CASE REPORT THE KOREAN JOURNAL OF PANCREAS AND BILIARY TRACT 양성담관협착진단 3 개월후간문부담관암으로확진된 1 예 전창균ㆍ장희윤ㆍ차상우ㆍ조영덕 순천향대학교의과대학내과학교실, 소화기병센터소화기연구소 A Benign Bile Duct Stricture, Finally Diagnosed as a Klatskin Tumor after 3 Months Follow Up Chang Gyun Chun, Hee Yoon Jang, Sang-Woo Cha, Young Deok Cho Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea Klatskin tumors are difficult to diagnosis in the early stages. Therefore early detection is important for curative treatment and prognosis of these tumors. We report a case of a 70-year-old female who was diagnosed with a benign stricture of the common bile duct during a routine health examination at an outside hospital. She underwent dilation of the stricture but of follow up examination the stricture worsened and she was referred to our center. On admission no malignance was suspected on initial abdominal ultrasound, CT, MRCP, ERCP and brush cytology. On follow up CT and MRI at our center, Klatskin tumor was suspected and adenocarcinoma was confirmed on ERCP and IDUS with brush cytology and forcep biopsy. Even if malignancy is not suspected on radiologic and histologic examination it is prudent to perform close follow up of benign biliary strictures. Key words: Klatskin tumor, Endoscopic diagnosis, Benign bile duct stricture 서론 간문부담관암은간외담관에서기원한선암 (adenocarcinoma) 으로해부학적으로간외담관중에서특히간문부, 즉간외담관이좌우간내담관으로분지되는부위에발생한담관암을말한다. 비교적진행이느리고원격전이가흔하지않은질환으로알려져있으나증상발현이늦고간문부주위의주요혈관및신경을쉽게침범하여진단시진행된수술이불가능한경우가많다. 1,2 완치는기본적으로광범위절제가가능한경우에 Corresponding author. 차상우서울시용산구대사관로 59 (140-743) 순천향대학교서울병원소화기병센터소화기연구소 Tel: 02-709-9202, Fax: 02-709-9696 E-mail: swcha@schmc.ac.kr 국한되어질환의초기진단여부가치료성적에많은영향을미친다. 저자들은양성담관협착으로일차진단후추적관찰도중 3개월만에악화된담관확장으로재검사후간문부담관암으로최종진단된증례를경험하였기에이를보고하는바이다. 증례 70세여자환자가 3개월전타병원에서양성담관협착에의한담관확장진단후추적관찰하던도중담관확장의악화소견이관찰돼전원되었다. 음주력, 흡연력, 약물복용력및가족력에는특이소견이없었다. 전신고찰및신체검진결과특이소견은관찰되지않았다. 본원내원당시시행한혈액검사결과 AST/ALT 22/11 IU/L, Total bilirubin 0.3 mg/dl, r-gtp/alp 26/184 IU/L, amylase/lipase 76.9/36 IU/L로측정되 29
전창균외 3 인 30 Fig. 1. (A) Wall thickening of proximal CBD was noted on abdominal ultrasonography. (B) Periductal enhancement and wall thickening of proximal CBD with mild IHD dilatation were noted on CT. (C) There was mild bile duct dilatation with focal lesion in first order branch of right intrahepatic duct on MRCP (white arrow). (D) ERCP showed a focal stricture in first order branch of right intrahepatic duct (white arrow) similar to MRCP. 었으며, 암표지자검사결과 CA19-9 129.31 IU/mL로상승된소견이관찰되었다. 내원 3개월전타병원에서시행한복부초음파, 복부전산화단층촬영, 자기공명영상및내시경적역행성췌담관조영술결과를재검토하였다. 당시복부초음파에서양측담관의확장및총담관기시부벽이두꺼워져있는것이관찰되었고 (Fig. 1A), 복부전산화단층촬영에서총담관기시부의벽이일부조영증강되어보였다 (Fig. 1B). 그러나자기공명췌담관조영술에서는우측담관제 1 분지의부분적인협착이외에특이소견은보이지않아양성협착에의한담관확장이더의심되었으며 (Fig. 1C), 당시내시경적역행성췌담관조영술 (Fig. 1D) 을시행하면서협착된부위에서시행한솔질세포검사 (brush cytology) 에서도상피세포처럼보이는비정형세포 (atypical cells looked like epithelial cell clusters) 만관찰되어양성협착에의한담관확장으로진단후짧은주기로추적관찰하기로했다. 그러나 3개월후, 복부전산화단층촬영을시행한결과좌측담관의확장은큰변화없고우측담관의확장은오히려약간호전되어보였으나총간관부터우측담관의제 2 분지까지담 관주위의조영증강및담관벽의비후가관찰되었다 (Fig. 2A, B). 이후본원으로전원되었으며, 본원에서자기공명영상을촬영한결과전산화단층촬영에비해총간관부위에서조영증강되는편심성담관벽종괴 (eccentric wall enhancing mass) 가더욱선명하게나타났고 (Fig. 2C) 자기공명췌담관조영술에서도총간관부위에서종괴에의한충만결손이확실하게나타났다 (Fig. 2D). 내시경역행성췌담관조영술 (Fig. 3A) 및담관내시경초음파를시행하였고병변의심부위에서솔질세포검사와조직검사 (Fig. 3B) 를시행하였다. 병리검사결과분화도가좋은선암으로확진되었다 (Fig. 4A, B). 이후환자는타병원으로전원총담관, 담낭, 간, 췌십이지장절제술 (common bile duct, gallbladder, liver, pancreas and duodenum, hepatic pancreaticoduodenectomy) 을시행받았고, 병리학적으로 10% 의편평상피암성분이포함된중등도분화를보이는침습성선암 (invasive adenocarcinoma, moderately differentiated with squamous cell carcinoma component, 10%) 소견을보였고, 혈관및림프선의침윤과간좌엽절제면에서잔존종양이확인되었다. 환자는 Klatskin tumor, Bismuth type IIIa (T1N1M0, stage
Klatskin Tumor Mimicking Benign Bile Duct Stricture 31 Fig. 2. (A, B) Periductal enhancement and wall thickening from right 2nd order branch to proximal CBD and cystic duct with mild worsening of IHD dilatation were noted on CT. (C) Eccentric wall enhancing mass was noted at CHD level on MRI (white arrow). (D) Filling defect due to CHD mass was noted on MRCP. Fig. 3. (A) Filling defect due to CHD mass was noted on ERCP. (B) Forcep biopsy was done. IIIb)로 최종확진 되었으며 수술 후 항암 및 방사선 치료 예정이 의 경우 담관주위 침윤형에 해당한다. 담관주위 침윤형은 총 다. 간문부 담관암의 70% 이상을 차지하는 가장 흔한 형으로 병리 학적으로 암조직이 담관벽을 따라 침윤성으로 증식함에 따라 고찰 담관벽이 두꺼워지고 담관이 완전 폐쇄되며 주위 혈관이나 간 실질을 침범한다. 담관암의 진단을 위해서는 영상학적 검사와 간문부 담관암은 육안적 소견에 따라 종괴형성형, 담관내 발 조직병리학적 검사가 필요하다. 영상 진단에는 주로 횡단면 영 육형, 담관주위 침윤형의 세 가지로 분류할 수 있으며, 본 증례 상 기법인 복부 초음파, CT), MRI 등이 사용되고 있으며 침습 대한췌담도학회지 2012년 17권 1호
전창균외 3 인 32 Fig. 4. (A) There was a 3-dimensional cell cluster, which consists of cytoplasm-rich cells with round to oval shaped nucleus. (B) Tumor cells with cytoplasm-rich, increased N/C ratio and hyperchromatic nuclei were noted, which is in accordance with well differentiated adenocarcinoma. 적방법으로경피경간담관조영술, 내시경역행성담관조영술, 수술중담관조영술이사용된다. 3 일반적으로초음파는일차적인선별검사로사용되며, CT와 MRI가병기결정을위한검사로사용된다. 그러나조직학적확진은내시경시술을통하여가능하며, 또한영상학적검사에서불분명한간문부의담관협착에대한악ㆍ양성의정확한감별진단을위해서는내시경접근을통한검사들이필수적이다. 내시경역행성담관조영술을통한조직병리학적검사는통상적으로방사선투시하에서경유두적으로솔질세포검사나겸자생검 (forceps biopsy) 을통하여이루어진다. 솔질세포검사는담관조영술중에유도철선을따라비교적쉽고간편하게적용할수있는검사방법으로널리시행되고있지만, 담관암의세포성이적고결합조직을형성하는특성으로인하여정확한진단을위해필요한충분한양의세포를얻기가쉽지않아암진단의민감도는 30 50% 정도로만족스럽지못하다. 4,5 방사선투시하에서시행되는경유두적겸자생검은 40 80% 의다양한민감도가보고되고있는데, 내시경시술자의경험과겸자생검당시얻어진조직의양에따라결정된다. 6,7 솔질세포검사나겸자생검모두만족스러운진단율을보이지못하지만여러연구에서두가지이상의조직획득방법을함께시행하여민감도가증가됨을보고하였다. 8-10 따라서담관암의조직병리학적확진을얻기위하여내시경역행성담관조영술중에가능한여러방법을동원하여가능한많은조직을얻도록노력하는것이필요하다. 최근담관암의진단율을높이기위해여러가지진보된내시경적방법들이개발ㆍ연구되고있다. 과거 30여년간경구적담관내시경검사는모자내 시경방식이표준으로여겨졌으나여러단점들로인해매우제한적으로시행되어왔는데, 최근에는 SpyGlass direct visualization system이개발되어담관질환에대한진단적유용성이보고되고있으며, 직접경구적담관내시경 (direct peroral cholangioscopy, DPOC) 및프로브기반의공초점레이저현미내시경 (probe-based confocal laser endomicroscopy, pcle) 등이개발되어임상적유용성에대한연구가진행중이다. 11 요약 간문부담관암은조기진단이매우어려운질환으로절제가가능한경우완치도기대해볼수있지만, 대부분의경우진행된상태에서확진되므로예후가매우불량하다. 영상학적검사및조직병리학적검사에서양성으로판명되더라도악성이강하게의심될경우진단을위한보다적극적인노력이필요할것으로생각된다. 저자들은양성담관협착으로진단 3개월후추적검사에서진행된간문부담관암으로확진된 1예를보고하는바이다. 국문색인 : 간문부담도암, 내시경적진단, 양성담도협착 참고문헌 1. Kremer B, Henne-Bruns D, Vogel I, Grimm H, Soehendra N. Impact of staging on treatment of biliary carcinoma. Endoscopy 1993;25:92-99.
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