Mantle Cell Lymphoma with Atypical Radiologic Presentation: Case Report

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1 Mntle Cell Lymphom with Atypicl Rdiologic Presenttion: Cse Report Linfom de céluls del mnto con presentción rdiológic típic: Presentción de cso Key words (MeSH) Lymphom, mntle-cell Lyphom Tomogrphy, X-ry computed Plrs clve (DeCS) Linfom de céluls del mnto Linfom Tomogrfí computrizd por ryos X Jun Esten López Amy 1 Betriz Molinres Arévlo 1 Crlos González Vásquez 1 An Mrí Alvrdo Benvides 2 Rfel Llms Otero 2 Ricrdo Urie González 2 Crolin Echeverri Jrmillo 3 Summry Mntle cell lymphom is Non-Hodgkin Lymphom (NHL). In cses of disseminted disese, lymphdenopthy, splenomegly, B-symptoms nd skin disese re present. Lymphom ffecting the spleen hs severl rdiologic presenttions, rnging from norml ppernce, to splenomegly or multiples focl solid lesions. Cystic presenttion of lymphom is rre nd few cses hve een reported, none of them involving the spleen. We report cse of 59-yer old femle ptient with cystic spleen lesions tht fter splenectomy were dignosed s Mntle cell lymphom. Resumen El linfom de céluls del mnto hce prte del sugrupo de linfoms no Hodgkin (LNH). Este se mnifiest con denoptís, esplenomegli, síntoms B y compromiso cutáneo socido enfermedd disemind. El compromiso esplénico se present como: Esplenomegli sin lesión focl; lesiones sólids únics o múltiples e infiltrción del zo sin cmios morfológicos ni lesiones focles. L prición de linfom con lesiones quístics es extremdmente rr, se encuentrn solo unos cuntos csos en l litertur, ninguno de ellos en el zo. Se expone el cso de un pciente de 59 ños de edd, quien consultó por dolor dominl intermitente. En los estudios dignósticos se oservó esplenomegli con lesiones sólids y quístics. Se relizó esplenectomí con estudio histoptológico que confirmó compromiso por linfom de céluls del mnto. 1 Rdiologist, Hospitl Plo Toon Urie. Medellin, Colomi. 2 Rdiology resident, Universidd CES. Medellin, Colomi. 3 Pthologist, Hospitl Plo Toon Urie. Medellin, Colomi. Introduction Mntle cell lymphom (MCL) is B-cell neoplsm originting in the mntle re of the lymphoid follicles (1). It is prt of non-hodgkin s lymphoms, nd is chrcterized y eing ggressive (2). In the spleen, this pthology mnifests minly with splenomegly (3). The cystic form of the lymphom is extremely rre, in the literture we present cse presenttions, ut none, to our knowledge, with splenic involvement. Cse presenttion It is femle ptient of 59 yers of ge, with intermittent dominl pin of long durtion. In extr-institutionl studies, intr-dominl cystic lesions were found with no conclusive cytologicl findings. In order to expnd the studies, tomogrphy Computed tomogrphy (CT) (Figure 1) of the domen reveled splenomegly with solid, low density nd confluent nodulr lesions, nd multiple cystic lesions, the lrgest exophytic without cler origin (pncres vs. spleen). Mgnetic resonnce imging (MRI) with contrst medium (Figure 2) confirmed its splenic origin. A splenectomy ws performed (Figure 3) to clrify the etiology of the lesions nd the pthologicl study demonstrted mntle cell lymphom (Figure 4). Rev. Colom. Rdiol. 2017; 28(3):

2 Figure 1. CT scn of the domen in the xil plne: ) A lrge cystic lesion (sterisk) is oserved in the midline with enhncement of its wlls, displcing the hed of the pncres, mesenteric vessels nd intestinl loops. Also, low-density solid nodulr lesion is oserved in the spleen (rrows). ) Coronl reconnissnce: Splenomegly, cystic lesions nd solid spleen nd lrge intrdominl, centrl cystic lesion. It is not cler whether it origintes in the spleen or the pncres. c d e Figure 2. Sequence with coronl T2 informtion: ) It shows splenic cystic lesions of high signl with some thin sept nd smll peripherl solid nodulr component in the dominnt cystic (rrow), exophytic lesion. Solid lesions show slight low signl. ) Coronl with T1 informtion fter contrst medium: A slight enhncement of the exophytic centrl cyst wll is noted. c) Sequences B1000 nd d) ADC mp: They show splenic lesions of high signl (T2 shinethrough) (rrows), there is no restriction to the diffusion. e) Axil imge with T2 informtion: Shows the sign of the pek (rrow) which confirms the splenic origin of the dominnt cystic lesion Mntle Cell Lymphom with Atypicl Rdiologic Presenttion: Cse Report. López J., Molinres B., González C., Alvrdo A., Llms R., Urie R., Echeverri C.

3 Figure 3. () Surgicl specimen: Enlrged spleen, loulted contours y solid nd cystic lesions. ) Mcroscopic prt: Lrge exophytic cyst originting in the spleen. Courtesy of Dr. Víctor Quintero. c d Figure 4. ) Hemtoxylin nd eosin stining, centrl (str) nodule mde up of uniform popultion of intermedite size lymphocytes with thick chromtin. ) Immunohistochemicl stining for CD20 intensely positive y B lymphocytes (cells surrounded y white circle). c) Positive CD5 stining (cells surrounded y circle) confirming tht they re B lymphocytes. d) Positive cyclin D1 stining in mntle cell lymphoms (rown cells). Overview of Mntle Cell Lymphom MCL is B cell neoplsm originting in the mntle re of the lymphoid follicles. Three ptterns re recognized: mntle re, nodulr nd diffuse. These, efore immunohistochemicl techniques, show CD20 +, CD5 +, CD43 +, nd cyclin D1 + s nucler mrker. MCL hs lso een relted to the trnsloction of chromosomes (4, 7). It ppers t men ge of 65 yers, with predominnce in the mle popultion (8) nd is usully dignosed in dvnced stges, where extrnodl compromise is found. The clinicl Rev. Colom. Rdiol. 2017; 28(3): mnifesttions re not very different from those of other types of NHL. It usully ppers with lymphdenopthy nd splenomegly (50%), B symptoms (30%), centrl nervous system involvement (10%) nd cutneous involvement ssocited with disseminted disese (9). The course of the disese is vrile. It cn e pinless or symptomtic nd chronic s the presented clinicl cse or, on the contrry, very ggressive, in the literture n verge survivl is mentioned etween 3 to 5 yers (10). 4761

4 Discussion The lymphom comprises group of tumors derived from cells of the immune system, heterogeneously histologiclly. It is the fifth most common tumor in the United Sttes nd the fifth cuse of mortlity secondry to cncer (4). It is divided into 2 lrge groups: Hodgkin s lymphom (HL) nd non-hodgkin s lymphom (NHL) (5), the ltter eing sudivided into B-cell lymphom nd T cells (4) (Tle 1). Lymphom is the tumor tht most commonly ffects the spleen (2), either primry or secondry (2). It my e y HL or NHL, lthough the second (1) is more common, which hs een found in up to 70% of postmortem studies (2). The histologic sutype tht most commonly ffects the spleen is lrge cell lymphom. On the other hnd, primry splenic lymphom is rre, representing only 1-2% nd hs the histologicl chrcteristics of NHL in most cses. Mntle cell lymphom is n ggressive B-cell NHL (6). It hs n evolution with reltively frequent relpses, short remissions nd, unlike other sutypes of NHL, therpy hs not een shown to e curtive (7). Tle 1. Sutypes of lymphoms Hodgkin No Hodgkin Tipo de linfom Nodulr Clssic Nodulr sclerosus Rich in lymphocytes Mixed Cellulrity Lymphocyte Depletion B cells T cells Lympholstic leukemi Chronic lymphocytic leukemi Plsmocytom MALT Mntle Folliculr B-cell diffuse Burkitt Lympholstic lymphom Mycosis fungoides Peripherl T-cell lymphom Angioinmunolstic Anplstic Spleen nd lymphom in imges According to studies showing high correltion of mcroscopic imges nd pthology, possile ptterns of lymphom in the spleen re descried (5, 2): Norml spleen, only with microscopic infiltrtion.» Splenomegly without focl lesion. This is the most common pttern. The MCLs tht compromise the spleen re mnifested minly y splenomegly, chrcteristic finding of this lymphom (11).» Single focl mss.» Multiple nodulr lesions tht my hve milir presenttion or multiple msses lrger thn one centimeter. In TC, the msses re usully low signl without significnt enhncement with the contrst medium (2). In some cses its density my e low, lmost like wter, ecuse of its high cellulrity (3) or necrosis of liquefction (2). Other reported fetures re dystrophic clcifictions secondry to necrosis, ut this is exceptionlly uncommon in untreted lymphoms (2). The sequences with T1 nd T2 MRI informtion for the evlution of spleen lymphoms hve low sensitivity ecuse lymphomtous infiltrtion hs ehvior very similr to tht of helthy splenic prenchym (2). The solid lesions re of low signl in imges with informtion T1 nd of high signl with informtion T2 (2). Altertions in the sequences with T1 informtion following the dministrtion of contrst medi llow more ccurte determintion of the extent of compromise in the spleen (2). Differentil dignoses of low signl lesions in the spleen correspond 4762 Mntle Cell Lymphom with Atypicl Rdiologic Presenttion: Cse Report. López J., Molinres B., González C., Alvrdo A., Llms R., Urie R., Echeverri C.

5 to mcrocystic lymphtic mlformtion, the most common of ll. Also, we must think of infectious diseses, hemngiom nd, s lst option, the ngiom of the littorl cells (2). Cystic lymphoms reported in the literture re few, one of which corresponds to lrge, diffuse, heptic B-cell lymphom, mnifested s single cystic lesion (12). There re two cses in the literture studied s pseudocysts: one in the drenl glnd nd nother in the prtesticulr region, with dignosis of diffuse lrge B-cell lymphoms, n extremely rre cse (13). Another cse of cystic lesion of the lrynx tht corresponds to mntle cell lymphom is found (6). Finlly, diffuse lrge B-cell lymphom ws descried in the cystic jw (14). The cse is interesting ecuse it is primry lymphom of the spleen tht, in ddition to splenomegly, shows solid lesions of low signl nd cystic lesions, one of them exophytic of gret size with mss effect on the neighoring structures. The role of the imges is limited in these cses since the min finding is the splenomegly, which is cused y multiple pthologies. In ddition, it does not present ny specific signs for its dignosis; however, this dignosis should e tken into ccount etween splenomegly differentils. B-cell lymphom (DLBCL) occurring in pseudocysts: do these tumors elong to the ctegory of DLBCL ssocited with chronic inflmmtion? Am J Surg Pthol. 2012;36(7): Koivisto T, Bowles WR, Mgjn WA, Rohrer M. Mlignnt lymphom in mxill with cystic involvement: A cse report. J Endod. 2013;39(7): Aville from: Correspondence Ricrdo Urie González Residente de Rdiologí Universidd CES Digonl 29D # 9A Sur-150 Medellín, Colomi ricrdouriegonzlez@gmil.com Received for evlution: Decemer 22, 2016 Accepted for puliction: My 18, 2017 Conclusion MCL is n ggressive B-cell NHL, which t present does not hve therpies with curtive potentil. It minly ffects extrnodl orgns, isolted splenomegly is its most frequent form of ppernce. Our cse is interesting ecuse it is lymphom with typicl presenttion due to its cystic nd solid presenttion nd, to our knowledge, is the first cse found in the primry lymphom literture of the spleen with cystic lesions. References 1. Alousi A, Ptls MN, Scglione M, Romno L, Soto JA. Cross-sectionl imging of nontrumtic emergencies of the spleen. Curr Prol Dign Rdiol. 2014;43(5): Bhti K, Shdev A, Reznek RH. Lymphom of the spleen. Semin Ultrsound, CT MRI. 2007;28(1): Welch JS, Foyil K V, Powers MLE, Middleton WD, Brtlett NL. Solid, low-ttenution splenic lesions on computed tomogrphy in ptients with indolent lymphom often signl trnsformtion: A series of ten ptients. Clin Lymphom, Myelom Leuk. 2012;12(6): Rdemker J. Hodgkin s nd Non-Hodgkin s lymphoms. Rdiol Clin North Am. 2007;45(1): Anis M, Irshd A. Imging of dominl lymphom. Rdiol Clin North Am. 2008;46(2): Groom KL, Ruhl DS, Sniezek JC. Mntle cell lymphom presenting s scculr cyst. Otolryngol. Hed Neck Surg. 2011;146(1): Khl BS, Gordon LI, Dreyling M, Gscoyne RD, Sotomyor EM. Advnces nd issues in mntle cell lymphom reserch: Report of the 2014 mntle cell lymphom consortium workshop. Leuk Lymphom. 2015;(April): Gollu MJ. Imging of gstrointestinl lymphom. Rdiol Clin North Am. 2008;46(2): Chen R, Sánchez J. Clinicl mngement updtes in mntle cell lymphom. Oncol J. 2016;30(4): Lewis RB, Mehrotr AK, Rodríguez P, Mnning MA, Levine MS. Gstrointestinl lymphom: Rdiologic nd pthologic findings. RdioGrphics. 2014;34(1): Frmps E. Lymphoms: Bsic points tht rdiologists should know. Dign Interv Imging. 2013;94(2): Genro V, L Tonzi A, Weisenerg E. Primry heptic lymphom presenting s n. J Clin Oncol. 2013;31(2): Boroumnd N, Ly TL, Sonstein J, Medeiros LJ. Microscopic diffuse lrge Rev. Colom. Rdiol. 2017; 28(3):