MRI Findings of an Ampulla of Vater Neuroendocrine Tumor with Liver and Lymph Node Metastasis: a Case Report

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1 pissn eissn imri 2018;22: MRI Findings of n Ampull of Vter Neuroendorine Tumor with Liver nd Lymph Node Metstsis: Cse Report Jung Hyun Noh 1, Mi Hyun Prk 1, Seung Kyu Choi 2 1 Deprtment of Rdiology, Dnkook University Hospitl, Dnkook University Shool of Mediine, Cheonn, Kore 2 Deprtment of Pthology, Dnkook University Hospitl, Dnkook University Shool of Mediine, Cheonn, Kore Cse Report Reeived: April 10, 2018 Revised: My 29, 2018 Aepted: My 30, 2018 Correspondene to: Mi Hyun Prk, M.D. Deprtment of Rdiology, Dnkook University Hospitl, Dnkook University Shool of Mediine, 201 Mnghyng-ro, Dongnm-gu, Cheonn 31116, Kore. Tel Fx E-mil: deepv@hnmil.net This is n Open Aess rtile distriuted under the terms of the Cretive Commons Attriution Non-Commeril Liense ( y-n/3.0/) whih permits unrestrited non-ommeril use, distriution, nd reprodution in ny medium, provided the originl work is properly ited. Copyright 2018 Koren Soiety of Mgneti Resonne in Mediine (KSMRM) An mpull of Vter neuroendorine tumor (AOV-NET) is rre suset of gstroenteropnreti neuroendorine tumors (GEP-NETs). Very few studies hve een undertken regrding MRI findings of n AOV-NET. We report on se of 59-yer-old womn dignosed with n AOV-NET with liver nd lymph node metstsis, with n emphsis on the MRI findings. This se shows rre nd preious typil MRI findings of n AOV-NET. The MRI visulized the AOV-NET very well nd is helpful for the differentition of n AOV-NET from other tumors in the mpullry re s well s with tretment plnning. Keywords: Neuroendorine tumor; Ampull of Vter; Mgneti resonne imging (MRI) INTRODUCTION Gstroenteropnreti neuroendorine tumors (GEP-NETs) re heterogeneous group of tumors, whih rise in the ells of the neuroendorine system (1). Ampull of Vter neuroendorine tumors (AOV-NETs) re n extremely rre suset of GEP-NETs nd ounts for 0.3% to 1% of ll GEP-NETs (2). Further, AOV-NETs ount for less thn 2% of mpullry tumors (2-4). Aout 140 ses of AOV-NETs hve een reported (5). However, there re few reports on the MRI findings of AOV-NETs, euse AOV-NETs re not only rre, re lso usully not evluted with MRI. Therefore, we report the MRI findings of rre se of n AOV-NET with liver nd lymph node metstsis. CASE REPORT A 59-yer-old womn with Crohn's disese underwent follow-up esophgogstroduodenosopy (EGD) nd olonosopy, whih reveled the disese ws in remission. However, fol hyperemi nodulr lesion ws deteted t the duodenl mpull. Bulging of the duodenl mpull ws noted while preserving the overlying muos. Lortory exmintion, inluding totl iliruin nd tumor mrkers, were unremrkle. 123

2 Ampull of Vter Neuroendorine Tumor Jung Hyun Noh, et l. An endosopi ultrsound (EUS) nd dominl CT sn were performed. The EUS showed 1.5 m low ehoi mss t the mpull of Vter (AOV). The enhned pnreti phse dominl CT imges show 1.5 m mss with vid, homogeneous enhnement t the AOV (Fig. 1), 9.7 m loulted enhning mss with multifol low ttenuting ysti or neroti hnge in the right hepti loe, nd severl smll, low ttenuting lesions in oth hepti loes (Fig. 1). A 2.3 m enlrged lymph node with prominent enhnement nd entrl neroti hnge ws noted in the left pr-orti re (Fig. 1). Considering the EUS nd CT findings, n AOV NET ws the most likely suggested. In ddition, we suggested tht the lrge hepti mss ws hypervsulr liver metstsis of the AOV-NET. The liver MRI (Ingeni 3.0T, Philips Helthre, Best, the Netherlnds) ws performed in order to further evlute the hepti metstsis. A dynmi enhnement study, using Gd-EOB-DTPA (Primovist), ws performed. The AOV mss mnifested s 1.5 m round lesion with slightly high signl intensity when ompred with pnreti prenhym on T2-weighted imge (Fig. 2) nd low signl intensity on T1-weighted imge (Fig. 2). The AOV mss showed vid, homogeneous enhnement on the rteril phse (Fig. 2) nd isosignl intensity when ompred with pnreti prenhym on the delyed phse (Fig. 2d). The AOV mss exhiited high signl intensity on high vlue diffusionweighted imge (Fig. 2e) without definitive low pprent diffusion oeffiient (ADC) vlue (Fig. 2f). The ommon ile dut nd the min pnreti dut were mildly dilted. The single hepti mss showed ring-like, rdil pperne nd strong enhnement with multifol low signl intensity ysti portions on the rteril phse (Fig. 3) of the enhned liver MRI. The hepti mss showed heterogeneous isosignl intensity, thn liver prenhym on Fig. 1. A 59-yer-old womn with n mpull of Vter (AOV) neuroendorine tumor. The enhned pnreti phse dominl CT imges show () 1.5 m mss (rrow) with vid, homogeneous enhnement t the AOV, () 9.7 m loulted enhning mss with multifol low ttenuting ysti or neroti hnge in the right hepti loe nd () 2.3 m enlrged lymph node (rrow) with entrl neroti hnge ws noted in the left pr-orti re. 124

3 d e Fig. 2. An mpull of Vter (AOV) neuroendorine tumor in 59-yer-old womn. A 1.5 m sized round mss in the AOV (rrow) showed slightly high signl intensity when ompred with pnreti prenhym on T2-weighted imge () nd low signl intensity on T1-weighted imge (). On the enhned MRI, using GD-EOB-DTPA (Primovist), the AOV mss (rrow) showed vid, homogeneous enhnement on the rteril phse () with isosignl intensity when ompred with pnreti prenhym on the delyed phse (d). The AOV mss (rrow) exhiited high signl intensity on high vlue diffusion weighted imge (e) without definitive low pprent diffusion oeffiient vlue (f). f 125

4 Ampull of Vter Neuroendorine Tumor Jung Hyun Noh, et l. d e Fig. 3. A metstti hepti mss in 59-yer-old womn. The single hepti mss showed ring like, rdil pperne, strong enhnement with multifol low signl intensity ysti portions on the rteril phse () of enhned MRI. The hepti mss showed heterogeneous isosignl intensity, thn liver prenhym on the portl-venous phse (), wshout on the delyed phse () nd low signl intensity on the heptoiliry phse (d). There were multifol right signl intensity ysti lesions in the hepti mss on T2-weighted imge (e). f 126

5 g Fig. 3. The hepti mss showed high signl intensity on diffusion weighted imge (f) without definite low pprent diffusion oeffiient vlue (g). Fig. 4. A metstti prorti lymph node in 59-yerold womn. An enlrged lymph node (rrow) with entrl neroti portion ws noted in the left pr-orti re. This lymph node showed prominent enhnement on the rteril phse of n enhned MRI (). The enhned portion of this lymph node (rrow) showed high signl intensity on diffusion weighted imge () nd low pprent diffusion oeffiient vlue (). the portl-venous phse (Fig. 3), wshout on the delyed phse (Fig. 3), nd low signl intensity on the heptoiliry phse (Fig. 3d). There were multifol, right-signl intensity ysti lesions in the hepti mss on T2-weighted imge (Fig. 3e). The hepti mss showed high signl intensity on diffusion-weighted imge (Fig. 3f), without definite low ADC vlue (Fig. 3g). The liver MRI reveled tht severl smll, low ttenuting lesions, previously noted on the CT sn, were T2 right signl intensity ysts. Further, n enlrged lymph node with entrl neroti portion ws noted in the left pr-orti re. This lymph node showed prominent enhnement on the rteril phse 127

6 Ampull of Vter Neuroendorine Tumor Jung Hyun Noh, et l. of n enhned MRI (Fig. 4). Enhned portions of the lymph node showed high signl intensity on diffusionweighted imge (Fig. 4), nd low ADC vlue (Fig. 4), whih indite diffusion restrition. An endosopi iopsy ws performed for the mss nd n AOV nd ultrsonogrphy-guided perutneous iopsy ws performed for the liver mss. Pthologi exmintion onfirmed the AOV mss s NET nd the liver mss s metstti NET. The ptient underwent right heptetomy, pyloruspreserving pnretio-duodenetomy, nd lymph node dissetion. The gross speimen showed 1.5 m welldemrted whitish solid mss t the AOV (Fig. 5) nd 9.0 m well-demrted red tn mss with fishflesh onsisteny in the right hepti loe (Fig. 5). Upon mirosopi exmintion (Hemtoxylin & Eosin stining, 100), the tumor ells of the AOV mss showed nested (Fig. 5), treulr growth ptterns s well s moderte mounts of ytoplsm nd round nulei. Immunostining for synptophysin ws positive in the tumor ells (Fig. 5d). Mirosopi exmintion onfirmed the AOV mss s NET. No invsion to the pnres hed, ommon ile dut, or duodenum ws oserved. The liver mss nd left pr-orti lymph node were onfirmed s metstti NET. DISCUSSION The inidene of GEP-NETs hs inresed in reent yers, due to improved imging tehniques nd the inresed use of endosopy. However, AOV-NETs re very rre (5). AOV NETs re onsidered to hve severl iologi fetures Fig. 5. The gross speimen nd mirosopi exmintion. The gross speimen showed () 1.5 m well-demrted whitish solid mss t the mpull of Vter (AOV) nd () 9.0 m well-demrted red tn mss with fish-flesh onsisteny in the right hepti loe. Upon mirosopi exmintion (Hemtoxylin & Eosin stining, 100), the tumor ells of the AOV mss showed () nested, treulr growth pttern nd hd moderte mounts of ytoplsm nd round nulei. Immunostining for synptophysin ws positive in the tumor ells (d). d 128

7 when ompred with other GEP-NETs. AOV-NETs re onsidered more likely to present s smll lesions, with high tendeny of metstsis (6). However, the nturl history nd prognosti ftors of n AOV-NET hve yet to e estlished. Therefore, there re no stndrd tretments for AOV NETs. Pnretio-duodenetomy is onsidered to e the tretment of hoie. However, few studies suggest endosopi lol resetion nd surgil mpulletomy for well-differentited, smll AOV-NETs (less thn 2 m) nd ptients with high surgil risk (3-5). Endosopi retrogrde holngiopnretogrphy (ERCP) nd EUS re ommonly used for the evlution of mpullry lesions, inluding AOV-NETs. EUS filittes the evlution of the depth of invsion from n AOV-NET (2). Adominl CT nd MRI re generlly used in order to evlute metstsis, ut not to evlute the mpullry lesion itself, euse smll mpullry lesions re usully poorly visulized on CT nd MRI (2). The MRI finding of GEP-NET is mostly hypervsulr tumor, whih is pprent s high enhnement on the rteril phse. These tumors show vrying degrees of heterogeneity, suh s ysti or neroti hnge, depending on the tumor size nd ehvior. Lrge nd fst-growing NETs re more likely to show heterogeneity. NETs show low signl intensity on T1-weighted imge nd high signl intensity on T2-weighted imge (1, 7). Further, the reported CT findings of AOV-NETs re hypervsulr msses t the AOV with or without dilttion of the ommon ile nd min pnreti dut (6, 8). The AOV-NET ws well visulized on the MRI s smll, homogeneous hypervsulr msses in the AOV, whih is typil imging feture of NET in the present se. In NET, liver metstsis is most ommon fter lymph node metstsis nd is importnt for stging, prognosis, nd tretment plnning (1, 7, 9). Therefore, urte evlution is importnt for liver metstsis in n AOV-NET. Similr to the imging findings of primry NET, liver metstsis mnifests s hypervsulr mss on the rteril phse with wshout on the delyed phse of CT or MRI (7). Liver metstsis mnifests s low signl intensity lesion on the heptoiliry phse using heptoyte-speifi gent. In ddition, lrger lesion typilly mnifests s ring-like or rdil heterogeneous enhnement with ysti or neroti res (1, 7, 10). In the present se, hepti metstsis mnifested s lrge hypervsulr mss with multifol T2 right signl intensity ysti portions. The MRI reveled the hepti metstsis s single lesion onfined to the right hepti loe nd the surgeon ould onsider urtive right heptetomy. The lymph node is the most ommon site of metstsis in NET (1, 9). Lymph node metstsis is relted to prognosis in pnreti NET. However, some studies hve reported tht lymph node metstsis is not orrelted with prognosis in n AOV-NET, wrrnting further investigtions. Lymph node metstsis of NET shows prominent enhnement on CT or MRI nd my show neroti hnge (7). In the present se, prominent enhning hypervsulr metstti lymph node with neroti portion ws noted on the rteril phse of MRI. Also, this lymph node showed diffusion restrition. In onlusion, s hypervsulr mss in n AOV with hypervsulr liver nd lymph node metstsis on the MRI, the present se shows rre nd preious typil MRI findings of n AOV-NET. The MRI visulized the AOV-NET very well nd n e helpful with the differentition of n AOV-NET from other tumors in the mpullry re s well s for tretment plnning. REFERENCES 1. Shni DV, Bonffini PA, Fernndez-Del Cstillo C, Blke MA. Gstroenteropnreti neuroendorine tumors: role of imging in dignosis nd mngement. Rdiology 2013;266: Jynt M, Puni R, Kushik R, et l. Neuroendorine tumors of the mpull of vter: presenttion, pthology nd prognosis. JOP 2012;13: Crter JT, Grenert JP, Ruenstein L, Stewrt L, Wy LW. Neuroendorine tumors of the mpull of Vter: iologil ehvior nd surgil mngement. Arh Surg 2009;144: Dumitrsu T, Dim S, Herle V, Tomulesu V, Ionesu M, Popesu I. Neuroendorine tumours of the mpull of Vter: linio-pthologil fetures, surgil pproh nd ssessment of prognosis. Lngeneks Arh Surg 2012;397: Yng K, Yun SP, Kim S, Shin N, Prk DY, Seo HI. Cliniopthologil fetures nd surgil outomes of neuroendorine tumors of mpull of Vter. BMC Gstroenterol 2017;17:70 6. Rmn SP, Fishmn EK. Anormlities of the distl ommon ile dut nd mpull: dignosti pproh nd differentil dignosis using multiplnr reformtions nd 3D imging. AJR Am J Roentgenol 2014;203: Lewis RB, Lttin GE Jr, Pl E. Pnreti endorine tumors: rdiologi-liniopthologi orreltion. Rdiogrphis 129

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