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
Summary
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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