Tomita N, Ishii Y, Takasaki H, Hashimoto C, Motomura S, Yamazaki E, Ohshima R, Numata A, Ishigatsubo Y, Sakai R
Leuk Lymphoma 2013 May;54(5):967-72. Epub 2012 Nov 1 doi: 10.3109/10428194.2012.733881. PMID: 23020783
Clinical, pathological and genetic features of primary mediastinal large B-cell lymphomas and mediastinal gray zone lymphomas in children.
Oschlies I, Burkhardt B, Salaverria I, Rosenwald A, d'Amore ES, Szczepanowski M, Koch K, Hansmann ML, Stein H, Möller P, Reiter A, Zimmermann M, Rosolen A, Siebert R, Jaffe ES, Klapper W
Haematologica 2011 Feb;96(2):262-8. Epub 2010 Oct 22 doi: 10.3324/haematol.2010.030809. PMID: 20971819Free PMC Article

Diagnosis

Mediastinal large B cell lymphoma and surrounding gray areas: a report of the lymphoma workshop of the 20th meeting of the European Association for Haematopathology.
Gibson SE, Dojcinov S, Dotlic S, Hartmann S, Hsi ED, Klimkowska M, Melle F, Pileri SA, Ramsower CA, Rech K, Rimsza LM, Rodriguez-Pinilla SM, Tousseyn TA, de Jong D, Sabattini E
Virchows Arch 2023 Dec;483(6):733-749. Epub 2023 Aug 2 doi: 10.1007/s00428-023-03550-5. PMID: 37530791Free PMC Article
Hodgkin Lymphoma and Its Differential Diagnosis: New Twists on an Old Challenge.
Sohani AR
Surg Pathol Clin 2023 Jun;16(2):287-346. Epub 2023 Mar 30 doi: 10.1016/j.path.2023.02.002. PMID: 37149361
Evolution in the definition and diagnosis of the Hodgkin lymphomas and related entities.
Tousseyn TA, King RL, Fend F, Feldman AL, Brousset P, Jaffe ES
Virchows Arch 2023 Jan;482(1):207-226. Epub 2022 Oct 24 doi: 10.1007/s00428-022-03427-z. PMID: 36274093
Mediastinal Lymphoproliferative Disorders.
Pina-Oviedo S
Adv Anat Pathol 2021 Sep 1;28(5):307-334. doi: 10.1097/PAP.0000000000000305. PMID: 34074861
Management of Primary Mediastinal B-Cell Lymphoma and Gray Zone Lymphoma.
Dunleavy K
Oncology (Williston Park) 2017 Jun 15;31(6):499-501. PMID: 28620906

Therapy

Nivolumab combined with brentuximab vedotin for relapsed/refractory mediastinal gray zone lymphoma.
Santoro A, Moskowitz AJ, Ferrari S, Carlo-Stella C, Lisano J, Francis S, Wen R, Akyol A, Savage KJ
Blood 2023 Jun 1;141(22):2780-2783. doi: 10.1182/blood.2022017951. PMID: 36898084
Mediastinal Gray Zone Lymphoma.
Bhardwaj M, Saha R, Misra DS, Malhotra V
Indian J Chest Dis Allied Sci 2015 Oct-Dec;57(4):255-7. PMID: 27164737
Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach?
Dunleavy K, Wilson WH
Blood 2015 Jan 1;125(1):33-9. Epub 2014 Dec 11 doi: 10.1182/blood-2014-05-575092. PMID: 25499450Free PMC Article
A prospective study of mediastinal gray-zone lymphoma.
Wilson WH, Pittaluga S, Nicolae A, Camphausen K, Shovlin M, Steinberg SM, Roschewski M, Staudt LM, Jaffe ES, Dunleavy K
Blood 2014 Sep 4;124(10):1563-9. Epub 2014 Jul 14 doi: 10.1182/blood-2014-03-564906. PMID: 25024303Free PMC Article
Gray zone lymphoma: better treated like hodgkin lymphoma or mediastinal large B-cell lymphoma?
Dunleavy K, Grant C, Eberle FC, Pittaluga S, Jaffe ES, Wilson WH
Curr Hematol Malig Rep 2012 Sep;7(3):241-7. doi: 10.1007/s11899-012-0130-5. PMID: 22833351Free PMC Article

Prognosis

Gray-zone lymphoma. Examples of rare clinical manifestation.
Misyurina AE, Kravchenko SK, Mangasarova YK, Magomedova AU, Kovrigina AM, Shimanovskaya LT, Fastova EA, Misyurin VA, Model SV, Chebotarev DI, Kostina IE, Savchenko VG
Ter Arkh 2019 May 16;91(4):107-113. doi: 10.26442/00403660.2019.04.000071. PMID: 31094484
Management of Primary Mediastinal B-Cell Lymphoma and Gray Zone Lymphoma.
Dunleavy K
Oncology (Williston Park) 2017 Jun 15;31(6):499-501. PMID: 28620906
Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association.
Sarkozy C, Molina T, Ghesquières H, Michallet AS, Dupuis J, Damotte D, Morsschauser F, Parrens M, Martin L, Dartigues P, Stamatoullas A, Hirsch P, Fabiani B, Bouabdallah K, da Silva MG, Maerevoet M, Laurent C, Coiffier B, Salles G, Traverse-Glehen A
Haematologica 2017 Jan;102(1):150-159. Epub 2016 Oct 6 doi: 10.3324/haematol.2016.152256. PMID: 27758822Free PMC Article
Clinicopathological analysis of mediastinal large B-cell lymphoma and classical Hodgkin lymphoma of the mediastinum.
Yamamoto W, Nakamura N, Tomita N, Ishii Y, Takasaki H, Hashimoto C, Motomura S, Yamazaki E, Ohshima R, Numata A, Ishigatsubo Y, Sakai R
Leuk Lymphoma 2013 May;54(5):967-72. Epub 2012 Nov 1 doi: 10.3109/10428194.2012.733881. PMID: 23020783
Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma.
Hutchinson CB, Wang E
Arch Pathol Lab Med 2011 Mar;135(3):394-8. doi: 10.5858/2009-0463-RSR.1. PMID: 21366467

Clinical prediction guides

Targeted Mutational Profiling Reveals Clonal Relationships in Metachronous Occurrence of Classic Hodgkin and Mediastinal Large B-Cell Lymphomas.
Singh K, Lezama LS, Kurzer J, Oak J, Schultz LM, Walkush A, Cheng TC, Chen EH, May WA, Chang C, Link MP, Advani RH, Suarez CJ, Natkunam Y
Am J Surg Pathol 2023 Jan 1;47(1):81-90. Epub 2022 Aug 25 doi: 10.1097/PAS.0000000000001956. PMID: 36001451
The immunophenotypic spectrum of primary mediastinal large B-cell lymphoma reveals prognostic biomarkers associated with outcome.
Bledsoe JR, Redd RA, Hasserjian RP, Soumerai JD, Nishino HT, Boyer DF, Ferry JA, Zukerberg LR, Harris NL, Abramson JS, Sohani AR
Am J Hematol 2016 Oct;91(10):E436-41. Epub 2016 Aug 4 doi: 10.1002/ajh.24485. PMID: 27419920
Recurrent mutations of the exportin 1 gene (XPO1) and their impact on selective inhibitor of nuclear export compounds sensitivity in primary mediastinal B-cell lymphoma.
Jardin F, Pujals A, Pelletier L, Bohers E, Camus V, Mareschal S, Dubois S, Sola B, Ochmann M, Lemonnier F, Viailly PJ, Bertrand P, Maingonnat C, Traverse-Glehen A, Gaulard P, Damotte D, Delarue R, Haioun C, Argueta C, Landesman Y, Salles G, Jais JP, Figeac M, Copie-Bergman C, Molina TJ, Picquenot JM, Cornic M, Fest T, Milpied N, Lemasle E, Stamatoullas A, Moeller P, Dyer MJ, Sundstrom C, Bastard C, Tilly H, Leroy K
Am J Hematol 2016 Sep;91(9):923-30. Epub 2016 Jul 4 doi: 10.1002/ajh.24451. PMID: 27312795
Methylation profiling of mediastinal gray zone lymphoma reveals a distinctive signature with elements shared by classical Hodgkin's lymphoma and primary mediastinal large B-cell lymphoma.
Eberle FC, Rodriguez-Canales J, Wei L, Hanson JC, Killian JK, Sun HW, Adams LG, Hewitt SM, Wilson WH, Pittaluga S, Meltzer PS, Staudt LM, Emmert-Buck MR, Jaffe ES
Haematologica 2011 Apr;96(4):558-66. doi: 10.3324/haematol.2010.033167. PMID: 21454882Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...