Extensive colorectal lymphomatous polyposis complicated by acute intestinal obstruction: a case report

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.

Primary Mantle Cell Lymphoma of Rectum with Rare Presentation: A Unique Case Report and Review of Literature

“…An aggressive frontline immunochemotherapy induction regimen combining rituximab with cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) and a high dose of cytarabine is advised for patients younger than 65 years of age who are in good condition. Conventional immunotherapy (R-CHOP) followed by rituximab is the gold standard for the group of elderly patients or those in poor health [17,18]. Unfortunately, remissions are frequently brief, the likelihood of recurrence is high, and the median survival is only 3 to 4 years, despite the high response rate to aggressive chemotherapy regimens that typically result in the regression of macroscopic and occasionally microscopic lesions [15].…”

“…Unfortunately, remissions are frequently brief, the likelihood of recurrence is high, and the median survival is only 3 to 4 years, despite the high response rate to aggressive chemotherapy regimens that typically result in the regression of macroscopic and occasionally microscopic lesions [15]. Unsatisfactory clinical status, involvement of several extra-nodal locations, advanced age (greater than 70 years), increased lactate dehydrogenase levels, and bone marrow infiltration are all indicators of a poor prognosis [18].…”

Peritonitis due to colon mantle cell lymphoma

“…Colon lymphomas represent 0.4% of colon neoplasms [ 4 ]. The cell lymphoma mantle is a rare and aggressive subtype of B-cell non-Hodgkin lymphoma caused by overexpression of the cyclin D1 protein on chromosome 11 due to t translocation (11,14) (q13; q23) [ 1 ]. Classically affect lymph nodes, up to 80% of patients show extra-nodal involvement, depending on the molecular pathways involved in development [ 5 ].…”

“…The mantle cell lymphoma (MCL) a rare type of non-Hodgkin lymphoma, characterized by overexpression of protein cyclin D1 on chromosome 11 [ 1 ]. It presents as advanced disease with clinical variable, mainly affecting lymph nodes, up to 80% show extranodal involvement; of this, 20% affect gastrointestinal tract presenting a mass or numerous smalls polyps called multiple lymphomatous polyposis (MLP) [ 2 ].…”

Complicaciones quirúrgicas del linfoma con compromiso gastrointestinal

“…La obstrucción se puede presentar secundaria a una estenosis intra o extraluminal. Hasta el 54 % de los linfomas de ubicación ileocecal requieren intervención quirúrgica, comúnmente por intususcepción 24,28 . En adultos la obstrucción intestinal por intususcepción representa del 1 al 5 % de todas las causas mecánicas de obstrucción.…”