Primary Gastrointestinal Follicular Lymphomas: A Prospective Study of 31 Patients with Long-term Follow-up Registered in the French Gastrointestinal Lymphoma Study Group (GELD) of the French Federation of Digestive Oncology (FFCD)

Abstract

Background/Aims: Primary gastrointestinal follicular lymphomas (PGFL) are very rare. Our aim was to analyze the clinical features, management, and long-term outcomes in a prospective series of patients diagnosed with PGFL. Methods:All adult patients with PGFL, consecutively enrolled into the multicenter French study between 1990 and 2017, were evaluated and followed up prospectively after undergoing a complete work-up. Clinical, pathological and endoscopic features, as well as treatment outcomes, were analyzed.Results: Thirty-one patients (16 men, median age 62 years, range 33 to 79 years) with PGFL were included. The median follow-up was 92 months (range, 6 to 218 months). In the majority of patients (n=14), lymphoma was incidentally diagnosed during endoscopy. Otherwise, the most frequent circumstances of diagnosis were abdominal pain (n=7) and dyspepsia (n=5). The duodenum was the most common site of involvement (n=19) and multifocal localizations were seen in seven patients (22%). The most frequent first line strategy was surveillance applied in 22 patients (71%), of whom nine reached spontaneous, complete remission and 11 had stable disease. Seven patients (23%) received chemotherapy as first line treatment, and two underwent resection. Of seven patients who received chemotherapy, four achieved complete remission. In three patients, transformation into a high-grade lymphoma occurred. Conclusions:The diagnosis of PGFL is frequently fortuitous. The most common localization is in the duodenum. The disease has an indolent course and a good prognosis, however, rare cases of transformation into aggressive high-grade lymphoma may occur. An appropriate characterization and follow-up of these lymphomas is mandatory for their optimal management. (Gut Liver,

Epidemiological features and prognosis for primary gastrointestinal follicular lymphoma

“…13,21 In contrast to N-FL's transformation propensity, the rate of which is estimated at 2%-3% per year, it is very rare for patients with PGI-FL to undergo histological transformation, and to our knowledge, only a few cases have been reported. 11,[22][23][24] In our study, the 5-year transformation rates of PGI-FL and N-FL were 2.1% and 2.6% respectively. In addition, PGI-FL patients reached a transformation plateau at 15 years, but N-FL patients did not.…”

“…Histological transformation is defined as an alteration of histology from FL to high‐grade lymphoma, such as DLBCL (rarely lymphoblastic lymphoma), which is associated with a poor prognosis 13,21 . In contrast to N‐FL's transformation propensity, the rate of which is estimated at 2%–3% per year, it is very rare for patients with PGI‐FL to undergo histological transformation, and to our knowledge, only a few cases have been reported 11,22–24 . In our study, the 5‐year transformation rates of PGI‐FL and N‐FL were 2.1% and 2.6% respectively.…”

Long-term outcomes of patients with primary intestinal follicular lymphoma managed with watch-and-wait strategy

“…The 5-year overall survival rate of the sample (125 patients) was 100%, and the 5-year progression-free survival rate was 93%. A prospective observational study on intestinal follicular lymphoma was recently conducted by Matysiak-Budnik et al 20 . Among 31 patients with stage IE (n = 23), IIE (n = 3), and IV disease (n = 5), 22 patients with stage IE disease were managed with the watch-and-wait strategy.…”

“…19 Department of Gastroenterology, Sumitomo Besshi Hospital, 3-1 Ojicho, Niihama, Ehime 792-8543, Japan. 20 Department of Internal Medicine, St. Mary's Hospital, 650 Nibuno, Himeji, Hyogo 670-0801, Japan. 21 Department of Gastroenterology, National Hospital Organization Iwakuni Clinical Center, 1-1-1 Atago-cho, Iwakuni, Yamaguchi 740-8510, Japan.…”

A Rare Case of Primary Duodenal Follicular Lymphoma

“…The most frequently involved sites of primary follicular lymphoma are the second part of the duodenum, followed by the jejunum [5]. A typical endoscopic finding is a whitish multinodular mucosal or polypoid lesion of 1-5 mm in size [3].…”