Primary diffuse large B-cell lymphoma of ampulla of Vater: A rare case report

Introduction: Primary lymphomas of the ampulla of Vater are extremely rare. Among these most frequent are follicular lymphomas followed by mucosa-associated lymphoid tissue-type lymphoma. Case Report: We report a case of a 36-year-old female diagnosed with diffuse large B-cell lymphoma (DLBCL) of the ampulla of Vater. Endoscopic biopsy was non-diagnostic. Whipple's resection was performed. The patient was given six cycles of chemotherapy. One year later, the patient developed recurrence at the anastomotic site and again treated with combination chemotherapy. Patient is doing well after 2 years of initial surgery. Discussion: Tumors of the ampulla of Vater are included in the pancreaticobiliary group of neoplasms; in some instances, it is almost impossible to ascertain the exact origin of the tumor anatomically. The patients of primary lymphoma of the ampulla of Vater may have a varied clinical presentation or may be completely asymptomatic and detected during routine medical check-ups for gastric cancer screening. Conclusion: We present a rare case of primary DLBCL of the periampullary region. Due to the rarity of these lesions, their ability to masquerade as periampullary carcinomas, and the fact that these tumors show a good response to chemotherapy, an early diagnosis is desirable. © Springer Science+Business Media, LLC 2010.