Abstract
Duodenal-type follicular lymphoma (DFL) is a rare variant of follicular lymphoma (FL) characterized by distinctive clinical features such as localization and favorable prognosis. We herein report a case of DFL in which histological transformation into diffuse large B-cell lymphoma developed 7 years after diagnosis. The transformed lymphoma was refractory to chemotherapy, and the patient passed away due to disease progression. To date, there have been only a limited number of reported cases of histological transformation of DFL, and the clinical outcomes of those cases except our present case have been favorable, with good responses to chemotherapy. Although the histological transformation of DFL is a rare event, the clinical course of the present case suggested that it would be a fatal event and underscore the importance of the life-long management of DFL. Further accumulation of cases is required to elucidate its incidence, characteristics, and prognosis.
Highlights
Follicular lymphoma (FL) is a common type of indolent B-cell lymphoma
Several investigators have consistently reported that Duodenal-type follicular lymphoma (DFL), which is predominantly confined to the second portion of the duodenum, could involve the distal small intestine and could present a notably indolent course [3,4,5,6]
We identified 7 additional reported cases of histological transformation of DFL (Table 1)
Summary
Follicular lymphoma (FL) is a common type of indolent B-cell lymphoma. FL could primarily or secondarily involve extranodal sites, including gastrointestinal tracts. We here report a case of DFL transforming into diffuse large B-cell lymphoma more than 7 years after diagnosis, whose clinical course was fatal after histological transformation. Histological findings of the sample obtained from the abdominal tumor by CT-guided biopsy showed the diffuse proliferation of large atypical lymphoid cells, which were positive for CD20, CD10, bcl, bcl, and negative for CD3 and Cyclin D1 (Figure 4). Both IGH-BCL2 and MYC rearrangements were positive by FISH analysis. Local field radiotherapy was given, but the response was poor He succumbed due to disease progression 7 months after the histological transformation and 8 years after the diagnosis of DFL
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