Unusual variants of mantle cell lymphoma

Abstract

Mantle cell lymphoma (MCL) is recognized as an aggressive and incurable small B-cell lymphoma and represents a group of t(11;14)(q13;q32)/CCND1+ neoplasms with a wide variety of morphology. Thus, the identification of various MCL variants is important for guiding appropriate therapies and predicting outcomes. Histopathologic diagnosis of classical MCL is usually straightforward due to its typical morphology and nuclear overexpression of cyclin D1. However, approximately 10% of the MCL are represented by its own morphologic variants, mimicking other lymphomas, including the challenging cyclin D1-negative MCL. Furthermore, typical MCL immunophenotype and genetic abnormalities may not be found in every case. Therefore, MCL variants may be a diagnostic trap for hematopathologists. Taking into account that histopathological diagnosis remains the gold standard for MCL diagnosis, this paper summarizes the practical approaches to the diagnosis of the uncommon variants of MCL in accordance with the 2016 revised WHO classification of lymphoid neoplasms.

Keywords

Mantle cell lymphoma, lymphoma variants, cyclin D1, Sox11, in situ mantle cell neoplasia, double hit lymphoma

How to Cite

Fratoni, S., (2017) “Unusual variants of mantle cell lymphoma”, Hematopathology 2(1), p.31-49.

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Authors

Stefano Fratoni (St. Eugenio Hospital of Rome, Italy)

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Creative Commons Attribution-NonCommercial 4.0

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This article has been peer reviewed.

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