Abstract

Follicular lymphoma (FL) is one of the most common B-cell malignancies worldwide. While the diagnosis of conventional cases is straightforward, rare clinical–pathological variants may be challenging due to their misleading morphology, aberrant phenotype and/or atypical presentation. To add to the spectrum of unusual FLs, we report on a rare disease pattern characterized by (i) inguinal presentation, (ii) massive necrosis, (iii) Hodgkin/Reed–Sternberg (HRS)-like cells, and (iv) adjacent areas of diffuse large B-cell lymphoma evolution. All cases occurred in elderly patients (median age at diagnosis: 69.5 years), disclosed a low stage at diagnosis (Ann Arbor stage IA-IIA), and had deceiving clinical features. Despite the alarming histology, excellent responses to conventional therapies were reported in all patients. In conclusion, necrotizing FL of the inguinal region is a rare neoplasm characterized by peculiar clinical and histological features. This lymphoma should always be considered in the differential diagnosis of massively necrotic inguinal lesions.

Highlights

  • Follicular lymphoma (FL) is the second-most common non-Hodgkin lymphoma (NHL) worldwide, accounting for about 35% of all adult lymphoid neoplasms [1]

  • Epstein– Barr virus (EBV) status was assessed by chromogenic in situ hybridization (CISH; Leica Biosystems) for EBV-encoded small RNAs (EBER)

  • Three patients had unremarkable past medical history, while one was affected by chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and had a remote diagnosis of grade 1 FL of the left arm

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Summary

Introduction

Follicular lymphoma (FL) is the second-most common non-Hodgkin lymphoma (NHL) worldwide, accounting for about 35% of all adult lymphoid neoplasms [1]. The clinical and histological features of FL are greatly variable. Most cases present with nodal disease and are histologically characterized by a nodular to nodular and diffuse proliferation of atypical B cells with germinal center (GC) phenotype. The neoplastic population includes small- to medium-sized centrocytes and variable numbers of large nucleolated centroblasts. Over 85% of FLs carry the t (14;18) (q32;q21), which leads to the juxtaposition of the BCL2 and IGH genes and to the overexpression of the anti-apoptotic protein Bcl2 [1]. This genetic event confers a survival advantage to the B cells and (together with other molecular derangements) promotes neoplastic transformation [1]

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