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An abundance of polyps: an intriguing case of lymphomatous polyposis.

INTRODUCTION

Mantle cell lymphoma (MCL) is an aggressive small B-cell neoplasm that makes up about 2.5 percent to 7 percent of non-Hodgkin lymphoma (NHL). The median survival of patients with MCL is three to five years, and has a worse prognosis when compared to some other subtypes of NHL. Gastrointestinal (GI) tract involvement is a common site of extranodular disease in patients with MCL. (1)

Multiple lymphomatous polyposis (MLP) is rare form of NHL in which MCL presents as numerous polyps through a long portion of the GI tract. In 1961, Cornes first used the term multiple lymphomatous polyposis to describe this rare presentation of MCL. (2)

MLP can involve any part of the GI tract, but the most frequent involvement is of the stomach (74.3 percent), followed by intestinal (62.9 percent) and esophageal (5.7 percent) lesions. (3) MLP rarely involves the entire GI tract. MLP lesions can be in different forms: superficial, protruded, fold thickening, and ulcerative.

Patients with MLP most often present with abdominal pain, hematochezia, diarrhea, obstruction, and/or intestinal malabsorption. Biopsy of the polyps or lesions via colonoscopy or endoscopy is performed to establish a diagnosis. We present the case of man with classic features of MLP, along with a brief discussion of relevant literature.

CASE

A 66-year-old African-American male with a history of type 2diabetes mellitus and hyperlipidemia presented to our colorectal surgery department with a 2-month history of constipation and altered bowel habits, including reduced caliber of stools. He had tried eating a high-fiber diet and taking polyethylene glycol, docusate, and sennosides. However, this resulted in only minimal relief of his symptoms.

[FIGURE 1 OMITTED]

His physical examination, including perianal and digital rectal examination, was unremarkable. He had no previous history of cancer and no family history of colorectal cancer. Laboratory examination showed a peripheral white blood cell count of 5,900 K/uL, hemoglobin 110 g/L, platelets 204,000 K/uL, lactate dehydrogenase 161 U/L, and albumin 2.8 g/dL.

Colonoscopy showed multiple 8-30 mm semisessile nonbleeding polyps throughout the colon (Figures 1 and 2). There was no evidence of obstruction or inflammation. Pathological findings of the biopsied specimens from colon mucosa showed diffuse infiltrate of intermediate size, atypical lymphoid cells with angulated nuclei and occasional small nucleoli consistent with NHL (Figure 3). Immunohistochemistry stains were positive for CD20, CD5, CD43, Bcl-2, and cyclin D1. CD10 and Bcl-6 were negative, and Ki-67 was 40 percent, consistent with MCL.

[FIGURE 2 OMITTED]

Staging computed tomography (CT) scans showed abundant enlarged lymph nodes in the upper abdomen and mesentery. There was a 5 cm index lymph node in the abdomen and a 3 cm node in the mesentery. The liver, spleen, and pancreas were unremarkable. Positron emission tomography-CT (PET-CT) showed hypermetabolic activity in the neck, abdomen, and pelvis. Bone marrow biopsy was negative for involvement of lymphoma. The patient's abundant polyps throughout the entire colon were consistent with a diagnosis of lymphomatous polyposis.

The patient has been started on clinical trial on at our facility. Currently, he is receiving treatment with bendamustine, rituximab, and bortezomib. He is tolerating the chemotherapy well and his bowel symptoms have improved. Restaging scans have shown a dramatic improvement in the bulk of his disease.

DISCUSSION

The majority of lymphomas are found in lymph nodes, but lymphomas can also be found in extranodal sites, including the GI tract (5 to 30 percent). Of all of the GI tumors, GI lymphomas represent roughly 1-4 percent. (4) Most lymphomas of the GI tract are solitary tumors; multiple tumors represent a small percentage of cases.

MLP is a rare extranodal non-Hodgkin B cell lymphoma of the GI tract, characterized by multiple neoplastic polyps. Since Cornes' report in 1961, fewer than 100 cases of MLP have been described in the literature.

It is important to distinguish MLP from mucosa-associated lymphoid tissue (MALT) lymphoma. MLP presents with diffuse involvement of a large portion of the GI tract, occurring anywhere from esophagus to rectum. The primary focus of disease can be at any site along the GI tract, with polyps rapidly proliferating. In addition, widespread lymph node involvement of mesenteric and paraaortic nodes are often seen at presentation. (5) In contrast, MALT lymphomas are primarily unifocal and remain localized at presentation.

MLP most commonly presents in middle-aged men ranging in age from 55-70 years. Presentation is usually nonspecific with symptoms of abdominal pain, diarrhea, constipation, hematochezia, lymphadenopathy, and weight loss. (6) A double-contrast barium enema and/or CT scans may show multiple masses along the GI tract.

On endoscopic examination, the polyps in MLP are often seen as multiple white nodular or polyploid tumors ranging from 0.2 to 2 cm. The larger polyps tend to be clustered and may be ulcerated. Biopsy is required for diagnosis. Histologically, the cells in MLP exhibit uniformity as a population of small cells with irregular nuclei and minimal cytoplasm. These monoclonal lymphocytes typically inhabit the mantle zone of follicles but eventually take over the entire follicle in the lamina propria of the GI tract. (5) In contrast, MALT lymphomas contain lymphocytes that invade glandular epithelium to form the characteristic lymphoepithelial lesions. (5) Markers associated with MLP are CD20, cyclin D1, CD79a, CD43, and Bcl-2. Negative findings for CD5, CD23, and IgM are also important in the diagnosis for MLP.1,5 Rearrangement of Bcl-1 on chromosome 11 with (11;14) translocation is usually seen on cytogenetic analysis resulting in cyclin D1 overexpression.

Several treatment options are available for GI MCL, and while initial response rates are usually greater than 50 percent, relapse rates remain high, especially in patients who cannot tolerate aggressive combination chemotherapy. The average survival of patients with MLP is usually less than 3 years. (7) The cyclophosphamide, doxorubicin, and prednisone (COP) regimen and the cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen can be used for treatment of MCL and GI MLP. The hyper-CVAD regimen, which consists of treatment with cyclophosphamide, doxorubicin, vincristine, dexamethasone, methotrexate, and cytarabine, can achieve complete remission in up to 68 percent of patients, and a response is seen in 90 percent of treated patients. The addition of rituximab can increase remission rates to 87 percent. But the hyper-CVAD regimen can only be tolerated by young and otherwise healthy patients secondary to its adverse effects. Most elderly patients do not tolerate such aggressive regimens or peripheral blood stem cell transplantation. Chlorambucil with or without corticosteroids may be considered in patients who may not tolerate combination chemotherapy. Radiotherapy has been used as an adjuvant to chemotherapy in the treatment of MLP to produce palliative symptomatic relief. Newer targeted therapy regimens with fewer adverse effects include drugs such as bortezomib and bendamustine. These are now being tested and used clinically, as in our case.

In conclusion, our case illustrates the importance of considering MLP or other forms of NHL in elderly patients found to have multiple GI polyps, especially those who have a history of clear colonoscopy within the previous 1 to 2 years.

[FIGURE 3 OMITTED]

Courtney Fox; Teja Poosarla, MD; Kamal Mandalapu, MD; H. David Vargas, MD; Marc R. Matrana, MD

REFERENCES

(1.) Tamura S, Ohkawauchi K, Yokoyama Y, et al. Non-multiple lymphomatous polyposis form of mantle cell lymphoma in the gastrointestinal tract. J Gastroenterol 2004;39:995-1000.

(2.) Cornes JS. Multiple lymphomatous polyposis of the gastrointestinal tract. Cancer 1961;14:249-257.

(3.) Iwamuro M, Okada H, Kawahara Y, et al. Endoscopic features and prognoses of mantle cell lymphoma with gastrointestinal involvement. World J Gastroenterol 2010;16:4661-4669.

(4.) Craig O, Gregson R. Primary lymphoma of the gastrointestinal tract. Clin Radiol 1981;32:63-72.

(5.) Sagar SM, Karp SJ, Falzon M. Malignant lymphomatous polyposis: diagnosis and response to chemotherapy. Clinical Oncol (R Coll Radiol) 1990;2:230-234.

(6.) Remes-Troche JM, De-Anda J, Ochoa V, et al. A rare case of multiple lymphomatous polyposis with widespread involvement of the gastrointestinal tract. Arch Pathol Lab Med 2003;127:1028-1030.

(7.) Michopoulos S, Petraki K, Matsouka C, Kastritis E, Chrysanthopoulou H, Dimopoulos MA. Mantle-cell lymphoma (multiple lymphomatous polyposis) of the entire GI tract. J Clin Oncol 2008;26:1555-1557.

Ms. Fox is a medical student associated with the University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA. Dr. Poosarla is affiliated with the Department of Internal Medicine, Dr. Mandalapu is affiliated with the Department of Hematology-Oncology and Dr. Vargas is associated with the Department of Colon and Rectal Surgery at the Ochsner Clinic Foundation, New Orleans, LA. Dr. Matrana is associated with both the University of Queensland School of Medicine, Ochsner Clinical School and the Department of Hematology-Oncology, Ochsner Clinic Foundation, New Orleans, LA.
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Author:Fox, Courtney; Poosarla, Teja; Mandalapu, Kamal; Vargas, H. David; Matrana, Marc R.
Publication:The Journal of the Louisiana State Medical Society
Article Type:Clinical report
Date:Jul 1, 2015
Words:1438
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