Small bowel lymphoma - duodenum

Case contributed by Michael P Hartung
Diagnosis certain

Presentation

Abdominal pain. No history of cancer.

Patient Data

Age: 63 years
Gender: Male

There is an infiltrative, circumferential, and intermediate attenuation mass involving the second and third portions of the duodenum. The patient was administered oral contrast, and it is clear that this mass is not obstructing the gastroduodenal outflow. There is a smaller soft tissue mass which extends from the duodenal mass into the right mid abdominal mesentery. There is obstruction of the common bile duct by the duodenal mass, with dilation of the gallbladder. No adenopathy or disease elsewhere in the abdomen or pelvis is present. 

MRCP images were performed prior to ERCP at the request of the GI service. There is dilation of the intrahepatic ducts, gallbladder, cystic duct, and common bile duct. There are no findings of obstructive stone. The distal CBD tapers abruptly near the papilla, and is obstructed due to the duodenal mass (either due to invasion or mass effect). The duodenal mass is again seen and better characterized on CT, with intermediate T2 signal. 

PATHOLOGY REPORT:

Specimen:

B - Duodenal bulb polyp and mass

D - Duodenal mass

Tissue "B" and "D" - Immunohistochemical evaluation, performed on specimen B, reveals the neoplastic large lymphoid cells to be immunoreactive for CD20, CD10, and BCL-6, without significant staining for BCL-2, MUM-1, CD30, or cyclin-D1 (BCL-1); there is patchy staining among the neoplastic cells for c-MYC, with some foci demonstrating staining of approximately 30% of the population. The Ki-67 (MIB-1) labeling index is approximately 70%. An immunostain for CD3 highlights background non-neoplastic small T lymphocytes. The neoplastic cells are negative for chromogranin and synaptophysin.

The constellation of features indicates a diffuse large B-cell lymphoma of germinal center B-cell type. The sampled biopsy material does not reveal an accompanying low-grade lymphomatous component.

Diagnoses:

Tissue "B" - Duodenal bulb polyp and mass, biopsy - Diffuse large B-cell lymphoma, not otherwise specified, germinal center B-cell type (see comment).

Tissue "D" - Duodenal mass, biopsy - Diffuse large B-cell lymphoma, not otherwise specified, germinal center B-cell type (see comment).

 

Case Discussion

This is a biopsy-proven case of primary B-cell lymphoma of the duodenum. The duodenal mass results in obstruction of the common bile duct. The patient was managed with stenting of the common bile duct and chemotherapy. 

Lymphoma is the most common malignancy of the small bowel, and the distal ileum is the most common site of B-cell lymphoma because of the higher concentration of lymphoid tissue. 

Small bowel lymphoma can present as a circumferential bulky mass which extends into the mesentery and regional lymph nodes. It characteristically dilates the lumen without obstruction the GI tract (obstruction is more associated with adenocarcinoma). 

The differential diagnosis in this case would be lymphoma, adenocarcinoma and gastrointestinal stromal tumor. A biopsy could be performed endoscopically at the time of biliary decompression or with ultrasound/CT guidance. 

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