Primary Follicular Lymphoma of the Gastrointestinal Tract

The James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
The American journal of surgical pathology (Impact Factor: 5.15). 09/2011; 35(9):1255-63. DOI: 10.1097/PAS.0b013e318224e661
Source: PubMed


Follicular lymphoma (FL), a common nodal lymphoma, is rare in the gastrointestinal (GI) tract. We report our experience with primary FL of the GI tract.
The surgical pathology computer files at the Massachusetts General Hospital were searched for cases of FL involving the GI tract. Patients were included if on staging, the major site of disease was the GI tract. Thirty-nine cases were identified. Clinical data were collected from electronic medical records.
The 27 women and 12 men ranged in age from 29 to 79 years (median, 59 y). Thirty tumors involved the small bowel (19 the duodenum); 8 involved the colon; and 1 involved the stomach. Eight of 10 tumors that were resected involved the small bowel (jejunum and/or ileum without duodenum) of which 5 presented with intestinal obstruction. All tumors were grade 1 or 2. Immunostains showed consistent expression of CD20 (100%), CD10 (97%), and Bcl-2 (97%). Among the 34 cases with Ann Arbor staging information, 22 were stage I, 10 were stage II, and 2 were (6%) stage IV. Of 36 cases with follow-up (median, 4.5 y), 27 patients are alive without disease, 7 are alive with disease, and 2 died of other causes. No lymphoma-related deaths were recorded.
Primary FL of the GI tract occurs most often in middle-aged adults with a 2:1 female preponderance. The most frequent site of involvement is the duodenum, followed by the ileum and colon. Distal small bowel involvement is more likely to present as bowel obstruction requiring resection. The disease is localized in the bowel and regional lymph nodes in the vast majority of cases. The prognosis is favorable even when the disease is disseminated.

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    • "Schmatz et al. reported that 2 of 24 previously untreated patients had developed nodal disease approximately 5 years after diagnosis [13]. A case series described by Misdraji et al. included five patients with stage I intestinal follicular lymphomas who were assigned to a watch-and-wait strategy [14]. Two of the five patients developed nodal disease 2 years and 4 years after diagnosis, respectively. "
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    ABSTRACT: A 62-year-old Japanese male was diagnosed with primary intestinal follicular lymphoma involving the duodenum, jejunum, and rectum without lymph node involvement. The patient was classified as low risk by the follicular lymphoma international prognostic index (FLIPI) system. Treatment was deferred because he had no symptoms. Eleven months after the diagnosis, his soluble interleukin-2 receptor (sIL-2R) levels had risen from 383 to 617 U/mL. Lymphoma progression involving an enlarged perigastric lymph node was also documented. This report illustrates a case of rapidly progressed intestinal follicular lymphoma, suggesting the possible usefulness of sIL-2R levels as an indicator of lymphoma progression.
    Case Reports in Oncological Medicine 04/2014; 2014:549248. DOI:10.1155/2014/549248
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    • "Our study is also consistent with a recent study of primary GI FL from France by Damaj et al., which showed that primary FL of the GI tract tends to be a disease of the small intestine [31]. Similar findings were reported in a recent large US study which revealed small intestinal FLs made up 63% of cases while gastric FLs accounted for only 3% of primary gastrointestinal FLs [32]. Similarly, 14 of the 25 (56%) of primary FLs in our series were found in the small intestine. "
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    ABSTRACT: BackgroundGastrointestinal (GI) lymphomas are very common types of extranodal lymphomas, and we hypothesize there are regional differences in subtype, distribution in the GI tract, and epidemiological features among the different populations.MethodsWe retrospectively evaluated the clinical, molecular and histologic features of North American primary and secondary GI lymphomas diagnosed from 2000–2009 seen at our institution. We utilized immunohistochemistry and fluorescence in situ hybridization to further evaluate a subset of the gastric lymphomas.ResultsExtranodal marginal zone lymphomas of mucosal associated lymphoid tissue (MALTs) and diffuse large B cell lymphomas (DLBCLs) were the most common subtypes of GI lymphomas. Select gastric DLBCLs (N = 6) and MALTs (N = 13) were further examined for API2-MALT1 and IGH translocations, and P16 and P53 protein expression. Gastric MALTs showed frequent API2-MALT1 (38%) but not IGH translocations (0%), and the DLBCLs showed neither translocation. Expression of P16 and P53 proteins and the proliferative index were compared between high grade gastric lymphomas (gastric DLBCLs) and low grade gastric lymphomas (gastric MALTs). P53 overexpression (P = 0.008) and a high proliferation index [Ki-67] (P = 0.00042) were significantly associated with gastric DLBCL, but no statistically significant difference was observed in P16 expression (p = 0.108) between gastric DLBCL and gastric MALT.ConclusionOur study revealed that GI lymphomas from a Central-Midwestern North American population showed differences and similarities to non-North American cohorts. In addition, API2-MALT1, P16 and P53 abnormalities occurred frequently in gastric lymphomas from this North American population.Virtual slidesThe virtual slides for this article can be found here:
    Diagnostic Pathology 06/2012; 7(1):76. DOI:10.1186/1746-1596-7-76 · 2.60 Impact Factor
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    ABSTRACT: La localisation duodénale des lymphomes folliculaires est considérée comme rare, mais l’incidence de ces tumeurs est en augmentation. Nous rapportons une observation de lymphome primitif du duodénum, montrant la similitude des données endoscopiques avec celles de la papulose lymphomateuse des lymphomes du manteau. Les techniques immunohistochimiques et cytogénétiques aident à différencier ces deux tumeurs. Il est essentiel de les différencier car leur pronostic et les modalités de traitement sont différents. The duodenal localization of follicular lymphoma is considered to be rare, but the incidence of these tumors is rising. We report a case of a primary follicular lymphoma of the duodenum, showing that the endoscopic findings are often similar to those found in lymphomatous polyposis associated with mantle-cell lymphomas. The immunohistochemical and cytogenetic techniques help differentiate between these two conditions. It is essential to differentiate between these two forms of non-Hodgkin lymphomas, as they do not carry the same prognosis and the therapeutic protocols.
    Acta Endoscopica 06/2005; 35(3):395-400. DOI:10.1007/BF03003289 · 0.16 Impact Factor
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