Presentation
Right upper quadrant abdominal pain with distension.
Patient Data
There is diffuse wall thickening of the small bowel mainly involving the jejunum and the ileum. There are no findings of obstruction. There are multiple enlarged mesenteric lymph nodes.
Case Discussion
This is a case of small bowel Burkitt lymphoma.
A fine needle aspiration of a periportal lymph node was performed. The neoplastic cells appeared medium-sized on hematoxylin and eosin stain with inconspicuous but sometimes noticeable nucleoli and hazy chromatin. There appeared to be apoptotic debris and possible focal starry sky appearance. The neoplastic cells appeared to be positive for EBER ISH, MYC (>80%), ki-67 (nearly 100%), HGAL, CD20, CD79a, PAX5, CD10 and BCL6. FISH Kappa and ISH lambda showed a lambda/kappa ratio greater than 10:1. BCL2 was essentially negative in neoplastic cells, with a subset of neoplastic cells exhibiting very dim reactivity versus artifact/background staining.
Intradepartmental review of the histopathology revealed findings most compatible with Burkitt lymphoma, with potential differentials including EBV-positive diffuse large B-cell lymphoma or high grade B-cell lymphoma with EBV positivity (double- or triple-hit). Further supplementary testing was recommended.
Supplementary FISH demonstrated findings consistent with MYC amplification and t(8;14) translocation. There was no evidence for a double or triple hit lymphoma. In light of this FISH result, the diagnosis was confirmed to represent Burkitt lymphoma.
The patient began chemotherapy shortly after the diagnosis.
Co-authors:
Sanket Desai
Peter Kim, MD
Unable to process the form. Check for errors and try again.