| Summary: | 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.
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