Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Gray-Zone Lymphoma: A Still Open Issue

Version 1 : Received: 17 May 2023 / Approved: 18 May 2023 / Online: 18 May 2023 (12:25:07 CEST)

A peer-reviewed article of this Preprint also exists.

Pileri, S.; Tabanelli, V.; Chiarle, R.; Calleri, A.; Melle, F.; Motta, G.; Sapienza, M.R.; Sabattini, E.; Zinzani, P.L.; Derenzini, E. Mediastinal Gray-Zone Lymphoma: Still an Open Issue. Hemato 2023, 4, 196-206. Pileri, S.; Tabanelli, V.; Chiarle, R.; Calleri, A.; Melle, F.; Motta, G.; Sapienza, M.R.; Sabattini, E.; Zinzani, P.L.; Derenzini, E. Mediastinal Gray-Zone Lymphoma: Still an Open Issue. Hemato 2023, 4, 196-206.

Abstract

The concept of gray-zone lymphoma (GZL) has been progressively refined since its in-troduction 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 mediasti-nal forms (PMBL). Officially recognized 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 lym-phoma and classical Hodgkin lymphoma”, it was limited to tumours, which showed either morphologic features reminiscent of classical HL (CHL) but carrying a complete B-cell pheno-type or conversely provided with a PMBL morphology but revealing CHL phenotypic charac-teristics. The definition of GZL has been further revised in the recently published International Lymphoma Classification and 5th Edition of the WHO Classification of Haemato-lymphoid Tu-mours, which have limited it to mediastinal neoplasms based on emerging molecular evidences. The aim of this review is to critically discuss the issue of GZL, also in the light of the suboptimal response to current therapies.

Keywords

mediastinal gray-zone lymphoma; classical Hodgkin lymphoma; primary mediastinal B-cell lymphoma; diffuse large B-cell lymphoma; NOS; EBV-positive diffuse large B-cell lymphoma; NOS; morphology; phenotype; gene expression profile; mutational landscape

Subject

Medicine and Pharmacology, Pathology and Pathobiology

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