Primary Gastrointestinal Mantle Cell Lymphoma: A Retrospective Study

Dasappa, Lokanatha; Babu, M C Suresh; Sirsath, Nagesh T; Suresh, T. M.; Babu, K Govind; Sathyanarayna, Vishwanatha; Lokesh, K N; Lakshmaiah, K C

doi:10.1007/s12029-014-9655-2

Cited by 13 publications

(30 citation statements)

References 31 publications

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“…The colon and rectum are usually the most affected segments (approximately 90% of mantle cell lymphoma cases, including this one), followed by the small intestine, stomach, and duodenum [2, 12]. The ileocecal region is frequently the original focus of the mantle cell lymphoma, and it generally remains the primary site of the disease [2, 9]. The macroscopic appearance of mantle cell lymphoma in the GI tract is variable, and it can look like tumor masses, ulcers, mucosal thicknesses, or multiple polypoid lesions, with the latter being characteristic of MLP [2, 9].…”

Section: Discussionmentioning

confidence: 99%

“…Cornes [13] coined the disease’s name, multiple lymphomatous polyposis , in 1961 to designate numerous polypoid lesions in the GI tract comprising lymphoma with mucosal involvement. Since this first description, only a few cases have been reported [2, 9, 10]. 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.…”

Section: Introductionmentioning

confidence: 99%

“…Mantle cell lymphoma comprises 2.5–7.0% of all NHLs, and the GI tract is involved in approximately 20% of cases [8, 9]. Mantle cell lymphoma is often diagnosed at an advanced stage.…”

Section: Introductionmentioning

confidence: 99%

“…Multiple lymphomatous polyposis (MLP) is characterized by the presence of multiple lymphomatous polyps along one or more segments of the digestive tract [2, 9, 10]. MLP is characterized by multiple white, superficially infiltrated nodular or polypoid lesions involving long segments of the GI tract [12].…”

Section: Introductionmentioning

confidence: 99%

“…MLP is characterized by multiple white, superficially infiltrated nodular or polypoid lesions involving long segments of the GI tract [12]. In most MLP cases, lymphoma cells originate from the mantle zone of the lymphoid follicle, so this disease is actually considered a subtype of mantle cell lymphoma [8, 9], which accounts for 2–9% of primary GI lymphomas [2, 9, 10]. …”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Mantle cell lymphoma comprises 2.5–7.0% of all NHLs, and the GI tract is involved in approximately 20% of cases [8, 9]. Mantle cell lymphoma is often diagnosed at an advanced stage.…”

Section: Introductionmentioning

confidence: 99%