Primary mucosa-associated lymphoid tissue (MALT) lymphoma occurring in the rectum: a case report and review of the literature.Abstract: The primary extranodal B-cell lymphoma of mucosa-associated lymphoid tissue The mucosa-associated lymphoid tissue (MALT) (also called mucosa-associated lymphatic tissue) is the diffuse system of small concentrations of lymphoid tissue found in various sites of the body such as the gastrointestinal tract, thyroid, breast, lung, salivary glands, eye, and (MALT) is a distinct clinical pathologic entity that develops in diverse anatomic locations such as the stomach, salivary gland salivary gland Any of the organs that secrete saliva. Three pairs of major glands secrete saliva into the mouth through distinct ducts: the parotid glands (the largest), between the ear and the back of the lower jaw; the submaxillary glands, along the side of the lower jaw; , thyroid, lung, and breast; however, colorectal involvement is rare. To the best of our knowledge, only 30 cases of primary rectal MALT lymphoma MALT Lymphoma Definition MALT lymphomas are solid tumors that originate from cancerous growth of immune cells that are recruited to secretory tissue such as the gastrointestinal tract, salivary glands, lungs, and the thyroid gland. have been published in the English language literature, mostly from Japan. A single case has been reported from the US before this report. The most common symptoms ranged from asymptomatic to occult or gross gastrointestinal bleeding gastrointestinal bleeding Any hemorrhage into the GI tract lumen, from esophagus–eg, from ruptured esophageal varices, to anus–eg from hemorrhoids . Simultaneous involvement of the cecum cecum (sē`kəm): see intestine. or colon was seen in 20% of the patients. Ninety percent of the patients were classified as low grade, Stage 1 at the time of diagnosis. Polypoid polypoid /pol·yp·oid/ (pol´i-poid) resembling a polyp. pol·yp·oid adj. Resembling a polyp. polypoid resembling a polyp. lesions were 10-fold more common than ulcerative ulcerative /ul·cer·a·tive/ (ul´se-ra?tiv) (ul´ser-ah-tiv) pertaining to or characterized by ulceration. ulcerative pertaining to or characterized by ulceration. lesions. Seven patients were reported to have H pylori H pylori Helicobacter pylori, see there in the stomach. The majority of the patients underwent surgical or endoscopic en·do·scope n. An instrument for examining visually the interior of a bodily canal or a hollow organ such as the colon, bladder, or stomach. en resection as a cure; however, controversy exists with regards to antibiotic treatment or observation alone because of unknown etiopathogenesis. Infection with microorganisms other than H pylori has been postulated in the development of rectal MALT lymphoma; however, this hypothesis remains unproven. The overall prognosis of rectal MALT lymphoma appears favorable; however, long-term follow-up data is lacking. Therefore, periodic clinical monitoring should be done in these patients. Key Words: mucosa-associated lymphoid tissue (MALT), lymphoma, rectum ********** The primary extranodal B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is a distinct clinical pathologic entity. (1) MALT lymphoma is characterized histologically by the diffuse infiltration of small lymphoid lymphoid /lym·phoid/ (lim´foid) resembling or pertaining to lymph or tissue of the lymphoid system. lym·phoid adj. Of or relating to lymph or the lymphatic tissue where lymphocytes are formed. cells (centrocyte-like or monocytoid-looking cells), infiltration of lymphoma cells around the epithelium (lymphoepithelial lesions) and the proliferation of plasma cells Plasma cells A type of white blood cell. Mentioned in: Bence Jones Protein Test in the lamina propria of the mucosa. The clinical behavior of MALT lymphomas has been reported as favorable. (2) The MALT lymphoma develops in diverse anatomic locations such as the stomach, salivary gland, thyroid, lung, and breast (3); however, colorectal involvement is extremely rare. (4,5) To our knowledge, only 30 cases of primary rectal MALT lymphoma have been reported in the English language literature and a single case has been reported from the United States. In the present paper, we describe a patient with MALT lymphoma arising in the rectum and review the literature. Case Report A 69-year-old Hispanic man was referred for colonoscopy because of rectal polyps Rectal Polyps Definition Rectal polyps are tissue growths that arise from the wall of the rectum and protrude into it. They may be either benign or malignant (cancerous). that were seen on a screening sigmoidoscopy Sigmoidoscopy Definition Sigmoidoscopy is a procedure by which a doctor inserts either a short and rigid or slightly longer and flexible fiber-optic tube into the rectum to examine the lower portion of the large intestine (or bowel). . Three sessile polyps ranging in size from 5 to 6 mm were seen in the rectum and a 6 mm sessile polyp was present in the cecum (Fig. 1). All polyps Polyps A tumor with a small flap that attaches itself to the wall of various vascular organs such as the nose, uterus and rectum. Polyps bleed easily, and if they are suspected to be cancerous they should be surgically removed. were removed with cold snare. Histologic sections of the biopsies taken showed typical features of low grade MALT lymphoma: atypical lymphoid hyperplasia with reactive follicles follicles, n the masses that are embedded in a meshwork of reticular fibers within the lobules of the thyroid gland. See also thyroid gland. surrounded by hyperplastic mantle and/or marginal zones and scattered epithelioid histiocytes (Fig. 2). Scattered lymphoepithelial lesions were also present. By immunohistochemistry on both blocks, the majority of cells in the expanded mantle zones or marginal zones were positive for CD 20 and BCL-2, negative for CD5, cyclin D1, CD10, CD3 and CD43. Kappa and lambda showed scattered polyclonal polyclonal /poly·clo·nal/ (-klon´'l) 1. derived from different cells. 2. pertaining to several clones. polyclonal derived from different cells; pertaining to several clones. plasma cells. Polymerase chain reaction polymerase chain reaction (pŏl`ĭmərās') (PCR), laboratory process in which a particular DNA segment from a mixture of DNA chains is rapidly replicated, producing a large, readily analyzed sample of a piece of DNA; the process is analysis done on both rectal and cecal cecal /ce·cal/ (se´k'l) 1. ending in a blind passage. 2. pertaining to the cecum. ce·cal adj. Of, relating to, or having the characteristics of the cecum. biopsy specimens showed identical monoclonal heavy chain gene rearrangement in both specimens, confirming the diagnosis of MALT lymphoma on the rectal and cecal biopsy. No extraintestinal involvement was found on staging evaluation, which included computed tomography Computed tomography (CT scan) X rays are aimed at slices of the body (by rotating equipment) and results are assembled with a computer to give a three-dimensional picture of a structure. (CT) of the abdomen, chest, pelvis and a bone marrow biopsy Bone marrow biopsy A procedure in which cellular material is removed from the pelvis or breastbone and examined under a microscope to look for the presence of abnormal blood cells characteristic of specific forms of leukemia and lymphoma. . His upper endoscopy Upper endoscopy A medical procedure in which a thin, lighted, flexible tube (endoscope) is inserted down the patient's throat. Through this tube the doctor can view the lining of the esophagus, stomach, and the upper part of the small intestine. was negative for H pylori. He did not have a family history of similar tumors. Follow-up upper endoscopy and colonoscopy after 8 months showed a 5 mm sessile polyp in the gastric body, diminutive polyp polyp, in medicine, a benign tumor occurring in areas lined with mucous membrane such as the nose, gastrointestinal tract (especially the colon), and the uterus. Some polyps are pedunculated tumors, i.e. in the ascending colon ascending colon n. The part of the colon between the ileocecal orifice and the right colic flexure. and a 10 mm sessile polyp in the rectum near the dentate dentate /den·tate/ (den´tat) notched; tooth-shaped. den·tate adj. Edged with toothlike projections; toothed. . In addition, a submucosal submucosal /sub·mu·co·sal/ (-mu-ko´sal) 1. pertaining to the submucosa. 2. beneath a mucous membrane. nodule nodule: see concretion. nodule In geology, a rounded mineral concretion that is distinct from, and may be separated from, the formation in which it occurs. approximately 5 to 6 mm in size was also seen in the rectum near the dentate line (Fig. 3). Biopsies from gastric, colon and rectal polyps revealed reactive lymphoid aggregates. On rectal endoscopic ultrasound Endoscopic ultrasound (EUS) A medical procedure in which sound waves are sent to the stomach wall by an ultrasound probe attached to the end of an endoscope. (EUS Endoscopic ultrasonography (EUS) A medical procedure in which sound waves are sent to the stomach wall by an ultrasound probe attached to the end of an endoscope. ), the rectal submucosal nodule appeared hypoechoic and was limited to the mucosa and submucosa submucosa /sub·mu·co·sa/ (sub?mu-ko´sah) areolar tissue situated beneath a mucous membrane. sub·mu·co·sa n. A layer of loose connective tissue beneath a mucous membrane. . The muscularis propria pro·pri·a n. Plural of proprium. was intact and no local lymph node lymph node Small, rounded mass of lymphoid tissue contained in connective tissue. They occur all along lymphatic vessels, with clusters in certain areas (e.g., neck, groin, armpits). was noted. The patient was referred to the colorectal surgeon colorectal surgeon A surgeon specialized in managing colorectal disease–eg, hemorrhoids, cancer for excision of the rectal submucosal nodule because of close proximity to the dentate line; however, the patient was then lost to follow-up. Discussion Isaacson and Wright introduced the concept of lymphoma of the MALT type in 1983. (1) MALT-type lymphomas usually arise in organs originally devoid of lymphoid tissue lymphoid tissue Cells, tissues, and organs composing the immune system, including the bone marrow, thymus, spleen, and lymph nodes. The most highly organized components are the thymus and lymph nodes, and the least organized are the cells that wander in the loose , which is acquired by persistent antigenic stimulation triggered by infectious or autoimmune processes. (6) Most MALT-type lymphomas arise from gastrointestinal organs as well as from extragastrointestinal organs such as the salivary gland, thyroid gland, lung, bladder, and skin. (3) The stomach is the most common site of gastrointestinal involvement by MALT lymphoma and constitutes approximately 70% of all cases. (3) Primary colorectal involvement by MALT lymphoma is very rare. (4,5) [FIGURE 1 OMITTED] We searched the MEDLINE The online medical database of the U.S. National Library of Medicine (NLM) whose parent is the National Institutes of Health, Bethesda, MD. MEDLINE contains millions of articles from thousands of medical journals and publications. The consumer section of the site (http://medlineplus. for English language articles from 1966 to 2003 using the terms "MALT," "colon" and "rectum." Only 30 published cases of primary rectal MALT lymphoma were found. These are summarized in the Table. Most of the reported cases of primary rectal MALT lymphoma are from Japan (Table) and one is reported from the United States before this report. In these 30 patients and our present patient, the ages ranged from 33 to 83 years (mean 59.8 yr) and the male to female ratio was approximately 1:1. The most common presenting symptoms ranged from asymptomatic to occult or gross gastrointestinal bleeding. Simultaneous involvement of the cecum was seen in 6 patients. Ninety percent of patients (27 of 30) were low grade and in stage I at the time of diagnosis. Two patients each were in stage II and IV. Only 7 patients out of 30 were reported to be H pylori positive on biopsy from the gastric mucosa gastric mucosa, n the lining of the stomach. or by serology Serology The division of biological science concerned with antigen-antibody reactions in serum. It properly encompasses any of these reactions, but is often used in a limited sense to denote laboratory diagnostic tests, especially for syphilis. . Macroscopically, polypoid lesions were 10-fold more common than ulcerative lesions. Differences in gross appearance of gastric and rectal MALT lymphoma may be related to H pylori infection of the stomach. In gastric lymphoma gastric lymphoma Oncology A diffuse lymphoma composed of monotonous mature or atypical lymphocytes, or of large lymphocytes Etiology GL, especially MALT lymphoma, is linked to H pylori , H pylori-negative cases tend to form submucosal tumor-like lesions. (30) [FIGURE 2 OMITTED] MALT lymphoma is characterized by infiltrate of centrocyte-like cells, which may show plasma cell plasma cell n. An antibody-producing lymphocyte derived from a B cell upon reaction with a specific antigen. Also called plasmacyte. Plasma cell differentiation, lymphoepithelial lesions, and reactive lymphoid follicles. Colonization of the follicles may be seen, which can cause confusion with follicular lymphoma follicular lymphoma n. See nodular lymphoma. follicular lymphoma Follicle center lymphoma A heterogeneous group of NHLs arising in follicular center cells, which comprises 50% of all NHLs in adults–US, . Another differential diagnosis differential diagnosis n. Determination of which one of two or more diseases with similar symptoms is the one from which the patient is suffering. Also called differentiation. includes mantle cell lymphoma Mantle cell lymphoma (MCL) is one of the rarer of the non-Hodgkin's lymphomas, comprising about 6% of NHL cases.[1] There are only about 15,000 patients presently in the U.S. (The incidence seems to be somewhat higher in Europe. that presents as lymphomatous polyposis polyposis /pol·yp·osis/ (pol?i-po´sis) the formation of numerous polyps. familial polyposis , familial adenomatous polyposis in the gastrointestinal tract and is composed of small to medium-sized lymphocytes with irregular nuclei arranged in a nodular nodular marked with, or resembling, nodules. nodular dermatofibrosis see dermatofibrosis. nodular episcleritis see nodular fasciitis (below). nodular fasciitis a firm painless nodular swelling, 0. or mixed nodular pattern. There is no epithelial invasion and the cells are positive for CD5 and cyclin D1, which are negative in MALT lymphoma. Also, low-grade MALT lymphoma is distinguished from reactive conditions by showing light chain restriction. Reported forms of treatment for rectal MALT lymphoma include surgical resection in 9 patients (30%), surgical resection followed by chemotherapy in 3 patients (10%), radiation and chemotherapy in 7 patients (23%), endoscopic resection in 5 patients (17%), endoscopic resection followed by antibiotic treatment in 3 patients (10%), antibiotic treatment alone in 6 patients (20%), and observation alone in 2 patients (6%). Thus, the majority of patients have undergone surgical or endoscopic resection as a cure. However, controversy exists with regards to antibiotic treatment or observation because of unknown etiopathogenesis of primary rectal MALT lymphoma. In contrast, gastric MALT lymphoma is often associated with infection by H pylori. Eradication of H pylori has been investigated as the first line of treatment for patients with MALT-type lymphoma of the stomach, and has been found to be highly effective in patients with localized low-grade disease. (31) Patients with more disseminated disease, t(11:18) or t(l:19) chromosomal translocations or those with high-grade malignancy are generally not thought to be responsive to antibiotic treatment alone. MALT lymphoma of other sites have been associated with infections. For example, cutaneous cutaneous /cu·ta·ne·ous/ (ku-ta´ne-us) pertaining to the skin. cu·ta·ne·ous adj. Of, relating to, or affecting the skin. Cutaneous Pertaining to the skin. MALT lymphoma has been associated with Borrelia burgdorferi infections (32) and orbital adnexal adnexal /ad·nex·al/ (ad-nek´sal) pertaining to adnexa. adnexal pertaining to, or emanating from, the adnexa. adnexal tumors MALT lymphoma with Chlamydia psittaci infections. (33) In both types, there have been reports of lymphoma regression with successful treatment of the infection. [FIGURE 3 OMITTED] Regression of low-grade MALT lymphoma after H pylori eradication therapy has been reported in extragastric sites of the gastrointestinal tract. (8,34-36) Nakase et al (20) reported three H pylori-negative cases of rectal MALT lymphoma which regressed after antibiotic treatment. Regression of rectal MALT lymphoma after administration of quinolones has also been reported. (26,27) These reports suggest involvement of microorganisms other than H pylori in the development of rectal MALT lymphoma; however, this hypothesis remains unproven. Spontaneous regression of rectal MALT lymphoma has also been reported in 2 patients (15,16); however, the follow-up in each patient was very limited. Neither our patient nor any of the reported cases had a family history of similar tumors, suggesting an unlikely genetic predisposition. We treated our patient with local excision alone, without H pylori eradication treatment, given the negative biopsy. Our patient's follow up after 9 months showed no recurrence of MALT lymphoma; however, the exact etiology of his rectal submucosal nodule was unclear in the absence of excision biopsy or further endoscopic follow-up and therefore, recurrence of MALT lymphoma could not be ruled out. The follow-up of reported cases ranged from 0.2 to 10 years (mean 2 yr). The prognosis appears favorable and none of the patients died due to rectal MALT lymphoma. However, close follow-up examination is important since the long-term follow-up data is lacking in these patients. References 1. Isaacson PG, Wright DH. Malignant lymphoma of mucosa-associated lymphoid tissue. A distinctive type of B-cell lymphoma. Cancer 1983;52:1410-1416. 2. Isaacson PG. The malignant lymphoma concept updated. Ann Oncol 1995;6:319-320. 3. Zucca E, Roggera E, Bertoni F, et al. Primary eztra-nodal non-Hodgkin's lymphomas. Part 1: gastrointestinal, cutaneous and genitourinary genitourinary /gen·i·to·uri·nary/ (jen?i-to-u´ri-nar-e) pertaining to the genital and urinary organs. gen·i·to·u·ri·nar·y adj. Abbr. lymphomas. Ann Oncol 1997;8:727-737. 4. Schmid C, Vazquez JJ, Diss TC, et al. Primary B-cell mucosa-associated lymphoid tissue lymphoma mucosa-associated lymphoid tissue lymphoma See MALT lyphoma. presenting as a solitary colorectal polyp. Histopathology his·to·pa·thol·o·gy n. The science concerned with the cytologic and histologic structure of abnormal or diseased tissue. Histopathology The study of diseased tissues at a minute (microscopic) level. 1994;24:357-362. 5. Onishi Y, Fujisawa T, Maeda M, et al. Large mucosa-associated lymphoid tissue lymphoma simulating multiple polypoid lesions at the cecum and rectum. Digestive endoscopy endoscopy Examination of the body's interior through an instrument inserted into a natural opening or an incision, usually as an outpatient procedure. Endoscopes include the upper gastrointestinal endoscope (for the esophagus, stomach, and duodenum), the colonoscope (for the 2001;13:225-229. 6. Greiner A, Marx A, Heesemann J, et al. Idiotype id·i·o·type n. A determinant that confers on an immunoglobulin molecule an antigenic individuality that is analogous to the individuality of the molecule's antibody activity. identity in a MALT-lymphoma and B-cells in Helicobacter pylori associated chronic gastritis. Lab Invest 1994;70. 7. Bschorer R, Lingenfeiser T E K, et al. Malignant lymphoma of the mucosa-associated lymphoid tissue (MALT)--consecutive unusual manifestation in the rectum and gingiva gingiva /gin·gi·va/ (jin´ji-vah) (jin-ji´vah) pl. gin´givae [L.] the gum; the mucous membrane, with supporting fibrous tissue, covering the tooth-bearing border of the jaw. . J Oral Pathol Med 1993;22:190-192. 8. Matsumoto T, Lida M M S. Regression mucosa-associated lymphoid-tissue lymphoma of rectum after eradication of Helicobacter pylori. Lancet 1997;350:115-116. 9. Yatabe Y. Nakamura S, Nakamura T, et al. Multiple polypoid lesions of primary mucosa-associated lymphoid-tissue lymphoma of colon. Histapathology 1998;32:116-125. 10. Gianni L, Tassinari D, Sartori S, et al. Gastric, duodenal duodenal /du·o·de·nal/ (doo?o-de´n'l) (doo-od´ah-n'l) of or pertaining to the duodenum. Duodenal Refers to the duodenum, or the first part of the small intestine. and rectal multifocal multifocal /mul·ti·fo·cal/ (mul?te-fo´k'l) arising from or pertaining to many foci. mul·ti·fo·cal adj. Relating to or arising from many foci. MALT lymphoma: the possible co-existence of two different eel] populations. Eur J Cancer 1998;34:1640-1641. 11. Matsumoto T, Shirnizu M, lida M, et al. Primary low-grade, B-cell, mucosa-associated lymphoid tissue lymphoma of the colorectum: clinical and colonoscopie features in six cases. Gastrointest Endosc 1998;48:501-508. 12. Orita M, Yamashita K, Okino M, et al. A case of MALT (Mucosa-Associated Lymphoid Tissue) lymphoma occurring in the rectum. Hepato-Gastroenterology 1999;46:2352-2354. 13. Inoue F, T C. Regression of MALT lymphoma of the rectum after anti-H. Pylori therapy in a patient negative for H. pylori. Gastroenterology 1999;117:514-515. 14. Hosaka S, Akamatsu T, Nakamura S, et al. Mucosa-associated lymphoid tissue (MALT) lymphoma of the rectum with chromosomal translocation of the t(11;18)(q21;q21) and an additional aberration of trisomy trisomy /tri·so·my/ (tri´so-me) the presence of an additional (third) chromosome of one type in an otherwise diploid cell (2n + 1). See also entries under syndrome. triso´mic tri·so·my n. 3. Am J Gastroenterol 1999;94:1951-1954. 15. Takenaka R, Tomoda J, Sakata T, et al. Mucosa associated lymphoid tissue lymphoma of the rectum that regressed spontaneously. J of Gastroenterology & Hepatology 2000;15:331-335. 16. Okamura S, Katsuhiko H T S. Regression of rectal mucosa-associated lymphoid tissue lymphoma unrelated to Helicobacter pylori. Annals of Internal Medicine Annals of Internal Medicine (Ann Intern Med) is an academic medical journal published by the American College of Physicians (ACP). It publishes research articles and reviews in the area of internal medicine. Its current editor is Harold C. Sox. 2000;132:247. 17. Gavioli M, Bagni A, Santacroce G, et al. Endorectal sono graphic appearances of rectal MALT lymphoma, its response to therapy, and local recurrence. J Clin Ultrasound 2001;29:401-405. 18. Tsang RW, Gospodarowicz MK, Pintilie M, et al. Stage I and II MALT lymphoma: results of treatment with radiotherapy, Int J Radial Oncol Eiol Phys 2001;50:1258-1264. 19. Tanimoto A, Hamada T, Yamamoto T, et al. MALT lymphoma with extreme plasma cell differentiation of the rectum. Am J Gastroenterol 2002;97:1860-1862. 20. Nakase H, Ohana M, Ikeda K, et al. The possible involvement of microorganisms other than Helicobacter pylori in the development of rectal MALT lymphoma in H. pylori-negative patients. Endoscopy 2002;34: 343-346. 21. Hisabe T, Imamura K, Furukawa K, et al. Regression of CD5-positive and Helicobacter pylori--negative mucosa associated lymphoid tissue lymphoma of the rectum after administration of antibiotics. Dis Colon Rectum 2002;45: (7) 1267-1270. 22. Rey J, Coso D, Ramuz O, et al. Low-grade rectal malt lymphoma occurring in a patient with chronic lymphocytic leukaemia, Br J Haematol 2002;119:1137-1138. 23. Tanaka S, Ohta T, Kaji E, et al. EMR (ElectroMagnetic Radiation) The emanation of energy from everything in the universe. Although the EMR from electrical and electronic devices is typically measured for practical, every-day situations, every object, including humans, emanates energy. of mucosa-associated lymphoid tissue lymphoma of the rectum. Gastrointest Endosc 2003;57:956-959. 24. Navarra G, Adani GL, Bardella E, et al. Primary rectal lymphoma as a cause of bowel obstruction. Report of a case. Tumori 2003;89:88-90. 25. Chim CS, Shek TW, Chung LP, et al. Unusual abdominal tumors: case 3. Multiple lymphomatous polyposis in lymphoma of colon. J Clin Oncol 2003;21:953-955. 26. Dohden K, Kaizaki Y, Hosokawa O, et al. Regression of rectal mucosa-associated iymphoid tissue Jymphoma but persistence of Helicobacter pylori infection of gastric mucosa after administration of levofloxacin: report of a case. Dis Colon Rectum 2004;47:1544-1546. 27. Hori K, Suguro M, Koizuka H, et al. Disappearance of rectal mucosa-associated lymphoid tissue lymphoma following antibiotic therapy. Dig Dis Sci 2004;49:413-416. 28. Lee YG, Lee S, Han SW, et al. A case of multiple mucosa-associated lymphoid tissue (MALT) lymphoma of the colon identified as simple mucosal discoloration dis·col·or·a·tion n. 1. a. The act of discoloring. b. The condition of being discolored. 2. A discolored spot, smudge, or area; a stain. Noun 1. . J Korean Med Sci 2005;20:325-328. 29. Ahlawat S, Haddad N, Kanber Y. et al. Primary mucosa-associated lymphoid tissue lymphoma occurring in the rectum. Gastrointest En dosc 2005;62:443-444; discussion 444. 30. Yokoi T, Nakamura T, Nagashima S, et al. [Differential diagnosis of gastric MALT lymphomas]. Ito Cho [Stomach Inttestine] 2000;36:13-20 (in Japanese with an English abstract). 31. Thiede C, Morgner A, Alpen B, et al. What role does Helicobacter pylori eradication play in gastric MALT and gastric MALT lymphoma. Gastroenterology 1997;113 (Suppl 6):S61-S64. 32. Roggero E, Zucca E, Mainetti C, et al. Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin. Hum Pathol 2000;31:263-268. 33. Ferreri AJ, Guidoboni M M P. Evidence of an association between chlamydia psittaci and ocular adnexal lymphoma. J Natl Cancer Inst. 2004;96:571-573. 34. Nagashima R, Takeda H. Maeda K, et al. Regression of duodenal mucosa-associated lymphoid-tissue lymphoma after eradication of Helicobacter pylori. Gastroenterology 1996;111:1674-1678. 35. Fischbach W, Tacke W, Greiner A, et al. Regression of immunoproliferative small intestinal disease after eradication of Helicobacter pylori. Lancet 1997;349:31-32. 36. Raderer M, Pfeffel F, Pohl G, et al. Regression of colonic low grade B cell lymphoma of the mucosa-associated lymphoid tissue type after eradication of Helicobacter pylori. Gut 2000;46:133-135. Sushil Ahlawat, MD, Yonca Kanber, MD, Aline Charabaty-Pishvaian, MD, Metin Ozdemirli, MD, PhD, Philip Cohen, MD, Stanley Benjamin, MD, and Nadim Haddad, MD From the Division of Gastroenterology, Department of Pathology, and Division of Hematology and Oncology. Georgetown University Hospital Georgetown University Hospital was founded in 1898 as part of Georgetown University, a Catholic, Jesuit University in the Georgetown neighborhood of , Washington DC. Reprint requests to Nadim Haddad, M.D., Division of Gastroenterology, Department of Medicine, 3800 Reservoir Road, NW, Washington DC 20007. Email: ngh3@georgetown.edu Accepted January 17, 2006. RELATED ARTICLE: Key Points * Mucosa-associated lymphoid tissue (MALT) lymphoma accounts for approximately one-third of colorectal lymphomas. * It is unclear if colorectal MALT lymphoma is related to Helicobacter pylori infection, and there are reports of colorectal MALT lymphoma regressing after treatment for H. pylori even if such infection is absent, suggesting that microorganisms other than H. pylori may play a role in the pathogenesis of nongastric MALT lymphomas. * Local therapy, as in the present case, is reasonable because colorectal MALT lymphoma is slow to spread.
Table. Summary of primary rectal mucosa-associated lymphoid tissue
lymphoma cases reported in the English language literature
First author
(reference) Year Country Age Sex Symptoms
Bschorer (7) 1993 Germany 56 M Weight loss, anorexia,
painful defecation
Matsumoto (8) 1997 Japan 72 F Rectal bleeding
Yatabe (9) 1997 Japan 75 M None
38 F Ileus
Gianni (10) 1998 Italy 56 M NA
Matsumoto (11) 1998 Japan 71 F Hematochezia
72 F None
62 F Hematochezia
72 M Diarrhea
60 M Hematochezia
Orita (12) 1999 Japan 64 M Stool occult blood positive
Inoue (13) 1999 Japan 62 F Hematochezia
Hosaka (14) 1999 Japan 56 M Stool occult blood positive
Takenaka (15) 2000 Japan 76 M Stool occult blood positive
Okamura (16) 2000 Japan 54 M NA
Onishi (5) 2001 Japan 76 M Hematochezia
Gavioli (17) 2001 Italy 45 M Rectal bleeding
Tsang (18)+ 2001 Canada NA NA NA
Tanimoto (19) 2002 Japan 75 F Rectal bleeding
Nakase (20) 2002 Japan 66 M Hematochezia
33 F Hematochezia & fever
Hisabe (21) 2002 Japan 70 F NA
Rey (22) 2002 France 42 F Abdominal pain, rectal
bleeding
Tanaka (23) 2003 Japan 62 M Screening
Navarra (24) 2003 Italy 54 F Abdominal pain
Chim (25) 2003 Hong Kong 43 F Rectal bleeding
Dohden (26) 2004 Japan 60 F Hematochezia
Hori (27) 2004 Japan 83 F Anal swelling
Lee (28) 2005 Korea 47 M Tenesmus, mucoid stool
Ahlawat (29) 2005 USA 57 F None
Present case 2005 USA 69 M Screening
Ann
First author Arbor Helicobacter
(reference) Endoscopic findings stage pylori status
Bschorer (7) Ulcerated polypoid lesion in the IBE NA
rectum (5 X 7.5 cm)
Matsumoto (8) Broad-based protrusion in the IAE Positive
rectum
Yatabe (9) 3 nodular lesions in the rectum I NA
7 nodular lesions in the colon and I NA
rectum
Gianni (10) Multifocal involving the stomach, NA Positive
duodenum and rectum
Matsumoto (11) Whitish, solitary protrusion in IE Positive
the rectum
Reddish solitary protrusion in the IE Positive
rectum
Reddish solitary protrusion in the IE NA
rectum
Multiple whitish protrusion and IE NA
erosion in the sigmoid colon and
rectum
Multiple whitish to reddish IE Positive
protrusions and erosions in the
ascending, transverse, sigmoid
colon and rectum
Orita (12) Oval-shaped tumor in the rectum IAE NA
(3.5 X 4.8 cm)
Inoue (13) Broad-based protrusion in the Negative
rectum
Hosaka (14) Multiple, red, nodular lesion in IE
the rectum
Takenaka (15) Slightly yellowish protruding IE Negative
lesion with grooved depression
in the lower rectum (2 cm) and
2 flat, slightly yellowish
elevations in the upper rectum
Okamura (16) Small, nodular elevations in the IE Negative
rectum
Onishi (5) Elevated lesion (8 X 7 cm) in the IIE Negative
rectum & cecum (4 X 3.5)
Gavioli (17) Circumferential polypoid IV NA
thickening with an irregular
friable surface
Tsang (18)+ NA IV NA
Tanimoto (19) Submucosal tumor with a central NA NA
area of ulcerated mucosa
(2 X 1 cm)
Nakase (20) Reddish, flat, elevated lesion in IE Negative
the descending & sigmoid colon
with a protruding lesion in the
rectum
Small, whitish lesion at the NA Negative
rectum
Hisabe (21) Hemispheric protrusion 1.5 cm in IE Negative
size
Rey (22) Tumoral and infiltrated aspect of IE Negative
the rectal mucosa with
hemorrhagic suffusions
Tanaka (23) Flat, yellowish, elevated lesion IAE Negative
(2 cm)
Navarra (24) Bulky, smooth, ulcerated, 5 cm IIE NA
mass occupying 70% of rectal
circumference
Chim (25) Multiple polypoid lesions in the IE NA
rectum and descending colon
Dohden (26) 3.5 cm submucosal tumor near the IE Positive
dentate line
Hori (27) Diffuse, protruding lesion 5 cm in IE Positive
length in the lower rectum
Lee (28) Granular and reddish mucosal IE Negative
thickening just above rectum,
reddish inflammatory mucosa at
appendiceal orifice
Ahlawat (29) Sessile, 1.5 X 2 cm nodular rectal IE Negative
mass
Present case Three sessile polyps (5-6 mm) in IAE Negative
the rectum and a 5 mm sessile
polyp in the cecum
First author Follow-up
(reference) Treatment Outcome in years
Bschorer (7) Chemo + RT Complete 4
remission
Matsumoto (8) HPE Cured (a) 1
Yatabe (9) Surgery NED 2
Surgery NED 2
Gianni (10) Chemo + HPE Relapse after NA
7 months
Matsumoto (11) Local resection NED 2.6
Partial endoscopic NED 0.6
resection + HPE
Local resection NED 10
Partial endoscopic NA 3.5
resection,
proctosigmoidectomy
Partial endoscopic NED 0.75
resection
Orita (12) APR Cured (a) 2
Inoue (13) HPE Cured (a) 1
Hosaka (14) Surgical resection + Cured (a)
adjuvant chemo
Takenaka (15) Observation Cured (a) 1.6
Okamura (16) Observation Spontaneous NA
regression.
biopsy
proven
Onishi (5) Ileocecectomy, APR + Cured (a) 1
adjuvant chemo
Gavioli (17) Chemo NED 2
Tsang (18)+ RT NED 4.2 (median)
Tanimoto (19) APR Cured (a) 0.9
Nakase (20) HPE Cured (a) 1.5
HPE Cured (a) 0.8
Hisabe (21) HPE Cured (a) 1.6
Rey (22) Chemo Cured NA
Tanaka (23) EMR Cured (a) 3
Navarra (24) Chemo, Low APR Cured 3
Chim (25) Chemo NED 1.5
Dohden (26) Antibiotics Cured 0.4
Hori (27) Antibiotics, HPE Cured 0.5
Lee (28) Chemo + RT NED 0.25
Ahlawat (29) EMR + HPE NED 1
Present case Snare polypectomy Likely cure 0.8
(a) Documented by endoscopy and histology.
EMR, endoscopic mucosal resection; HPE, Helicobacter pylori eradication;
APR, abdominoperineal resection; F, female; M, male; NED, no evidence of
disease; RT, radiotherapy.
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