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1 186 소아외과 : 제 11 권제 2 호 2005 년 Vol. 11, No, 2, December 2005 내배액술을시행한담관낭종에서발생한악성변성및간전이 - 1 예보고 - 경북대학교의과대학외과학교실 지문종 윤혁진 강신용 박진영 서 론 증 례 담관낭종에서암이발생하는경우는상당히드물지만절제해내지않은담관낭종의경우에주로 10대이후에 % 에서담관암이발생한다고한다 1. 특히황달혹은간기능의저하가동반된나이가많은남자환자에서악성의빈도가높으며, 만약담관낭종에서담관암이발생한경우에는 Whipple씨수술혹은추가적인간절제술과같은근치적인수술을시행하더라도예후가상당히불량하다고한다 2. 최근저자들은생후 3개월에타병원에서담관낭종으로진단받은후담관낭종을절제하지않고 Roux-en-Y 낭종공장문합술만시행하고, 10년후에담관낭종절제술을시행하기위하여본원으로전원된소아환아에서불완전한담관낭종절제술후에발생한담도암및간내전이성병변을경험하였기에보고하는바이다. 이논문의요지는 2005 년 6 월서울에서개최된제 21 회대한소아외과학회춘계학술대회에서구연되었음교신저자 : 박진영, 대구광역시중구삼덕 2 가 50 번지경북대학병원일반외과 Tel : 053) , Fax : 053) kpnugs@yahoo.co.kr 10세여아가한달간지속된간헐적인우측상복부동통을주소로본원에내원하였다. 과거력상생후 3개월째에타병원에서담관낭종으로진단받은후 Roux-en-Y 낭종공장문합술울시행받았으며그이후로는특별한문제없이지내오다가본원내원 1 달전부터간헐적인우측상복부동통이발생하였으나황달이나고열은동반되지않았다. 내원당시시행한이학적검사상다른이상소견은관찰되지않았으며, 말초혈액검사치는정상범위내에속했다. 복부초음파및복부전산화단층촬영상에서과거에소장을이용한내배액수술이시행된 1형담관낭종으로확인되었으며쓸개내에다발성담석들이발견되었다. 개복술을시행하였으며방사상으로확장된 1형담관낭종이 Roux-en-Y 형태로공장에연결되어있었다. 담낭절제술을시행하고담관낭종절제를시도하였으나장기간의상행성담도염에의한심한염증성변화로인하여담관낭종의벽이심하게두꺼워져있었고문맥과의유착이심해서완전히담관낭종을절제하

Fig. 2. The ultrasonography-guided liver biopsy reveals the moderately differentiated adenocarcinoma. (H & E stain, 100) 지못하고 Roux-en-Y 간공장문합술을시행하였다.

복부전산화단층촬영에서잔여담관낭종내에폴립모양의결절이있었으며간구역 7번에전이성병변으로의심되는병변이보였다 ( 그림 1). 십이지장은잔여담관낭종에서발생한종양성병변에의해폐쇄되어있었으며위장이 심하게늘어나있었다.

2 지문종외 : 내배액술을시행한담관낭종에서발생한악성변성및간전이 - 1 예보고 Fig. 1. Abdominal CT scan shows polypoid nodular lesion in the remnant choledochal cyst (A) and suspicious metastatic lesion in the segment 7 of the liver (B). Fig. 2. The ultrasonography-guided liver biopsy reveals the moderately differentiated adenocarcinoma. (H & E stain, 100) 지못하고 Roux-en-Y 간공장문합술을시행하였다. 수술후환아는특별한문제없이지내왔으며 1년후외래에서시행한복부전산화단층촬영에서직경 2cm 의담관낭종이남아있었다. 술후 2년 6개월째간헐적인복통과고열, 오심및구토를주소로다시본원에내원하였다. 복부전산화단층촬영에서잔여담관낭종내에폴립모양의결절이있었으며간구역 7번에전이성병변으로의심되는병변이보였다 ( 그림 1). 십이지장은잔여담관낭종에서발생한종양성병변에의해폐쇄되어있었으며위장이 심하게늘어나있었다. 종양표식자 CA 19-9 가 209 U/mL( 정상범위 : 0-37 U/mL) 로증가되었으며초음파유도하에시행한간침생검에서전이성선암으로판명되었다 ( 그림 2). 환아는지속적인구토로탈수가심했으며십이지장의완전폐쇄를해결하기위하여개복술을시행하였다. 간우엽에 4 cm 크기의종괴가만져졌으며. 십이지장및위장은심하게확장되어있었으며잔여담관낭종부위는심한유착과암성변화로접근자체가불가능하여고식적인위공장문합술만시행하였다. 환아는 3번째수술 4 개월후간부전으로사망하였다. 고찰담관낭종은선천적으로담도가확장되어있는드문질환으로주로아시아인들에서호발하며남녀비는 1:3-4로여성에서호발한다 3,4. 현재가장널리사용되는담관낭종분류법은 Todani 분류법이며제 1형이가장흔하다. 증상은성인에서는복통이주증상이며, 소아에서는황달이주된증상이다 5. 제

3 188 소아외과제 11 권제 2 호 2005 년 1형담관낭종은환자의 92 % 에서동반되는 common channel로알려진췌담관의이상배열 (abnormal arrangement of the pancreatobiliary ducts:apbd) 과관련이있다. 2 cm 이상의긴 common channel은췌장염, 바터씨팽대부의협착, 담관낭종과같은다양한병들을야기시키며 APBD는담도내로췌장액의역류를증가시켜서총담관의벽이췌장효소에노출이되어담관낭종이발생한다고한다 6. 담관낭종에서암이발생하는경우는상당히드물며, 그빈도는 2.5 % 에서 30 % 까지보고되고있다 4,7-10. 암발생의위험요소는나이와관련이있으며 10세미만에서 0.7 %, 11세에서 20세사이에 6.8 %, 20세이후에 14.3 % 가발생한다고한다 11. 특히황달혹은간기능의저하가동반된나이가많은남자환자에서악성의빈도가높으며, 만약담관낭종에서담관암이발생한경우에는 Whipple씨수술혹은추가적인간절제술과같은근치적인수술을시행하더라도예후가상당히불량하다고한다 2. 게다가과거에담관낭종의치료로내배액법을시행한환자는암발생률이 50% 증가하고술후평균 10년에서암이발생한다고한다 12. 담관낭종에서암이발생하는원인으로 Todani 등 12 (1987) 은내배액법을시행한경우에장액이담관낭종내로역류되어서췌장액이활성화되고결국담관낭종의염증성변화가심해지고담도상피세포의지속적인자극으로인해서암이발생한다고주장하였다. Imazu 등 13 (2001) 은담도내로췌장액이역류되는원인이 APBD로인한괄약근의기능저하및 Oddi 괄약근과십이지 장사이의높은압력차이로인해서생긴다고주장하였다. 과거에담관낭종의치료로배액술을시행한후에악성변성의위험성때문에최근 20년간간외담관낭종의치료에대한개념이바뀌어졌다 12,14. 과거에는담관낭종의치료로담관낭종을절제하지않고단순히십이지장이나공장을이용한내배액법을사용하였지만남아있는담관낭종에서담즙저류, 담석형성, 담도염, 췌장염그리고최악의경우에악성변화가동반될수가있다 15. 게다가단순담관낭종문합술을시행한경우에는지속적인상행성담도염으로염증성변화및유착이심해서추가적인수술이필요한경우어렵게만든다. 특히내배액술후에주로문합부협착과같은합병증의빈도가높다고보고되고있고 14,16 담도암의빈도가증가하기때문에간외담관낭종의치료는일차적인완전담관낭종절제술및담도위장관계문합을시행하는것이다. 담도계와위장관계의문합복원은보통 Roux-en-Y 간공장문합술을이용하지만드물게간십이지장문합술을사용하기도한다. 하지만제 3 형담관낭종의경우에는암이발생할확률이낮아서내시경적조임근절개술을시행하여효과적으로치료할수있다고한다 17. 제 4형담관낭종 ( 간내담관낭종 ) 의치료로부분간절제술이시행되는데이경우에는담도암이발생할확률이낮기때문에편측간을침범한경우와증상이있는환자에서만부분간절제술을시행한다 18. 하지만광범위한간내담관낭종의경우에는악성변화의예방을위해서간이식이고려되기도한다 19.

4 지문종외 : 내배액술을시행한담관낭종에서발생한악성변성및간전이 - 1 예보고 Fugisaki 등 20 (1999) 은담관낭종의일차절제술후에남아있던췌장내담도에서발생한악성종양을경험하였으며일차적으로담관낭종을제거하였어도췌장내담도에서암이발생할수있으므로지속적인추적관찰이필요하다고하였다. 저자들도생후 3 개월때내배액법을시행한후 10세에 2차적인담관낭종절제술을받았던환자에서절제되지않고남아있던담관낭종의벽에 암이발생한 1예를경험하였다. 담관낭종의일차적인치료로담관낭종의완전절제및 Roux-en-Y 간공장문합술을시행해야하며술후에도장기간의지속적인추적관찰이필요하다고사료된다. 참고문헌 1. Todani T, Toki A: Cancer arising in choledochal cyst and management. Nippon Geka Gakkai Zasshi 97: , Lee KF, Lai EC, Lai PB: Adult choledochal cyst. Asian J Surg 28:29-33, de Vries JS, de Vries S, Aronsono DC, Bosman DK, Rauws EA, Bosma A, Heij HA, Gouma DJ, van Gulik TM: Choledochal cysts: age of presentation, symptoms, and late complications related to Todani s classification. J Pediatr Surg 37: , Lipsett PA, Pitt HA, Colombani PM, Boitnott JK, Cameron JL: Choledochal cyst disease. A changing pattern of presentation. Ann Surg 220: , Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K: Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134: , Komi N, Takehara H, Kunitomo K, Miyoshi Y, Yagi T: Does the type of anomalous arrangement of pancreaticobiliary ducts influence the surgery and prognosis of choledochal cyst? J Pediatr Surg 27: , Bloustein PA: Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 67:40-46, Shi LB, Peng SY, Meng XK, Peng CH, Liu YB, Chen XP, Ji ZL, Yang DT, Chen HR: Diagnosis and treatment of congenital choledochal cyst: 20 years experience in China. World J Gastroenterol 7: , Liu CL, Fan ST, Lo CM, Lam CM, Poon RT, Wong J: Choledochal cysts in adults. Arch Surg 137: , Jan YY, Chen HM, Chen MF: Malignancy in choledochal cysts. Hepatogastroenterology 49: , Voyles CR, Smadja C, Shands WC, Blumgart LH: Carcinoma in choledochal cysts. Age-related incidence. Arch Surg 118: , Todani T, Watanabe Y, Toki A, Urushihara N: Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 164:61-64, Imazu M, iwai N, Tokiwa K, Shimotake T, Kimura O, Ono S: Factors of biliary carcinogenesis in choledochal cysts. Eur J Pediatr Surg 11:24-27, Stain SC, Guthrie CR, Yellin AE, Donovan AJ: Choledochal cyst in the adult. Ann Surg 222: , Rush E, Podesta L, Norris M, Lugo D, Makowka L, Hiatt JR: Late surgical complications of choledochal cystoenterostomy. Am Surg 60: , Rattner DW, Schapiro RH, Warshaw AL:

5 190 소아외과제 11 권제 2 호 2005 년 Abnormalities of the pancreatic and biliary ducts in adult patients with choledochal cysts. Arch Surg 118: , Ladas SD, Katsogridakis I, Tassios P, Tastemiroglou T, Vrachliotis T, Raptis SA: Choledochocele, an overlooked diagnosis: Report of 15 cases and review of 56 published reports from 1984 to Endoscopy 27: , Matos C, Nicaise N, Deviere J, Cassart M, Metens T, Struyven J, Cremer M: Choledochal cysts: Comparisons of findings at MR cholangiopancreatography and endoscopic retrograde cholangiopancreatography in eight patients. Radiology 209: , Bismuth H, Krissat J: Choledochal cystic malignancies. Ann Oncol (suppl 4)10:94-98, Fujisaki S, Akiyama T, Miyake H, Amano S, Tomita R, Fukuzawa M, Yamagami H, Tsubaki K, Arakawa Y, Aleemuzzaman S, Nemato N: A case of carcinoma associated with the remained intrapancreatic biliary tract 17 years after the primary excision of a choledochal cyst. Hepatogastroenterology 46: , 1999

6 지문종외 : 내배액술을시행한담관낭종에서발생한악성변성및간전이 - 1 예보고 Malignant Degeneration and Hepatic Metastasis Ten Years after Internal Drainage of a Choledochal Cyst - a Case Report - Moonjong Ji, M.D., Hyukjin Yoon, M.D., Shinyong Kang, M.D., Jinyoung Park, M.D. Department of Surgery, School of Medicine, Kyungpook National University, Taegu, Korea A 10-year-old-girl was referred to our hospital due to abdominal pain. She underwent Roux-en-Y cystojejunostomy for a choledochal cyst at another hospital at the age of 3 months. Abdominal ultrasonography(usg) and computed tomography (CT) showed type I choledochal cyst and multiple gallbladder stones. Because of severe inflammation and adhesion, partial resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy was performed. Two and one half years later, intermittent abdominal pain, fever, nausea and vomiting occured. Abdominal CT scan showed a polypoid nodular lesion in the remnant of the choledochal cyst and probable metastasis at segment 7 of the liver. The duodenum was obstructed by the mass. Liver biopsy revealed moderately differentiated adenocarcinoma. A palliative gastrojejunostomy was performed to relieve duodenal obstruction. She died of hepatic insufficiency 4 months later. (J Kor Assoc Pediatr Surg 11(2):186~191), Index Words:Choledochal cyst, Malignant degeneration Correspondence:Jinyoung Park, M.D., Department of Surgery, Kyungpook National University Hospital, 50 Samduk-2Ga, Chung-gu, Taegu , Korea Tel : 053) , Fax : 053) kpnugs@yahoo.co.kr

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(49-54)Kjhps004.hwp Biliary Cystadenoma and Cystadenocarcinoma of the Liver Jae Hoon Lee, M.D., Dong Ho Choi, M.D. 1, Kyeong Geun Lee, M.D., Hwon Kyum Park M.D. and Kwang Soo Lee, M.D. Department of Surgery, College of Medicine,