Mucosa-associated lymphoid tissue lymphoma in the terminal ileum:A case report

lNTRODUCTlON

Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade B-cell neoplasm of the extranodal marginal zone characterized by a lymphoid infiltrate in the mucous layer of hollow organs and glandular tissues[1,2].The gastrointestinal tract is involved in about 50% of the cases[2,3] with the stomach accounting for 85% of all cases and strongly related to the presence and infection by

(

)[1,4].Other,less usual regions can also be affected,such as salivary glands,lungs (14%),head and neck (15%),ocular attachments (12%) and skin (11%)[5].

MALT lymphomas in the ileum are extremely rare and few cases have been reported in the literature[5-9].In these,all patients had already presented with an advanced degree of involvement with notable symptoms and with lesions dispersed throughout the ileocecal region[5,6].

This is the first reported case of a terminal ileum MALT lymphoma in an asymptomatic patient reported in the literature.

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CASE PRESENTATlON

Chief complaints

Asymptomatic.

History of present illness

A 57-year-old asymptomatic female patient underwent a colonoscopy exam for screening.The examination revealed an ulcer of medium depth with well-defined borders covered by a thin layer of fibrin and a halo of hyperemia in the distal ileum portion (Figure 1).Findings are nonspecific but may signal infections by viruses,protozoa and parasites or inflammatory diseases such as Crohn's disease.Biopsies of the ulcer were taken.

The anatomopathological result revealed an atypical diffuse lymphocytic infiltrate of small cells with a characteristic cytoplasmic halo of marginal zone cells.The infiltrate presented with nodular and poorly delimited areas with dissection of collagen fibers and the muscular layer of the mucosa.There was no clear distinction regarding germinal centers.Signs of cellular atypia were also observed with enlarged nuclei.In the most superficial portion there was focal erosion,epithelial reactivity and eosinophilia (above 15

high-power field) (Figure 2).No granulomas were found and there were no signs of infection by parasitic agents.An immunohistochemical study was requested to investigate lymphoproliferative disease.

In all cases,biopsy with histological evaluation concurrently with immunohistochemical analysis was crucial for the diagnosis of MALT lymphoma.

The Captain returned to his lonely cave, which seemed frightful32 to him without his lost companions, and firmly resolved to avenge33 them by killing34 Ali Baba

The immunohistochemical study was performed by the EnVision FLEX Visualization System kit AGILENT (DAKO) method,which the results demonstrated a negative neoplastic infiltrate for the expression of cyclin D1 (Figure 2B) and cytokeratin AE1/AE3 (Figure 2C) and positive for BCL60 in the germinal center (Figure 2D).The test also revealed CD10 positivity in the glandular epithelium and germinal center of a reactive follicle (Figure 3A and B) with dual labeling of CD20 and CD3 demonstrating the B lymphocyte nature of the neoplastic infiltrate (Figure 3C and D).In BCL2 protein labeling,the neoplastic infiltrate is strongly positive with a negative germinal center (Figure 3E and F).

FlNAL DlAGNOSlS

The findings are consistent with immunophenotype B non-Hodgkin's lymphoma,better classified as extranodal MALT.The identification of lymphoid proliferation with atypical limits in a nodular and infiltrative pattern with foci of epithelial aggression was crucial for the diagnosis.Since MALT lymphomas are always negative for BCL6 and CD10 and positive for BCL2 with a negative germinal center,it was possible to rule out the differential diagnosis of follicular lymphoma.

TREATMENT

The patient was referred to the oncology team and treated with chemotherapy.

OUTCOME AND FOLLOW-UP

All other authors declare that they have no conflict of interest.

DlSCUSSlON

Extranodal marginal zone lymphoma (MALT lymphoma) is characterized by the proliferation of small B lymphocytes[10].The stomach is the most common site of involvement where the main etiology is

infection[1].In these cases,the endoscopic findings are varied and involve polyps,ulcerations,erythematous lesions,nodules and other non-specific findings[11].Extranodal marginal zone lymphomas that affect the ileum region are extremely rare and only a few cases have been reported in the literature[5-9].None of the previous studies showed

infections so the etiology of the disease remains unknown.

Endoscopic findings of primary small bowel lymphoma can be classified into 5 patterns:Mucosal fold thickening;nodular pattern,defined by the presence of nodules and micronodules of variable sizes;infiltrative pattern,where the bowel wall is immobile,not distended by insufflation,and firm over forceps;ulcerative pattern with ulcers of variable sizes and depths,and mosaic pattern[12].

The uniqueness of the case presented in this study is due to the fact that the patient was asymptomatic and her endoscopic findings had a more discrete and nonspecific pattern compared to other studies which made the diagnosis even more challenging.

The treatment of MALT lymphoma is initially made with the eradication of

,in cases with involvement of the bacteria.If there is no concomitant

infection or no tumor remission after

treatment,radiotherapy,chemotherapy,or immunotherapy with anti-CD20 monoclonal antibodies should be considered.Radiotherapy has an excellent prognosis when used in cases where the disease is localized.In the presence of disseminated or more advanced disease,the use of radiotherapy or immunotherapy is indicated.Treatment must be individualized according to the stage of the disease and symptoms,as well as the patient's preference[13].Although MALT lymphoma has a favorable prognosis and is responsive to systematic therapy,especially when identified early,when patients are symptomatic,unfortunately they already have a more advanced degree of involvement.

Terada[5] reported the case of a 34-year-old patient with abdominal pain and melena whose colonoscopy revealed multiple nodules and ulcers scattered throughout the ileum.Endoscopic images were suggestive of ileitis,mesenchymal tumor,or lymphoma.

Hasegawa

[6] described two cases of oligosymptomatic patients with abdominal pain being a common symptom.Colonoscopy in the first case found multiple whitish nodules in the region close to the ileocecal valve,which had a smooth and polished appearance.In the second case,a colonoscopy revealed an ileocecal valve with an enlarged,soft appearance and areas of enanthema.

Makino

[7] discussed a case of a patient with initial complaints of postprandial epigastric pain.Colonoscopy examination revealed multiple protruding lesions in the terminal ileum with an erosive surface covered by swollen mucosa.

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In the report by Ohashi

[8] colonoscopy identified multiple polyposis lesions in the terminal ileum with an absence of villi.

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Given the low sensitivity and specificity of endoscopic images in these cases the pathology can be confused with other important differential diagnoses such as inflammatory diseases (such as Crohn's disease) or infectious diseases,which makes the biopsy,even in asymptomatic patients,with anatomopathological analysis and performing immunohistochemistry,the gold standard for correct diagnosis[14].

Among the four cases published in the literature on ileum MALT lymphomas,all presented endoscopic findings with multiple protuberances:Two[5,7] cases with ulcerations and two[6,8] cases with smooth mucosa.In one case,the presence of a single mass in the intestine was demonstrated without erosions in the mucosa[6].

CONCLUSlON

The blessing11 to Karen and I was twofold. In addition to ending years of confusion and estrangement12（，） , we learned to share the joy of being the proud daughters of two extraordinary fathers.

When he beholds2 that picture he will fall violently in love with it and go off into a dead faint, and for her sake he will encounter many dangers; you must guard him from this

de Figueiredo VLP,Ribeiro IB,and de Moura EGH performed the data curation;Ribeiro IB and de Moura DTH contributed to the formal analysis;Ribeiro IB and de Moura EGH performed the investigation;de Moura EGH contributed to the supervision;de Figueiredo VLP and Ribeiro IB contributed to the writing of the original draft;Ribeiro IB contributed to the writing of the review and editing.

The work described has been conducted in accordance with the Code of Ethics of the World Medical Association (Declaration of Helsinki).Informed consent for publication of this case was obtained from the patient’s daughter (witnessed by two physicians).

However, whites did not do that with African Americans in the South in those days, and I conformed to the local practice -- though it conflicted with my Christian beliefs

Upon completion of treatment,the patient showed complete regression of the disease as evidenced by colonoscopy performed after treatment (Figure 4).

The authors have read the CARE Checklist (2016),and the manuscript was prepared and revised according to the CARE Checklist (2016).

This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers.It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BYNC 4.0) license,which permits others to distribute,remix,adapt,build upon this work non-commercially,and license their derivative works on different terms,provided the original work is properly cited and the use is noncommercial.See:http://creativecommons.org/Licenses/by-nc/4.0/

Brazil

Vitor Lauar Pimenta de Figueiredo 0000-0001-7304-8222;Igor Braga Ribeiro 0000-0003-1844-8973;Diogo Turiani Hourneaux de Moura 0000-0002-7446-0355;Cristiano Claudino Oliveira 0000-0001-6682-5230;Eduardo Guimar?es Hourneaux de Moura 0000-0003-1215-5731.

Fan JR

Filipodia

The moment the prince was left alone he drew the ring from under his pillow and kissed it a thousand times. Then he set his mind to find how he was to see the owner — for even he did not dare to confess that he had only beheld41 ‘Donkey Skin’ through a keyhole, lest they should laugh at this sudden passion. All this worry brought back the fever, which the arrival of the cake had diminished for the time; and the doctors, not knowing what else to say, informed the queen that her son was simply dying of love. The queen, stricken with horror, rushed into the king’s presence with the news, and together they hastened to their son’s bedside.

Melchior appears in the New Testament as one of the three sages from the east who came bearing gifts for the infant Jesus; usually represented as a king of Nubia (WordNet).

Fan JR

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