Mediastinal Gray-Zone Lymphoma: Still an Open Issue

Author:

Pileri Stefano12ORCID,Tabanelli Valentina1,Chiarle Roberto13,Calleri Angelica1,Melle Federica1ORCID,Motta Giovanna1,Sapienza Maria Rosaria1ORCID,Sabattini Elena4ORCID,Zinzani Pier Luigi25ORCID,Derenzini Enrico67

Affiliation:

1. Division of Haematopathology, European Institute of Oncology IRCCS, Via Ripamonti 435, 20141 Milan, Italy

2. Department of Medical and Surgical Sciences, Bologna University, Via Massarenti 9, 40138 Bologna, Italy

3. Department of Biomedical Sciences and Human Oncology, Turin University, Via Santena 7, 10126 Turin, Italy

4. Unit of Haematopathology, University-Hospital S. Orsola Polyclinic IRCCS, Via Massarenti 9, 40138 Bologna, Italy

5. Institute of Haematology “L. and A. Seràgnoli”, University-Hospital S. Orsola Polyclinic IRCCS, Via Massarenti 9, 40138 Bologna, Italy

6. Division of Haemato-Oncology, European Institute of Oncology IRCCS, Via Ripamonti 435, 20141 Milan, Italy

7. Department of Health Sciences, University of Milan, Via di Rudinì 8, 20146 Milan, Italy

Abstract

The concept of gray-zone lymphoma (GZL) has been progressively refined since its introduction in the literature in 1998. For several years, it was applied to a rather broad spectrum of conditions, posing the problem of the differential diagnosis between any type of Hodgkin lymphoma (HL) and diffuse large B-cell lymphoma, with special reference to primary mediastinal forms (PMBL). Officially recognised as a provisional entity in the 4th and revised 4th editions of the WHO Classification of Tumour of Haematopoietic and Lymphoid Tissues with the term “B-cell lymphoma unclassifiable with features intermediate between diffuse large B-cell lymphoma and classic Hodgkin lymphoma”, it was limited to tumours showing either morphologic features reminiscent of classic HL (CHL) but carrying a complete B-cell phenotype or conversely provided with a PMBL morphology yet revealing CHL phenotypic characteristics. The definition of GZL has been further revised in the recently published International Lymphoma Classification and 5th edition of the WHO Classification of Haematolymphoid Tumours, which have limited it to mediastinal neoplasms (MGZL) based on emerging molecular evidence. The aim of this review is to critically discuss the issue of MGZL, as well as in light of the suboptimal response to current therapies.

Funder

AIRC: Italian Association for Cancer Research

Publisher

MDPI AG

Subject

General Earth and Planetary Sciences,General Environmental Science

Reference29 articles.

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2. Report: Workshop on mediastinal gray zone lymphoma;Poppema;Eur. J. Haematol.,2005

3. Molecular diagnosis of primary mediastinal B cell lymphoma identifies a clinically favorable subgroup of diffuse large B cell lymphoma related to Hodgkin lymphoma;Rosenwald;J. Exp. Med.,2003

4. Shades of gray between large B-cell lymphomas and Hodgkin lymphomas: Differential diagnosis and biological implications;Harris;Mod. Pathol.,2013

5. Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., Thiele, J., and Vardiman, J.W. (2008). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC press. [4th ed.].

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