Abstract

BackgroundMultiple lymphomatous polyposis is a rare type of gastrointestinal lymphoma that extensively infiltrates the intestine. Multiple lymphomatous polyposis originates from the mantle zone of the lymphoma follicle and is considered to be a mantle cell lymphoma, which is a relatively aggressive type of B-cell non-Hodgkin’s lymphoma. We report an unusual case of a patient with multiple lymphomatous polyposis with extensive colorectal involvement and acute intestinal obstruction, an atypical complication of this rare disease. On the basis of this case study, the pitfalls in gastrointestinal tract lymphomatous polyposis diagnosis and prognosis, as well as the treatment options, are discussed.Case presentationOur patient was a 76-year-old white woman with asthenia, cramps, and swelling in the lower left quadrant of the abdomen, as well as weight loss within the previous 5 months. A colonoscopy revealed polyps in the rectum, sigmoid colon, descending colon, and right and left colic flexures. A biopsy revealed lymphomatous infiltration of the intestinal wall. Because of the large size of the polypoid masses, which narrowed the colonic lumen in multiple locations, the patient developed acute intestinal obstruction and was referred for laparotomy. She underwent a total proctocolectomy with a permanent ileostomy and a left salpingo-oophorectomy. Microscopic examination showed the presence of a multicentric, low-grade, small lymphocytic lymphoma. Immunohistochemical analysis revealed positive immunostaining for CD79a, CD20, and CD45. These results were consistent with the diagnosis of mantle cell lymphoma. Two weeks after surgery and prior to discharge, but before the beginning of chemotherapy, the patient’s general condition worsened as she experienced a severe and progressive respiratory tract infection, advanced respiratory insufficiency, and septic shock, and she ultimately died.ConclusionsMantle cell lymphoma develops as a progressive and aggressive disease with widespread polyposis of the gastrointestinal tract. The intensive chemotherapeutic regimens usually result in the regression of macroscopic and microscopic lesions; however, remissions are short in duration, and the median length of patient survival is 3–4 years. Mantle cell lymphoma is a rare disease that should be part of the differential diagnosis of polypoid diseases of the large intestine.

Highlights

  • Multiple lymphomatous polyposis is a rare type of gastrointestinal lymphoma that extensively infiltrates the intestine

  • We describe and discuss the clinical and pathological features of a rare case of a patient with mantle cell lymphoma with diffuse involvement of the colon and rectum and complicated by an intestinal obstruction

  • CD10 is a marker of diffuse, large B-cell lymphoma, and CD23 is a marker for follicular lymphoma; both are usually negative in mantle cell lymphoma

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Summary

Conclusions

Mantle cell lymphoma develops as a progressive disease with a relatively aggressive clinical evolution and a high response rate to multiagent chemotherapy, but the outcomes may be poor in some cases, in older patients. In the GI tract, so-called multiple lymphomatous polyposis is the counterpart of peripheral mantle cell lymphoma. Mantle cell lymphoma must be considered in patients with widespread polyposis of the GI tract. CV revised the manuscript language and style. All authors read and approved the final manuscript. Consent for publication Written informed consent was obtained from the patient for publication of this case report and any accompanying images. Ethics approval and consent to participate The patient involved in this study (or her legal guardian) provided written consent prior to study enrollment. Author details 1Department of Surgery, Hospital do Servidor Público Estadual de São Paulo, Avenida Ibirapuera 981, 2o andar – Vila Clementino, Sao Paulo, SP 04029-000, Brazil. Author details 1Department of Surgery, Hospital do Servidor Público Estadual de São Paulo, Avenida Ibirapuera 981, 2o andar – Vila Clementino, Sao Paulo, SP 04029-000, Brazil. 2Department of Pathology, Hospital do Servidor Público Estadual de São Paulo, Sao Paulo, SP 04029-000, Brazil

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