Abstract

BackgroundInterpretation of mediastinal biopsy is often challenging even for experienced pathologists especially when a hematolymphoid neoplasm is suspected. Primary mediastinal large B-cell lymphoma (PMLBCL) and classic Hodgkin lymphoma (CHL) represent two major types of mature B-cell lymphomas of the mediastinum. Although PMLBCL and mediastinal CHL share many clinicopathologic characteristics, their treatment strategies and responses are remarkably different. We therefore aimed to find distinctive histologic or protein markers to better differentiate these two lesions.MethodsSearch for primary mediastinal B-cell lymphomas found 52 consecutive cases from 3 university hospitals of Korea during 2005 to 2012. Among them, 32 cases that were available for additional immunohistochemistry (IHC) assessment were enrolled in this study. These cases consisted of the following: CHL (N = 13), PMLBCL (N = 16), and B-cell lymphoma unclassifiable, with features intermediate between diffuse large B-cell lymphoma and CHL (gray zone lymphoma, N = 3). Along with the clinicopathologic findings, the expression of p63, GATA3 and cyclin E was investigated by IHC in the three categorized lesions mentioned above.ResultsMost clinical features overlapped between PMLBCL and CHL except for the increased disease progression and mortality found in PMLBCL. In the pathologic review, the presence of epithelioid granuloma favored a diagnosis of CHL, whereas reticulated or alveolar patterns of fibrosis were characteristic of PMLBCL. For protein markers, p63 was predominantly positive in PMLBCL (15/16) compared with CHL (2/13), which indicates that p63 is a marker of the highest diagnostic accuracy when calculated by the area under the ROC curve. GATA3 was expressed in the majority of CHL cases (10/13) compared with PMLBCL (0/16), while the expression of cyclin E was only rarely present in a minor population of PMLBCL.ConclusionsP63 expression in tumor cells, even focal expression, and no GATA3 is the most helpful feature in distinguishing PMLBCL from mediastinal CHL.

Highlights

  • Interpretation of mediastinal biopsy is often challenging even for experienced pathologists especially when a hematolymphoid neoplasm is suspected

  • 32 cases were selected for this study: Primary mediastinal large B-cell lymphoma (PMLBCL) (N = 16), classic Hodgkin lymphoma (CHL) (N = 13), B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (N = 3)

  • Three patients with gray zone lymphoma were treated with the R-CHOP regimen because all had been previously diagnosed with DLBCL; the diagnosis was revised to gray zone lymphoma after review at the consensus meeting

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Summary

Introduction

Interpretation of mediastinal biopsy is often challenging even for experienced pathologists especially when a hematolymphoid neoplasm is suspected. Primary mediastinal large B-cell lymphoma (PMLBCL) and classic Hodgkin lymphoma (CHL) represent two major types of mature B-cell lymphomas of the mediastinum. PMLBCL and mediastinal CHL share many clinicopathologic characteristics, their treatment strategies and responses are remarkably different. We aimed to find distinctive histologic or protein markers to better differentiate these two lesions. Lymphomas of the mediastinum can originate either from the thymus or the lymph nodes, and thymic lymphomas show unique pathologic characteristics distinct from common nodal lymphomas [2]. PMLBCL and classic Hodgkin lymphoma (CHL) constitute two major lymphomas of mediastinal B-cell origin, other types of B-cell lymphomas such as marginal zone lymphoma do occur at a low incidence. Differentiation of PMLBCL from CHL or other B-cell lymphomas is crucial even in small specimens, because they each have different therapeutic strategies [5] and outcomes.

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