A case report of the histologic transformation of primary follicular lymphoma of the duodenum

Abstract

A 46-year-old woman underwent upper endoscopy for evaluation of anemia, which revealed whitish granules at the duodenal papilla, diagnosed as duodenal follicular lymphoma (DFL) by biopsy. Computed tomography and abdominal ultrasonography revealed that follicular lymphoma was confined to the duodenum. Seven years after the diagnosis, fluorine-18 fluorodeoxyglucose positron emission tomography scanning revealed multiple lesions including in bone marrow and lymph nodes. Bone marrow biopsy of the right iliac bone revealed diffuse large B-cell lymphoma, indicating systemic dissemination and histologic transformation of the DFL. The patient responded to chemotherapy and has been progression-free for 2.5 years. Although DFL is usually indolent even without any treatment, systemic dissemination with histologic transformation can occur. This case suggests that the life-time follow-up that is usually done for patients with nodal follicular lymphoma should be provided to patients with DFL.

FIGURE 1

The series of endoscopic findings. At diagnosis of follicular lymphoma, the side-viewing endoscope revealed whitish granules around Vater papilla (white arrow) (A). At the time of transformation, whitish granules at Vater papilla increased in number and clustered together at Vater papilla (white arrow) (B). After 6 cycles of chemotherapy, the number of whitish granules decreased dramatically (white arrow) (C).

FIGURE 2

Endoscopic biopsy of granules around Vater papilla revealed the balloon-like villous hypertrophy caused by extrafollicular lymphoma cells (HE staining, Scale bar: 250 μm) (A). Biopsy of right iliac bone marrow showed sheets of large B cells (centroblasts), suggesting histologic transformation of follicular lymphoma into diffuse large B-cell lymphoma. Normal hematopoietic cells were replaced by lymphoma cells (HE staining, Scale bar: 50 μm) (B).

FIGURE 3

The result of ¹⁸F-FDG-PET scanning at transformation. ¹⁸F-FDG accumulated in the iliac bone, left pubic bone, thoracic vertebrae, right neck and flank, and right iliac lymph nodes. Red signals indicate accumulation of ¹⁸F-FDG, suggesting high tumor proliferation. The maximum standardized uptake value was 18.7 in the right iliac bone. (coronal view [A], sagittal view[B]). ¹⁸F-FDG-PET = fluorine-18 fluorodeoxyglucose positron emission tomography.