High-Grade B-Cell Lymphoma Not Otherwise Specified (HGBL, NOS) in the Maxillary Sinus Mimicking Periapical Inflammation: Case Report and Review of the Literature

Andrea Brody,Csaba Dobo-Nagy,Zsuzsanna Oltyan,Karoly Mensch,Judit Csomor,Adrienn Dobai,Mariana Pacurar

doi:10.3390/app11198803

Andrea Brody, Csaba Dobo-Nagy + Show 5 more

Open Access

https://doi.org/10.3390/app11198803

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Journal: Applied Sciences	Publication Date: Sep 22, 2021
Citations: 1	License type: CC BY 4.0

Affiliation: Semmelweis University

Abstract

High-grade B-cell lymphoma not otherwise specified is listed as a new group in the WHO 2017 statement as a subtype of aggressive, mature B-cell lymphomas with a poor prognosis. To our knowledge, no description of this genetic type of maxillary lymphoma has appeared in the literature until now; thus, our case provides valuable data on its symptoms, clinical behavior, response to treatment and survival rate. The present report describes the early diagnosis and treatment of an extremely rare histological subtype of B-cell lymphoma, a case of high-grade B-cell lymphoma not otherwise specified, localized in the maxillary sinus and mimicking signs and symptoms of periapical inflammation. After chemotherapy, the presented patient showed complete remission without relapse and systemic spread. As far as we know, this is the first reported case of this rare type of lymphoma associated with the maxillary sinus. Considering that high-grade B-cell lymphomas are aggressive tumors with rapid growth and poor prognosis, which are often misdiagnosed in the early stages as inflammatory disease, it is relevant to highlight the importance of a detailed evaluation of clinical signs and radiological findings during diagnosis, especially if they contradict each other.

Highlights

Non-Hodgkin lymphoma (NHL) represent less than 1% of all malignant tumors in the head and neck region, they represent the second most common localization in the gastrointestinal tract [1,2]
Since very little data are available on the pathogenesis and clinical behaviour of high-grade B-cell lymphoma (HGBL), NOS
There are few reports of NHL in the maxillary sinus [9,31,32], but, to our knowledge, the present case represents the first description of HGBL, NOS located in the maxillary sinus

Summary

Introduction

Non-Hodgkin lymphoma (NHL) represent less than 1% of all malignant tumors in the head and neck region, they represent the second most common localization in the gastrointestinal tract [1,2]. In exceedingly rare cases the lymphoma is not attributed to BL, DLBCL or any other form of HGBL, with MYC translocation and BCL-2 or BCL-6 amplification These lymphomas are not double hit, but double-expressor lymphomas, with a poor prognosis [11,12,17]. The expression of BCL-6, a transcriptional repressor, helps in the development of lymphoma [15,18] Both subtypes, especially the HGBL, NOS, are rarely diagnosed [12,19], and while other mature B-cell lymphomas such as DLBCL have an average of 70% complete remission and a high average 5-year survival rate of 60% [1,20], the prognosis of HGBL, NOS is poor [14,21,22,23]. The report provides relevant information about the clinical features and therapeutic efficacy of the HGBL, NOS in the maxillary sinus and the difficulty of distinguishing the disease from a periapical inflammation

Case Report

Treatment

Findings

Discussion