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1 The Korean Journal of Gastrointestinal Endoscopy Case Report 흑색변을보인다발성위중복낭종 예 강석형ㆍ심기남ㆍ태정현ㆍ정혜경ㆍ정성애ㆍ이선화 * ㆍ이주호 ㆍ성순희 이화여자대학교의학전문대학원내과학교실, * 영상의학교실, 외과학교실, 병리학교실, 의과학연구소 A Case of Multiple Gastric Duplication Cysts Presenting with Melena Seok Hyung Kang, M.D., Ki-Nam Shim, M.D., Chung Hyun Tae, M.D., Hye-Kyung Jung, M.D., Sung-Ae Jung, M.D., Sun Wha Lee, M.D.*, Joo-Ho Lee, M.D. and Sun Hee Sung, M.D. Departments of Internal Medicine, *Radiology, Surgery and Pathology, Ewha Medical Research Institute, Ewha Womans University School of Medicine, Seoul, Korea A gastric duplication cyst is a rare congenital anomaly. Among gastrointestinal duplication cysts, gastric duplication cyst account for only 3.8%. They tend to be symptomatic in early childhood, but asymptomatic during adulthood. So its diagnosis is incidental. Abdominal pain is the most common complaint in adults, and most cases are discovered incidentally by radiological examination or endoscopic gastroduodenoscopy. Preoperative diagnosis of gastric duplication cyst is difficult, and definitive diagnosis requires pathological examination of the lesion. So far, about 8 cases of gastric duplication cyst have been reported in adults in the Korean literature. We report here a case of multiple gastric duplication cysts presenting with melena in a 4-year-old man, which were detected by endoscopic gastroduodenoscopy, abdominal computed tomography and endoscopic ultrasonography. They were treated by complete excision of the multiple gastric duplication cysts by laparoscopic. (Korean J Gastrointest Endosc 20;42: ) Key Words: Gastric duplication cyst, Melena 교신저자. 심기남이화여자대학교의학전문대학원내과학교실 (58-70), 서울시양천구목동 9- 전화 : 팩스 : 이메일 : shimkn@ewha.ac.kr 접수. 20 년 2 월 일승인. 20 년 5 월 7 일 서론위장관중복낭종은초기배아발달이상으로발생하는매우드문선천성질환으로, 이중위중복낭종은전체위장관중복낭종의약 3.8% 정도를차지한다. 대부분 2세이전에증상이발현되어발견되기때문에청소년기이후에발생한위중복낭종의증례는국내에서는 8예만보고되었다. 저자들은흑색변으로내원한 4세남자에서상부위장관내시경, 복부전산화단층촬영및내시경초음파검사로다발성위중복낭종을진단하고치료한 예를경험하여문헌고찰과함께보고한다. 증례 4세남자가하루전부터시작된흑색변과어지러움증을주소로내원하였다. 가족력과성장과정에서특별한병력은없었으며, 전신쇠약감으로 달전부터한약을복용한것이외의 약물복용력은없었다. 신체검사에서혈압 20/65 mmhg, 맥박수 85회 / 분, 호흡수 20회 / 분, 체온 36.8 o C이었으며, 급성병색을보였으나의식은명료하였고결막은창백하였으나공막에황달은관찰되지않았다. 심음과호흡음은정상이었으며복부진찰에서압통과반발통은없었고촉지되는종괴도없었다. 일반혈액검사에서혈색소 8.3 g/dl, 헤마토크리트 23.7%, 백혈구 4,900/mm 3, 혈소판 243,000/mm 3 이었고, 혈청생화학검사에서총단백 4.5 g/dl, 알부민 3. g/dl, 혈액요소질소 33 mg/dl, 크레아티닌 0.8 mg/dl이었고, AST 4 IU/L, ALT 6 IU/L, 총빌리루빈 0.4 mg/dl이었다. 직장수지검사에서종물은만져지지않았으나흑색변이관찰되었고, 비위관세척술에서선홍색혈액이배액되었다. 내원당일시행한상부위장관내시경검사에서상부위체부대만부와분문부에점막하종양이관찰되었고, 상부위체부대만부의종양표면에서출혈성궤양이관찰되어에피네프린 4 cc를주사하여지혈하였다 (Fig. A). 복부전산화단층촬영에서 378 The Korean Journal of Gastrointestinal Endoscopy

Figure. Endoscopic findings. (A) There are two soft cystic submucosal tumors with bloody materials at greater curvature of high body and fundus.

2). 입원 2일째추적상부위장관내시경을시행하였고, 상부위체부대만부의점막하종양에서더이상의출혈은관찰되지않았다 (Fig. B). 내시경초음파검사에서상부위체부대만부와분문부에점막하층에서기원한경계가분명한저에코성낭성종양들이관찰되었으며내부에고에코성부유물질을포함하고있었다 (Fig. 3). 출혈을동반한다발성위중복낭종을의심하고, 입원 4일째복강경적쐐기절제술을시행하였다.

5 cm 크기의낭종 (Fig. 4B) 과위점막내로돌출된상부위체부대만부의 4.0 3.3 2.0 cm 크기의낭종이있었으며, 점막쪽으로돌출된형태의낭종의내부에는갈색의점액질성액체로차있었다. 병리조직학적소견에서낭종은점막과점막고유층, 점막근층과고유근층이모두존재하였으며, 그중에점막쪽으로돌출된형태의낭종은주위의위벽과연결되어있었다.

2 Figure. Endoscopic findings. (A) There are two soft cystic submucosal tumors with bloody materials at greater curvature of high body and fundus. (B) At follow up endoscopy, a cystic tumor (arrow) with an active central ulceration is noted at greater curvature of high body. 중부위체부대만부에경계가분명한다격벽의낭성종양이관찰되었으며, 이는장막쪽으로돌출되어비장의앞쪽과왼쪽부신의위쪽까지닿아있었다 (Fig. 2). 입원 2일째추적상부위장관내시경을시행하였고, 상부위체부대만부의점막하종양에서더이상의출혈은관찰되지않았다 (Fig. B). 내시경초음파검사에서상부위체부대만부와분문부에점막하층에서기원한경계가분명한저에코성낭성종양들이관찰되었으며내부에고에코성부유물질을포함하고있었다 (Fig. 3). 출혈을동반한다발성위중복낭종을의심하고, 입원 4일째복강경적쐐기절제술을시행하였다. 장막쪽으로돌출되어복강경하에서관찰되는분문부의낭성종양과중부위체부대만부의다격벽의낭성종양을절제하였으며, 위점막내로돌출되어있어복강경으로관찰되지않는상부위체부대만부의낭성종양은내시경으로시야를확보하면서복강경적쐐기절제술로제거하였다. 육안소견에서낭종은모두세개로, 장막쪽으로돌출된분문부의 cm 크기의낭종 (Fig. 4A), 중부위체부대만부의 cm 크기의낭종 (Fig. 4B) 과위점막내로돌출된상부위체부대만부의 cm 크기의낭종이있었으며, 점막쪽으로돌출된형태의낭종의내부에는갈색의점액질성액체로차있었다. 병리조직학적소견에서낭종은점막과점막고유층, 점막근층과고유근층이모두존재하였으며, 그중에점막쪽으로돌출된형태의낭종은주위의위벽과연결되어있었다. 점막층의표면은점액질을가지고있는한층의세포로이루어져있고점막고유층에서는호산성의세포질을함유한벽세포가포함된샘구조가관찰되어다발성위중복낭종으로진단되었다 (Fig. 5). 환자는특별한합병증없이수술 6일째퇴원하였으며, 수술후 9개월이지난현재추적관찰중으로건강상태는양호하다. Figure 2. Abdominal CT finding. It shows multiple thin-wall cystic lesions with homogeneous density, surrounding a well-defined adjacent gastric wall. 고찰 위중복낭종은매우드문선천성질환으로, 67년 Vacchus와 Balsius가 85세환자의시신을부검하여처음발견하였으며 9년병리학적으로 Wendel에의해처음으로확인되었다. 위중복낭종의진단기준은낭벽이위장막쪽에위치하며위벽과연결되어있고, 낭의내벽이위장관점막상피세포로덮여있어야하며, 낭이평활근벽으로둘러싸이면서위벽과연결되어있어야한다. 2 그러나일부보고에따르면호흡기상피세포를동반하는경우도있으며, 췌장조직을포함하기도한다. 3,4 위장관중복낭종의발생기전은정확히알려진바없지만, 잘못형성된게실, 자궁내에서위장관의허혈성괴사및관강의재소통결손등으로발생한다고설명하고있다. 5 위중복낭종은남성보다여성에서 2배정도의높은빈도를보이며가족 Vol. 42,. 6 June, 20 ( ) 379

(B) It reveals two well-defined mass lesions located adjacent to the third layer (submucosal layer) of the gastric wall at greater curvature of the fundus.

Gross findings. (A) It shows gastric duplication cysts. There are endophytic cystic mass (4 3.3 2.0 cm) at greater curvature of high body. (B) Another exophytic cystic mass (2.5.

4,6 대개 75% 이상에서생후 2년이내에발견되어, 성인에서발견되는경우는매우드물다. 7 대부분특징적인증상없이모호한복통이나체중감소, 식후불편감을호소하게되나, 본증례처럼이차적으로궤양을발생하여매우드물게흑색변이나토혈, 빈혈등이발생할수있다.

3 Figure 3. EUS findings. (A) It reveals a well-defined mass lesion located adjacent to the third layer (submucosal layer) of the gastric wall at greater curvature of the high body. (B) It reveals two well-defined mass lesions located adjacent to the third layer (submucosal layer) of the gastric wall at greater curvature of the fundus. It shows that a heterogeneous low-echoic pattern is circumscribed by a high-echoic area, suggesting a cystic lesion filled with fluid containing sludge-like material. Figure 4. Gross findings. (A) It shows gastric duplication cysts. There are endophytic cystic mass ( cm) at greater curvature of high body. (B) Another exophytic cystic mass ( cm). 력과는무관한것으로알려져있다. 원위부회장에가장호발하고식도, 대장, 공장, 위, 십이지장순으로발생빈도를보이며, 상대적으로위중복낭종의발생빈도는 3.8% 정도로낮은편이다. 위중복낭종은대부분단일낭종으로 80% 에서위내강과연결성을보이지않는다. 직경은대개 2 cm 이하이며, 대만부혹은전벽이나후벽에흔하게발생한다. 4,6 대개 75% 이상에서생후 2년이내에발견되어, 성인에서발견되는경우는매우드물다. 7 대부분특징적인증상없이모호한복통이나체중감소, 식후불편감을호소하게되나, 본증례처럼이차적으로궤양을발생하여매우드물게흑색변이나토혈, 빈혈등이발생할수있다. 8 또한낭종의감염으로고열, 출혈및유문부폐쇄등이발생할수있으며, 드물게선암이나유암종등의악 성변화가 8% 에서보이기도한다. 8,9 35% 정도에서식도나십이지장낭종, 췌장기형이나부수비장, 장관의이상회전증, 척추기형, 구개열, 폐분리증, Turner 증후군, 대동맥관개존증, 심실중격결손, 비뇨기계이상등의선천성기형을동반할수있다. 6 이번증례를포함한국내보고 (Table ) 0-7 들은비특이적인복통이나식후불편감을보인환자에서진단된예가많았고, 대부분단일낭종이었으며치료전상부위장관내시경과복부전산화단층촬영으로검사하였다. 위중복낭종의방사선진단은과거에는바륨조영술에의존했지만, 최근에는상부위장관내시경과복부전산화단층촬영및 MRI가보편적으로이용되고있다. 8 그러나고농도의단백질성분및다양한성분들이 380 The Korean Journal of Gastrointestinal Endoscopy

4 Table. 9 Cases of Adult Gastric Duplication Cyst Reported in Korea Lee et al 0 Moon et al Yun et al 2 Hwangbo et al 3 Kim et al 4 Lee et al 5 Kim et al 6 Lim et al 7 Present case Age/Sex 23/F 5/M 45/F 8/M 9/M 29/M 7/F 7/F 4/M Symptom General weakness, amenorrhea Upper abdominal discomfort Hematemesis Epigastric pain Epigastric pain Upper abdominal discomfort Postprandial distress, nausea Chronic cough Melena Accompanied malformation Gut malrotation Dorsal pancreatic agenesis, pulmonary cyst Gastric duplication Location Size (cm) LC of antrum PW of HB GC of MB GC of HB GC of MB GC of HB data Diagnostic tool BE, EGD EGD EGD EGD, US, CT, EUS CXR,, EUS Treatment surgery, number; BE, barium enema; EGD, esophagogastroduodenoscopy; US, ultrasonography; CT, computed tomography; EUS, endoscopic ultrasonography; CXR, chest X-ray; LC, lesser curvature; PW, posterior wall; HB, high body; GC, greater curvature; MB, mid body. Figure 5. Microscopic findings. Resected cysts are composed of gastric mucosa with parietal cells (inlet), submucosa, and well developed muscle layers (H&E stain, 200). 중복낭종내에존재하는경우가많아 70% 까지도오진하는경우가발생한다. 9 따라서최근고형병변과낭성병변을감별할 수있으며낭종의벽과주변위장관구조와의상관관계를정확히검사할수있는내시경초음파검사가일반화되면서위중복낭종의진단에이용되는사례가보고되고있다. 8,9 본증례에서도내시경초음파검사는이소성췌장이나지방종, 평활근종, 후복벽에서기원한낭종, 정체성낭종 (retention cyst) 이나농양을감별진단하는데유용하였다. 내시경초음파검사에서위중복낭종은부유물질을포함하지않는저에코성의단순낭종으로관찰될수있으며, 종종고에코성의부유물질을포함하는저에코성의낭종으로도관찰될수있다. 9 수술적절제가주된치료이며, 낭종과위내강과사이가연결이있는경우는쐐기형절제를, 연결이없는경우는낭종절제만을시행한다. 5 중복낭종에동반된기형이나악성변화에의한경우를제외하면완전한외과적절제로별다른합병증없이치유되며, 예후는매우양호하다. 9 본증례는흑색변으로발견된다발성위중복낭종에관한보고로, 드물게출혈증상을보였고, 상부위장관내시경, 복부컴퓨터단층촬영과함께내시경초음파검사가진단에도움이되었으며, 3개의다발성중복낭종에대해복강경적쐐기절제술을했다는점에서의의를둘수있다. Vol. 42,. 6 June, 20 ( ) 38

5 요약 위장관중복낭종은드문선천성질환으로위장관어느부위에나발생하며, 위에서발생하는중복낭종은전체의 3.8% 정도를차지한다. 유아기에서는증상이있으나, 그이후에발견되는경우에는무증상인경우가많다. 위중복낭종은수술전의진단이어렵고최종진단을위해서는병변부의조직검사가필요하다. 본저자들은흑색변을주소로내원한 4세남자에서상부위장관내시경, 복부전산화단층촬영및내시경초음파검사로분문부, 상부위체부대만부와중부위체부대만부의다발성위중복낭종을진단하고복강경적쐐기절제술로치료한 예를경험하여보고한다. 색인단어 : 위중복낭종, 흑색변 참고문헌. Agha FP, Gabriele OF, Abdulla FH. Complete gastric duplication. AJR Am J Roentgenol 98;37: Rowling JT. Some observations on gastric cysts. Br J Surg 959;46: Kim YW, Lee JH, Byun KH, et al. A retroperitoneal foregut duplication cyst: a case report. J Korean Radiol Soc 2006; 54: Theodosopoulos T, Marinis A, Karapanos K, et al. Foregut duplication cysts of the stomach with respiratory epithelium. World J Gastroenterol 2007;3: Bower RJ, Sieber WK, Kiesewetter WB. Alimentary tract duplications in children. Ann Surg 978;88: Lee NK, Kim S, Jeon TY, et al. Complications of congenital and developemental abnormalities of the gastrointestinal tract in adolescents and adults: evaluation with multimodality imaging. Radiographics 200;30: Shew SB, Holcomb GW. Alimentary tract duplication. In: Ashcraft KW, Holcomb GW, Murphy JP, eds. Pediatric surgery. 4th ed. Philadelphia: W.B. Saunders, 2005: Hong SJ, Kim KS, Ham HW, Park JH. Gastric duplication in the newborn. J Korean Pediatr Soc 996;39: Smith JH, Hope PG. Carcinoid tumor arising in a cystic duplication of the small bowel. Arch Pathol Lab Med 985; 09: Lee WH, Yoon KI, Shin GJ, Lee SN, Lee YK. A case of duplication of gastric fundus associated with mal-rotation of the gut and anemia. Korean J Med 983;26: Moon SC, Kim BS, Kim DM, et al. A case of gastric duplication masquerading as gastric submucosal tumor. Korean J Gastroenterol 997;29: Yun JH, Choi DG, Oh SY, et al. A case of gastric duplication in adult. Korean J Gastroenterol 997;29: Hwangbo K, Lee CW, Yun SK, et al. A large gastric duplication cyst resembling a pancreatic pseudocyst in an adult: report of one case. Korean J Gastrointest Endosc 998; 8: Kim HJ, Lee BC, Kweon HJ, et al. A case report of gastric duplication cyst. Korean J Gastroenterol 998;3: Lee HJ, Cho SJ, Yang HK, Lee KU, Choe KJ. Gastric duplication: a case report. J Korean Surg Soc 999;57: Kim HY, Park DJ, Lee HS, et al. of multiple gastric duplication cysts in an adult. J Korean Surg Soc 2006;7: Lim SM, Lee H, Jeon EJ, et al. Gastric duplication cyst associated with dorsal pancreatic agenesis and a cystic pulmonary lesion. Korean J Med 2009;76: Eloubeidi MA, Cohn M, Cerfolio RJ, et al. Endoscopic ultrasound-guided fine-needle aspiration in the diagnosis of foregut duplication cyst: the value of demonstrating detached ciliary tufts in cyst fluid. Cancer 2004;02: Kim PN, Kim IY, Lee BH. CT finding of an esophageal duplication cyst: a case report. J Korean Radiol Soc 989;25: The Korean Journal of Gastrointestinal Endoscopy

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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,