A case of colonic mantle cell lymphoma: new insights in a rare disease

Table of Contents

An 82-year-old man was referred to our outpatient clinic because of a change in bowel habits and involuntary weight loss. His medical history consisted of atrial fibrillation. A few weeks before presentation he had suffered a varicella zoster infection of his face. A colonoscopy was performed to rule out colorectal carcinoma. Dozens of small sessile polyps, varying in size, were found in the entire colon ([Fig. 1]). The mucosa in the rectosigmoid region was diffusely swollen and polypous with multiple superficial ulcerations ([Fig. 2]). Biopsies of proximal and distal lesions revealed a mononuclear infiltration between muscularis mucosae and otherwise normal-appearing crypts. Lymphocytes showed nuclear irregularity. Immunohistochemistry showed positivity for CD5, CD20, CD43, CD79a, Bcl-2, and cyclin-D1 ([Fig. 3]). Stainings for CD10, CD15, CD30, CD138, IgA, IgG, and IgM were negative. This profile supports the diagnosis of a mantle cell lymphoma (MCL). Unfortunately, the patient suffered an ischemic cerebrovascular accident (CVA) the day after colonoscopy, 6 days after stopping anticoagulants temporarily, leaving him hemiplegic and bedridden. For this reason, it was decided to refrain from starting systemic chemotherapy. His recent varicella zoster infection was considered a paraneoplastic phenomenon. The patient died 6 weeks after diagnosis of complications of his CVA.

Fig. 1 Proximal colon.

Fig. 2 Rectosigmoid region.

Fig. 3 Histology showing positivity for cyclin-D1 (× 400).

MCL is a non-Hodgkin’s B-cell lymphoma. The reported frequency of gastrointestinal tract involvement in patients with MCL is 15 % – 30 % [1]. However, this is most likely an underestimate because most patients with MCL involving the gastrointestinal tract previously reported were examined endoscopically only if they had gastrointestinal symptoms [2]. Systemic chemotherapy is the mainstay of treatment, usually consisting of cyclophosphamide, adriamycin, vincristine, and prednisone (CHOP) plus rituximab. More aggressive treatment regimens have been proposed but according to recent data do not increase survival [3]. Median overall survival after diagnosis is 7 years.

Endoscopy_UCTN_Code_CCL_1AD_2AC

References

• 1 Romaguera J E, Medeiros L J, Hagemeister F B. et al . Frequency of gastrointestinal involvement and its clinical significance in mantle cell lymphoma.  Cancer. 2003;  97 586-591
• 2 Salar A, Juanpere N, Bellosillo B. et al . Gastrointestinal involvement in mantle cell lymphoma: a prospective clinic, endoscopic, and pathologic study.  Am J Surg Pathol. 2006;  30 1274-1280
• 3 Martin P, Chadburn A, Christos P. et al . Intensive treatment strategies may not provide superior outcomes in mantle cell lymphoma: overall survival exceeding 7 years with standard therapies.  Ann Oncol. 2008;  19 1327-1330