Primary follicular lymphoma arising in the ampulla of Vater

Abstract

Primary gastrointestinal lymphomas constitute more than one-third of all extranodal lymphomas, but among them follicular lymphomas (FL) are infrequent, accounting for only 1-4%. We report the case of a 55-year-old man diagnosed with a FL of the ampulla of Vater. He complained of dyspepsia. An upper gastroendoscopic study 1 year before showed a slight enlargement of the papilla with the biopsy initially interpreted as atypical follicular hyperplasia. The study performed because of persistence of the symptoms again showed an enlarged papilla, and the histological examination was diagnostic of FL (CD10+, bcl-2+). The full staging study did not show other FL involvement. The patient was treated with six courses of cyclophosphamide, hydroxydaunomycin, vincristine, and prednisone (CHOP), and the endoscopy as well as the biopsies revealed no abnormalities since the third course of chemotherapy. After 2 years of follow-up he remains asymptomatic and the gastroendoscopic study is normal.

Clinical Features of Gastrointestinal Follicular Lymphoma: Comparison with Nodal Follicular Lymphoma and Gastrointestinal MALT Lymphoma

“…In spite of the initial complete remission (CR) in the advanced disease following single alkylating agents, combination chemotherapy, or combined modality treatment, the relapse of the disease at a certain rate is observed [5,6] and the long-term prognosis is bad. Recently, there have been several reports of duodenal FLs [7,8,9,10,11,12,13,14], and most reported cases are confined to this region. Localized indolent lymphomas are rare except for mucosa-associated lymphoid tissue (MALT) lymphomas, which are extranodal lymphomas.…”

Hallazgo endoscópico de linfoma folicular primario

“…The median survival is 10 years after diagnosis, though in a recent multicenter, retrospective study male sex and the presence of abdominal symptoms were independently associated with worse clinical outcomes (1). Follicular lymphoma accounts for 1% to 3% of primary gastrointestinal lymphomas, and endoscopically appears in the form of small intestinal whitish smooth nodules as in our case (3). Follicular lymphoma can be part of a spectrum of disease called multiple lymphomatous polyposis, which is characterized by the formation of multiple mucosal nodules on various regions of the gastrointestinal tract including the esophagus, stomach, duodenum, and intestine.…”

A Rare Collision Tumor Composed of Follicular Lymphoma and Adenocarcinoma in the Ampulla of Vater: A Case Report

“…Primary GI-FLs usually arise in the small intestine, particularly in the terminal ileum [3], but duodenal FLs are rare. Three series of patients with duodenal FL [5–7] were recently reported as well as various previous case reports [1, 2, 8–11]. These reports characterized duodenal FLs as indolent and as having intermediate characteristics of MALT lymphomas and nodal follicular lymphomas [5–7].…”

“…Gastrointestinal (GI) primary lymphomas are the most frequent extranodal lymphomas, accounting for 30–40% [1]. The stomach is the most common site for lymphomas followed by the colorectal region and the terminal ileum [1]; the duodenum is less frequently affected [1].…”

“…The stomach is the most common site for lymphomas followed by the colorectal region and the terminal ileum [1]; the duodenum is less frequently affected [1]. Concerning histological subtypes, mucosa-associated lymphoid tissue (MALT) and high-grade B-cell lymphomas represent the vast majority, whereas follicular lymphomas (FLs) have a reported incidence of only 1.0–3.6% [2–4].…”