Gastrointestinal Involvement in Mantle Cell Lymphoma: A Prospective Clinic, Endoscopic, and Pathologic Study

Abstract

The frequency of gastrointestinal (GI) tract involvement in mantle cell lymphoma (MCL) at diagnosis is reported to be below 30%. To investigate the actual frequency of GI involvement by MCL, upper and lower endoscopy was prospectively performed on 13 untreated MCL patients at diagnosis. Multiple biopsies from endoscopically normal and abnormal gastric and colonic mucosa were studied with immunohistochemistry (IHC) for CD20, CD5, and cyclin D1, as well as fluorescence in situ hybridization (FISH) for t(11;14) and polymerase chain reaction (PCR) for immunoglobulin heavy chain gene. Abnormal mucosa was identified in 38% of cases by upper endoscopy (mainly mild nonspecific gastritis) and in 54% of cases by lower endoscopy (mostly micropolyps). Histologically, infiltration by MCL was demonstrated in the stomach in 77% of cases and in the colon in 77% of cases. As a whole, 92% of patients showed upper or lower GI tract infiltration by MCL. Histologic evidence of MCL involvement was present in all cases with endoscopically abnormal mucosa, but it was also observed in two-thirds of cases with endoscopically unremarkable mucosa. Positive cyclin D1 IHC was seen in all instances displaying CD20 and CD5-positive lymphoid infiltrates, whereas t(11;14) was demonstrated by FISH in 63.5% and PCR was clonal in 64% of those instances. In conclusion, the great majority of MCL patients showed GI tract involvement at the time of diagnosis, not uncommonly in the form of minute lymphoid infiltrates. IHC for cyclin D1 was significantly more sensitive than FISH t(11;14) or PCR for immunoglobulin heavy chain gene to confirm MCL in this setting.

Lymphoproliferative Disorders of the Gastrointestinal Tract: A Review and Pragmatic Guide to Diagnosis

“…46 Approximately 25% of patients with documented nodal mantle cell lymphoma have gastrointestinal symptoms, yet the lymphoma is present histologically in the lower gastrointestinal tract in 77% to 88% of patients and in the upper gastrointestinal tract in 43% to 77%. 51,52 Moreover, microscopic documentation of mantle cell lymphoma is reported in 84% of patients with normal visual findings by lower endoscopy and in 45% of patients with macroscopically normal findings by upper endoscopy. 51 Such examples can be histologically indistinguishable from focal lymphoid hyperplasia, but immunohistochemical stains, including nuclear cyclin D1 reactivity, confirm gastrointestinal involvement by mantle cell lymphoma.…”

Mantle Cell Lymphoma Presenting as a Gastric Mass & Multiple Lymphomatous Polyposis of the Duodenum: A Case Report and Review of the Literature

“…The frequency of GI involvement in MCL has been previously underestimated, with previous reports citing 30% of MCL cases (by EGD and colonoscopy) [13]. But with more recent studies, GI tract infiltration has been shown in up to 92% of cases microscopically (from biopsies of lymphoid aggregates in normal and abnormal mucosae) [10]. Upper GI tract involvement is more common than colonic [6].…”

“…In our case, the polypoid lesions in the duodenum were most probably MLP. Recent reports have endoscopically revealed that 46%-49% of MCL patients had esophago-gastroduodenal involvement, and that 38%-62% had colorectal involvement [10,13]. Endoscopic findings of the upper and lower GI tract in MCL have included inflammation, nodules, polyps, ulcers, thickened walls, and masses [13].…”

“…Pathogenesis is unknown, but a susceptible genetic background, a chemotherapeutic regimen or ionized radiation could contribute to the development of MCL [9]. In up to 80% of cases of MCL, patients have involvement of extranodal sites and organs like the spleen, bone marrow, peripheral blood, gastrointestinal tract (GI), etc [10]. Furthermore, involvement of more than 2 extra-nodal sites is seen in 30 to 50% of MCL patients [11].…”

Sometimes double negative is positive

“…The patient's abdominal pain may be attributed to a lymphoproliferative process such as mantle cell lymphoma, which involves the gastrointestinal tract in the majority of cases. B symptoms are present in 30% of patients (8,9) and splenomegaly may be massive. Cytopenias are usually secondary to BM infiltration, typically seen in advanced disease.…”