Multilocular cystic clear cell renal cell carcinoma imaging with histopathologic correlation

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1 CASE REPORT J. Trnsl. Med. Res 2015;20(4): Multiloculr cystic cler cell renl cell crcinom imging with histopthologic correltion Emi Mrinel Pred 1,3, Ion Griel Lupescu 1,3, Adrin Dijmrescu 3, Gelu A. Pop 1,3, Mugur C. Grsu 1,3, Monic Hortopn 2 1 Rdiology, Medicl Imging nd Interventionl Rdiology Deprtment, Fundeni Clinicl Institute, Buchrest, Romni 2 Deprtment of Pthologic Antomy, Fundeni Clinicl Institute, Buchrest, Romni 3 University of Medicine nd Phrmcy «Crol Dvil», Buchrest, Romni ABSTRACT Corresponding uthor: Ion G.Lupescu, MD Rdiology nd Medicl Imging Deprtment, Fundeni Clinicl Institute 258 Fundeni Str., Sector 2, Buchrest, Romni E-mil: Renl cell crcinom (RCC) cuses significnt moridity nd mortlity. Cler cell RCC (ccrcc) is the most common histologic sutype, with worse prognosis compred with other histologic sutypes. The indolent vrint of ccrcc is the multiloculr cystic RCC (McRCC). Imging fetures reflect the vrious histologicl findings of ech histologic sutypes. Recent dvnces in imging technology permit erly nd more pproprite dignosis of RCC nd fcilitte optiml mngement. Key words: Multiloculr cystic cler cell renl cell crcinom, renl cell crcinom, cler cell renl crcinom, imging, CT, MRI INTRODUCTION Renl cell crcinom (RCC) is the most common dult renl epithelil cncer, ccounting for more thn 90% of ll renl mlignncies nd is the most lethl of ll urologic cncers (1,2). The most of the cses re dignosed incidentlly during imging exmintions (2-6). In 2004 World Helth Orgniztion reviewing renl neoplsms clssifiction, recognizes severl distinct histologic sutypes of RCC. Cler cell RCC is the most common sutype nd hs less fvorle prognosis thn ppillry RCC nd chromophoe RCC. Multiloculr cystic RCC is distinct sutype of cler-cell RCC tht ppers to hve fvorle prognosis. We report cse of McRCC, which is rre entity, comprising pproximtely 1 to 2% of ll renl tumors nd pproximtely 4 % of ll ccrcc (lthough the truth is unknown due to the lck of this sutype efore 2004) (3,4,5) CASE REPORT Our ptient is men, 65 yers old, Known with type II dietes mellitus in tretment with orl mediction, treted hypertension, BRD, prosttic hypertrophy, hospitlized in the Urology Deprtment of Fundeni Clinicl Institute, Copyright Celsius Pulishing House Journl of Trnsltionl Medicine nd Reserch, 20 (4),

2 Emi Mrinel Pred et l in order for investigtions nd tretment for lrge right renl cyst dignosed y ultrsound. Cliniclly, the ptient hd t dmission: good generl condition, no fever, diuresis present, cler urine nd out 10 cm moile, plple tumor in right kidney lodge. Urinry trct ultrsound demonstrted norml left kidney, hlf-filled ldder, prostte volume = 24 cc; right kidney with 111 mm cyst of posterior vlve, with multiple sept. It is recommended computed tomogrphy exmintion with iodinted non-ionic contrst. Computed tomogrphy (CT) reveled voluminous, well-defined, extrrenl cystic mss (dimensions: 11/8 cm) with predominnt fluid component, contining one smll expnsile dense nodule (out 18/13 mm), with intense enhncement, locted just ove of some enhnced sept spects tht re chrcteristics for Bosnik cyst type IV. (figure 1). Left kidney with norml CT spect. Mgnetic Resonnce (MR) exmintion performed in conjunction with CT exmintion noted expnsive mss found in the right kidney with extrrenl development predominntly, hving the sme dimensions descried t CT, with predominnt cystic component mnifesting wter restriction on diffusion sequence nd contining smll solid nodule with strong enhncement, locted on the nterior wll nd severl enhncing, irregulr sept, which gives multiloculr ppernce - fetures tht suggests mlignnt cystic lesion, requiring surgicl resection. MR conclusion ws: multiloculr right renl cystic tumor. (figure 2) After clinicl evlution nd preliminry l tests is decided on , rdicl nephrectomy through the lumr pproch, with fvorle postopertive evolution Histopthologicl prmeters were put into evidence fter histologicl preprtion with H&E stining: cystic tumor formtion with exophytic development in right kidney prenchym (mx. 12/9 c d Figure 1 - CT exm [nonenhnced - () nd fter intrvenous contrst dmnistrtion in cortico-medullry - (), nephrogrphic - (c) nd dely, prenchyml phse - (d)]: voluminous, well-defined, extrrenl cystic mss with predominnt fluid component (*), contining n expnsive smll dense nodule (rrow), with intense iodinte enhncement, locted just ove of some enhnced sept (rrowhed) 254 Journl of Trnsltionl Medicine nd Reserch, 20 (4), 2015

3 Multiloculr cystic cler cell renl cell crcinom imging with histopthologic correltion - cse reports c e d f Figure 2 - MR exm shows n expnsive right kidney cystic mss with predominnt fluid component [etter ssessed on () - short TE SS FSE nd () long TE SS FSE cquistions] contining smll solid nodule (*), tht enhnce strongly [fter intrvenous gsdolinium dmnistrtion - (c, d)] nd severl enhncing, irregulr sept, which gives multiloculr ppernce (rrow). Note wter restriction of the fluid content on the diffusion weighted sequence (e nd f) cm), with multiloculr pttern, polymorph, polychromtic res with intrseptl solid spect. The histopthologicl spect suggest renl cell crcinom - cler cell type, Fuhrmn nucler grde 2, multiloculr cystic pttern, richly vsculrized, with foci of intrtumorl leeding. The prolifertion of renl tumor evolves out of the well defined outline of conjunctivecollgen pseudo-cpsule lmellr without invsive spects. (figure 3). Renl cpsule, vessels, nd perinephric ft were free. The histopthologicl conclusions where: multiloculr cystic cler cell crcinom cler, Fuhrmn nucler grde 2. DISCUSSION With the incresing vilility of imging nd methods ecoming more efficient, it incresed the numer of incidentlly detected renl tumors in smll stges (symptomtic). Ultrsound (US) is guilty for most of incidentl discovered renl msses. Computed Tomogrphy (CT) is considered the min method for renl cystic lesions chrcteriztion (6), llowing ccurte mesurement of oth fluid ttenution vlues (0-20HU), ut lso of ftty, solid or clcified components. Also, CT underpinned the foundtions of Bosnik clssifiction, widely emrced y rdiologists nd urologists. Tht CT-sed renl cyst clssifiction system, proposed in 1986 nd modified y Bosnik in 1993, provides guide for further imging evlution or intervention (7). In its ctul form, cysts re clssified into four ctegories, different from the point of view of mngement: ctegory I (simple renl cysts ), ctegory II (enign renl cysts, tht re minimlly complicted, ut not in need of surgery), ctegory III (more complicted cystic lesions, potentilly mlignnt, in need of surgery) nd ctegory IV (clerly mlignnt cystic crcinoms). The clssifiction revised in 1993 include suset of minimlly complicted lesions (ctegory IIF), lesions tht Journl of Trnsltionl Medicine nd Reserch, 20 (4),

4 Emi Mrinel Pred et l Figure 3 - Photomicrogrph of histologic section (H nd E, 200) shows: () typicl histologic ppernce of cler cell renl cell crcinom, showing epithelil cells with cler cytoplsm nd distinct cell memrne, seprted y fine rnching network of vsculr tissue. () cler cell epithelil lining with fluid-filled lumen, smll polypoid projection into lumen with foci of intrtumorl leeding (rrow); lso note dense firous component (*) Tel 1 - The Bosnik Renl Cyst Clssifiction System Ctegory I enign simple cyst II enign cystic lesions minimlly complicted IIF minimlly complicted lesions tht could e mnged with follow-up III more complicted cystic lesions IV clerly mlignnt cystic crcinoms Criteri nd Mngement - hirline-thin wll; does not contin sept, clcifictions, or solid components; wter ttenution / fluid signl nd does not enhnce; - no intervention, no follow-up is needed - my contin few hirline-thin sept in which perceived (not mesurle) enhncement my e pprecited; fine clcifiction my e present in the wll or sept; - uniformly high-ttenuting lesions tht re shrply mrginted nd do not enhnce re included in this group; - < 3 cm in dimeter - no intervention, no follow-up is needed - my contin multiple hirline-thin sept; perceived (not mesurle) enhncement of hirline-thin smooth septum or wll cn e identified; - there my e miniml thickening of wll or sept, which my contin clcifiction tht my e thick nd nodulr, ut no mesurle contrst enhncement is present; there re no enhncing soft-tissue components; - totlly intrrenl nonenhncing high-ttenuting renl lesions (>3 cm) re lso included in this ctegory; - generlly well mrginted; - need follow-up to prove their enignity y showing stility - with thickened irregulr or smooth wlls or sept nd in which mesurle enhncement is present; - this ctegory includes complicted hemorrhgic or infected cysts, multiloculr cystic nephrom, nd cystic neoplsms; - need surgicl intervention in most cses, s neoplsm cnnot e excluded; - need histologic dignosis, s even gross oservtion y the urologist t surgery or the pthologist t gross pthologic evlution is frequently indeterminte - cn hve ll of the criteri of ctegory III ut lso contin distinct enhncing soft-tissue components independent of the wll or sept; - need to e removed re more complex thn ctegory II cyst ut re still thought to e enign nd require only seril imging to confirm stility (8, 9) (tle 1). MR Imging (MRI) is helpful when renl lesions re detected y others imging methods ut not so well chrcterized. MRI is offering greter detils in complicted renl cysts, with sept nd solid nodules or in multiloculr cystic lesions (7). Multiplnr MRI cquisitions 256 Journl of Trnsltionl Medicine nd Reserch, 20 (4), 2015

5 Multiloculr cystic cler cell renl cell crcinom imging with histopthologic correltion - cse report re very importnt in cystic renl msses evlution, ecuse the imging plnes of the sequences my e modified to est depict the mss (10, 11). Multiloculr cystic RCC cnnot e lwys differentited from other complex cystic renl lesions on imges. As the nme suggests, multiloculr cystic RCC is multiseptted cystic RCC whose sept contin smll clusters of cler cells. In these cses, the CT or MR ppernces cn rnge from Bosnik IIF cyst to Bosnik IV cystic lesion. Multiloculr cystic RCCs typiclly mnifest s multiloculr cystic tumors, vrile-sized, seprted from the kidney y firous cpsule. Microscopic exmintion of the surgiclly resected tissue is often necessry for dignosis of certin (3,12, 13). Diffusion-weighted imging (DWI) is new technique for renl msses, providing quntifiction of the Brownin motion of wter molecules in tissues, which depends on tissue orgniztion, cellulrity, the integrity of cell memrnes, nd extrcellulr spce tortuosity (14). Qulittive nd quntittive informtion re otined regrding tissue chrcteriztion without the need for Gdolinium dministrtion. Severl uthors (Goyl, Touli nd Wng) showed tht the men ADC vlue of cler cell RCC ws found to e significntly higher thn tht of non-cler cell RCC, while Sndrsegrn did not find ny significnt difference in the ADC vlues of cler cell RCCs nd non-cler cell mlignncies (15,16,17,18). Histopthologicl nlysis demonstrtes cysts lined y monolyer of epithelil cells with cler cytoplsm (13). Multiloculr cystic renl cell crcinom is distinguished from the other sutypes of RCC sed on the high incidence of stge I disese t initil presenttion (83% to 88%), infrequent metstses, nd n extremely high cure rte following surgicl resection of the tumor (19,20). CONCLUSION Histology of cler cell RCC is reflected in dvnced imging chrcteristics. Accurte imging nd histologic chrcteriztion of RCC nd its sutypes (ie multiloculr cystic RCC) re very importnt for the proper mngement of the ptient nd its prognostic. Acknowledgement This work received finncil support through the project entitled CERO Creer profile: Romnin Resercher, grnt numer POSDRU/159/1.5/S/135760, co-finnced y the Europen Socil Fund for Sectoril Opertionl Progrmme Humn Resources Development REFERENCES 1. Ele JN, Suter G et l, Pthology nd genetics of urinry systemnd mle genitl orgns, Lyon, Frnce, IARC Press, Jeml A, Tiwri RC, Murry T, Ghfoor A, Smuels A, Wrd E, et l, Cncer sttistics, CA Cncer J Clin Jn-Fe;54(1): Hindmn NM, Bosnik MA, Rosenkrntz AB, Lee-Felker S, Melmed J. Multiloculr cystic renl cell crcinom: comprison of imging nd pthologic findings. AJR Am J Roentgenol Jn;198(1):W Murd T, Komiko W, Oysu R, Buer K. Multiloculr cystic renl cell crcinom. Am J Clin Pthol My;95(5): Coric FA, Iczkowski KA, Cheng L, Zincke H, Blute ML, Wendel A, et l. Cystic renl cell crcinom is cured y resection: study of 24 cses with long-term followup. J Urol Fe;161(2): Hrtmn DS, Choyke PL, Hrtmn MS. From the RSNA refresher courses: prcticl pproch to the cystic renl mss. Rdiogrphics Oct;24 Suppl 1:S Pred Emi Mrinel, Pop G.A., Lupescu Ion G. Wht s old, new nd especilly helpful in cystic renl msses imging dignosis?, ECR 2015, 8. Bosnik MA. Dignosis nd mngement of ptients with complicted cystic lesions of the kidney. AJR Am J Roentgenol Sep;169(3): Hwng JH, Lee CK, Yu HS, Cho KS, Choi YD, Hm WS. Clinicl Outcomes of Bosnik Ctegory IIF Complex Renl Cysts in Koren Ptients. Koren J Urol Jun;53(6): Isrel GM, Bosnik MA. How I do it: evluting renl msses. Rdiology Aug;236(2): Bosnik MA. The current rdiologicl pproch to renl cysts, Rdiology Jn;158(1): Murphy WM, Grignon DJ, Perlmn EJ. Tumors of the Kidney, Bldder, nd Relted Urinry Structures. Wshington, DC: Americn Registry of Pthology; 2004; AFIP Atls of Tumor Pthology; Fourth Series, Fscicle Prsd SR, Humphrey PA, Cten JR, Nrr VR, Srigley JR, Cortez AD, et l. Common nd uncommon histologic sutypes of renl cell crcinom: imging spectrum with pthologic correltion. Rdiogrphics Nov- Dec;26(6): ; discussion Pdhni AR, Liu G, Koh DM, Chenevert TL, Thoeny HC, Tkhr T, et l. Diffusion-weighted mgnetic resonnce imging s cncer iomrker: consensus nd recommendtions. Neoplsi Fe;11(2): Goyl A, Shrm R, Bhll AS, Gmngtti S, Seth A, Iyer VK, et l. Diffusion-weighted MRI in renl cell crcinom: surrogte mrker for predicting nucler grde nd histologicl sutype. Act Rdiol Apr 1;53(3): Touli B, Thkur RK, Mnnelli L, B JS, Kim S, Hecht EM, et l. Renl lesions: chrcteriztion with diffusion-weighted imging versus contrstenhnced MR imging. Rdiology My;251(2): Wng H, Cheng L, Zhng X, Wng D, Guo A, Go Y, et l. Renl cell crcinom: diffusion weighted MR imging for sutype differentition t 3.0 T. Rdiology Oct;257(1): Sndrsegrn K, Sundrm CP, Rmswmy R, Akisik FM, Ryderg MP, Lin C, Aisen AM. Usefulness of diffusion-weighted imging in the evlution of renl msses. AJR Am J Roentgenol Fe;194(2): Ele JN, Suter G, Epstein JI, et l eds. Pthology nd Genetics of Tumours of the Urinry System nd Mle Genitl Orgns. Lyon, Frnce: IARCC Press; World Helth Orgniztion Clssifiction of Tumours. 20. Suzign S, López-Beltrán A, Montironi R, Drut R, Romero A, Hyshi T, et l. Multiloculr cystic renl cell crcinom: A report of 45 cses of kidney tumor of low mlignnt potentil. Am J Clin Pthol Fe;125(2): Journl of Trnsltionl Medicine nd Reserch, 20 (4),

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