A Case of Intraductal Papillary Mucinous Carcinoma in the Head of the Pancreas Associated With Absence of the Duct of Wirsung

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1 CASE REPORT A Cse of Intrductl Ppillry Mucinous Crcinom in the Hed of the Pncres Associted With Asence of the Duct of Wirsung Ismu Mkino, Tkhis Ymguchi, Mitsuyoshi Okzki, Tomohru Miyshit, Hidehiro Tjim, Tetsuo Oht Deprtment of Gstroenterologic Surgery, Knzw University Hospitl, Jpn ABSTRACT A Seventy-one-yer-old womn received CT scn for checkup of elevted serum mylse. It reveled solid tumor ssocited with cystic portion in the hed of the pncres. The duct of Wirsung ws not detected y endoscopic retrogrde cholngio-pncretogrphy form Vter s ppill. The cystic lesion nd dilted pncretic duct of the ody of the pncres ws detected y pncretogrphy form the ccessory ppill. We performed pncreticoduodenectomy nd pthologicl dignosis ws intrductl ppillry mucinous crcinom. Fluorogrphy from Vter s ppill of the resected specimen reveled the sence of duct of Wirsung connecting to the Vter s ppill. Fluorogrphy from min pncretic duct in the stump of the resected specimen reveled communiction etween the cystic portion of the tumor nd the duct of Sntorini. Histopthologicl study with seril section lso reveled sence of duct of Wirsung connecting to Vter s ppill. However, the posterior re of the hed of the pncres ws positive for nti-pncretic polypeptide ntiody tht is specific mrker for ventrl pncres on immuno-histopthologicl nlysis, which explined the presence of ventrl pncres. Compring the resected specimen with preopertive pncretogrphy, we confirmed tht the pncretic duct existed in the ventrl pncres connecting to the ccessory ppill. The pthophysiology of sence of duct of Wirsung my not hypoplsi or deficiency of ventrl pncres itself, ut congenitl nomlous connection of the duct of ventrl pncres. INTRODUCTION Pncres divisum is most common vrition of pncretic ductl configurtion during orgnogenesis. Its pthophysiology is complete or incomplete deficiency of communiction etween dorsl nd ventrl pncretic ducts, nd some ptients show the sence of the ventrl pncretic duct of Wirsung (WD) [1, 2]. Pncres divisum is frequently ssocited with pncretitis ecuse of outflow lock of pncretic juice into duodenum through reltively smll orifice of the ccessory ppill [3]. Recently, pncres divisum is thought to hve significnt correltion with prevlence of pncretic cncer [4, 5, 6, 7] nd possily with intrductl ppillry mucinous neoplsm (IPMN) of the pncres [8, 9, 10]. We herein report cse of intrductl ppillry mucinous crcinom (IPMC) ssocited with sence of WD. We exmined the resected specimen with creful ttention to the existence Received Jnury 03rd, Accepted Jnury 28th, 2018 Keywords Pncres; Pncretic Ducts Arevitions IPMN intrductl ppillry mucinous neoplsm; MRCP mgnetic resonnce cholngiopncretogrphy; SD duct of Sntorini; SLE systemic lupus erythemtosus; WD duct of Wirsung Correspondence Ismu Mkino Deprtment of Gstroenterologic Surgery Knzw University Hospitl 13-1 Tkrmchi, Knzw Ishikw , Jpn Tel Fx E-mil i.mkino@stff.knzw-u.c.jp of ventrl pncres, nd considered out the essentil pthophysiology of sence of WD. CASE REPORT A seventy-one-yer-old womn hd received medicl tretment for systemic lupus erythemtosus (SLE). On routine lood exmintion during the tretment for SLE, elevted serum mylse ws pointed out. CT scn reveled well enhnced solid tumor with centrl cystic portion in the hed of the pncres nd dilted pncretic duct of distl side from the tumor (Figure 1). Mgnetic resonnce cholngiopncretogrphy (MRCP) detected cystic portion of the tumor in the hed of the pncres nd ovious dilttion of pncretic duct of distl side from the tumor (Figure 2). WD ws not detected y endoscopic retrogrde cholngiopncretogrphy (ERCP) form Vter s ppill (Figure 3, ). The orifice of the ccessory ppill ws slightly dilted y projection of mucin (Figure 3c). The cystic portion of the tumor in the hed of the pncres nd dilted pncretic duct of distl side from the tumor ws detected y pncretogrphy form the ccessory ppill (Figure 3d). We otined pthologicl finding of denocrcinom y endoscopic ultrsonogrphy (EUS) guided fine needle spirtion iopsy. We dignosed the tumor s IPMC, preopertively. We performed pyloruspreserving pncreticoduodenectomy. Pthologicl dignosis for the tumor ws IPMC derived from the duct of Sntorini (SD). Fluorogrphy from Vter s ppill of the resected specimen reveled the sence of WD connecting to the Vter s ppill (Figure 4). 53

2 Figure 1. CT scn reveled well enhnced solid tumor with centrl cystic portion (rrowhed) in the hed of the pncres nd dilted pncretic duct of distl side from the tumor (rrow). Figure 2. Mgnetic resonnce cholngiopncretogrphy detected cystic portion of the tumor (*) in the hed of the pncres nd ovious dilttion of pncretic duct of distl side from the tumor. Fluorogrphy from min pncretic duct in the stump of the resected specimen reveled communiction etween the cystic portion of the tumor nd SD (Figure 4). Histopthologicl study with seril section lso reveled sence of WD connecting to Vter s ppill. However, the posterior re of the hed of the pncres ws positive for nti-pncretic polypeptide ntiody tht is specific mrker for ventrl pncres on immuno-histopthologicl nlysis, which explined the presence of ventrl pncres (Figure 5, ). DISCUSSION Pncres divisum is common congenitl nomly of the pncres, which results from n norml fusion etween ventrl nd dorsl pncretic ducts during fetl development. The concept of this disorder ws 54

3 c d Figure 3. (). Endoscopic view of Vter s ppill showed no normlity. (). The duct of Wirsung ws not detected y endoscopic retrogrde cholngiopncretogrphy form Vter s ppill. (c). The orifice of the ccessory ppill ws slightly dilted y projection of mucin. (d). The cystic portion of the tumor (*) in the hed of the pncres nd dilted pncretic duct of distl side from the tumor (rrowhed) ws detected y pncretogrphy form the ccessory ppill. Figure 4. (). Fluorogrphy from Vter s ppill of the resected specimen reveled the sence of the duct of Wirsung connecting to the Vter s ppill. (). Fluorogrphy from min pncretic duct in the stump of the resected specimen reveled communiction etween the cystic portion of tumor (*) nd the duct of Sntorini (rrowhed). 55

4 Figure 5. (). The posterior re of the hed of the pncres ws positive for nti-pncretic polypeptide ntiody which explined the presence of ventrl pncres. (). Supposed orderline etween dorsl nd ventrl pncres y the result of immuno-histopthologicl nlysis ws drwn on the photogrphy of the resected specimen. D dorsl pncres; V ventrl pncres; VP Vter s ppill c Figure 6. Compring the resected specimen (, ). with preopertive pncretogrphy, (c). we confirmed tht the pncretic duct existed in the ventrl pncres connecting to the ccessory ppill. Arrowhed: pncretic duct in ventrl pncres connecting to the ccessory ppill Arrow: dilted pncretic duct of descending rnch in dorsl pncres AP ccessory ppill; BD ile duct; VP Vter s ppill redefined s dominnt dorsl duct syndrome (DDDS) nd its vrition ws clssified into 3 types y Wrshw in 1990 [11]. According to this clssifiction, type 1 is defined s seprtion of dorsl nd ventrl pncretic ducts, nd corresponded to clssicl pncres divisum. Type 2 is defined s sence of WD, nd type 3 is filmentous communiction etween dorsl nd ventrl pncretic ducts. Among these vritions, our cse pplied to type 2. In this sitution, whether the ventrl pncres itself is sent s well s WD hs not een clrified. Ventrl pncres cn e distinguished from dorsl pncres y immunohistochemicl technique ecuse islets of ventrl pncres contin lrge mount of pncretic polypeptide compred with those of dorsl pncres [12, 13]. In our cse, y the immunohistochemicl study with nti-pncretic polypeptide ntiody, we confirmed tht ventrl pncres existed in the posterior re of the hed of the pncres in spite of sence of WD connecting to Vter s ppill. Compring the resected specimen with preopertive pncretogrphy, we confirmed tht the pncretic duct existed in the ventrl pncres connecting to the ccessory ppill (Figure 6-c). CONCLUSION In other words, lthough WD connecting to Vter s ppill ws sent, there ws ventrl pncres whose duct connecting to the ccessory ppill. The pthophysiology of DDDS of Wrshw type 2 my not hypoplsi or deficiency 56

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