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1 대한내과학회지 : 제 81 권제 6 호 2011 위장관기질종양으로오인된거대유경성간세포암종 1 예 가톨릭대학교의과대학대전성모병원내과 하동천 김보경 김문성 김가영 권기현 안영용 이동수 A Case of Huge Pedunculated Hepatocellular Carcinoma Mimicking a Gastrointestinal Stromal Tumor Dong Cheon Ha, Bo Kyoung Kim, Moon Sung Kim, Ka Young Kim, Ki Hyun Kwon, Young Yong Ahn, and Dong Soo Lee Department of Internal Medicine, Daejeon St. Mary's Hostpital, The Catholic University of Korea School of Medicine, Seoul, Korea Pedunculated hepatocellular carcinoma (P-HCC) can be defined as a carcinoma protruding from the liver with or without a pedicle. Cases of P-HCC showing extrahepatic growth are very rare. P-HCC constitutes % of all HCC cases in Japan and Taiwan, but few cases in Korea have been reported. A 67-year-old female patient was admitted to our hospital with a 6-month history of epigastric discomfort. Examination identified an abdominal mass in the left upper quadrant, and abdominal computed tomography revealed a huge mass adjacent to the fundus of the stomach. We diagnosed this mass as a gastrointestinal stromal tumor because of its size and site. However, postoperative histopathological findings showed that the mass was HCC. Here, we report this case and review the relevant medical literature. (Korean J Med 2011;81: ) Keywords: Hepatocellular carcinoma; Gastrointestinal stromal tumor 서론유경성간세포암종 (pedunculated hepatocellular carcinoma) 이란간으로부터돌출된간세포암종을말한다 [1]. 유경성간세포암종은모든간세포암종의 % 정도인것으로일본과대만의논문에서소수보고된바있다 [2,3]. 우리나라의경우간세포암종은인구 10만명당남자 47.1명, 여자 11.4명 의발생률을보이며 1년에약 1만명의환자가새로발생하여위암, 폐암에이어암등록순위 3위인암일정도로많은질환이기때문에 [4,5] 유경성간세포암종의보고가많을것으로추정되나, 국내에서의유경성간세포암종의증례보고는두차례밖에없었다 [6,7]. 저자들은위저부 (fundus of stomach) 옆의거대종괴에대해위장관기질종양으로의심하고수술을시행하였던환자로, 간세포암종의뚜렷한위험 Received: Revised: Accepted: Correspondence to Dong Soo Lee, M.D. Department of Internal Medicine, Daejeon St. Mary's Hostpital, The Catholic University of Korea, Daeheung-dong, Jung-gu, Daejeon , Korea Tel: , Fax: , endoscope@hanmail.net

- Dong Cheon Ha, et al. A case of huge pedunculated hepatocellular carcinoma - 인자가없음에도불구하고, 수술후목 (pedicle) 이없는외성장형태의유경성간세포암종으로진단된 1예를경험하였기에문헌고찰과함께보고하는바이다.

신체검사에서만성병색을보였고, 의식은명료하였다. 흉부청진에서호흡음은정상이었으며, 심박동은규칙적이었고, 심잡음은들리지않았다. 복부청진에서장음은정상으로들렸으며, 복부촉진에서심와부에경미한압통이있었으나반동압통은없었고, 좌측상복부에큰덩어리가촉지되었다. 검사실소견 : 말초혈액검사에서백혈구는 4,600/mm 3, 혈색소 12.

2 - Dong Cheon Ha, et al. A case of huge pedunculated hepatocellular carcinoma - 인자가없음에도불구하고, 수술후목 (pedicle) 이없는외성장형태의유경성간세포암종으로진단된 1예를경험하였기에문헌고찰과함께보고하는바이다. 증례환자 : 김〇〇, 67세, 여자주소 : 상복부불쾌감과거력및현병력 : 특이과거병력이없는자로내원 6개월전부터시작되어점점악화되는상복부불쾌감을주소로내원하였다. 가족력및사회력 : 음주나흡연은하지않았고, 특이가족력도없었다. 진찰소견 : 내원당시활력징후는혈압 120/80 mmhg, 맥박 76회 / 분, 호흡 20회 / 분, 체온 36.3 였다. 신체검사에서만성병색을보였고, 의식은명료하였다. 흉부청진에서호흡음은정상이었으며, 심박동은규칙적이었고, 심잡음은들리지않았다. 복부청진에서장음은정상으로들렸으며, 복부촉진에서심와부에경미한압통이있었으나반동압통은없었고, 좌측상복부에큰덩어리가촉지되었다. 검사실소견 : 말초혈액검사에서백혈구는 4,600/mm 3, 혈색소 12.1 g/dl, 혈소판은 220,000/mm 3 이었다. 혈청생화학검 사에서총단백은 7.7 mg/dl, 알부민은 4.2 mg/dl, AST 37 IU/L, ALT 23 IU/L, ALP 63 IU/L, 크레아티닌 0.70 mg/dl였다. HBsAg 과 HBsAb 모두음성이었으며, HCV Ab도음성이었다. 종양표지자검사에서 α-fp ng/ml, CA (carbohydrate antigen) IU/mL, CA IU/mL, CEA (carcinoembryonic antigen) 1.03 ng/ml로 α-fp과 CA 125 표지자에서경도의상승이있었다. 방사선소견 : 복부전산화단층결과에서비장과위사이에 cm 정도크기의거대한종괴가있으며경계는비교적명확하고위저부와체상부에인접해있으며비장과횡행결장, 간만곡등과도인접해있었고, 동맥기에이질적인조영증강이있었으나주변에림프절비대나비정상적액체저류는관찰되지않았다 (Fig. 1A). 종괴의위치와크기등을고려하였을때위장관기질종양으로추정진단을하였고, 초음파유도를이용한경피적침생검을시행하였으나, 검체의크기가작아서악성종양의가능성이높은정도라는것밖에알지못했다. PET-CT를시행하였고, 좌상복부에높은대사능 (hypermetabolic) 을가진큰종괴가관찰되었으며, 원격전이가없음을확인하였다 (Fig. 1B). 조직학적소견 : 이에정확한진단및치료를위하여수술을시행하였다. 수술적소견상종괴는간좌측의외측엽 (left lateral lobe of liver) 에이어져있었고, 16.0 cm ( 가로 ) 13.0 cm A B Figure 1. (A) Contrast-enhanced abdominal computed tomography findings. A hepatic arterial-phase scan showed a heterogeneously enhanced huge mass (arrow) in the abdominal cavity. (B) Huge, hypermetabolic mass in the left upper quadrant without distal metastasis

- 대한내과학회지 : 제 81 권제 6 호통권제 616 호 2011 - A B Figure 2. (A) Gross findings of the resected mass; it was encapsulated, 16.0 13.0 8.0 cm in size, and 870 g in weight.

Microscopic findings showed the trabecular arrangement of individual tumor cells. These cells exhibited relatively abundant cytoplasm with bile and severe nuclear pleomorphism.

병리조직검사결과핵다형성 (nuclear pleomorphism) 의정도가심하였으며, 종양세포의세포질에서담즙이관찰되어, Edmondson and Steiner 분류상 Grade IV에해당하는간세포암종으로진단할수있었다 (Fig. 3) [8].

3 - 대한내과학회지 : 제 81 권제 6 호통권제 616 호 A B Figure 2. (A) Gross findings of the resected mass; it was encapsulated, cm in size, and 870 g in weight. (B) On sectioning, the entire cut surface was yellowish-gray, solid, diffusely necrotic, and focally hemorrhagic. 고 찰 Figure 3. Microscopic findings showed the trabecular arrangement of individual tumor cells. These cells exhibited relatively abundant cytoplasm with bile and severe nuclear pleomorphism. This carcinoma corresponded to poorly differentiated hepatocellular carcinoma (Edmondson-Steiner grade 4; hematoxylin & eosin stain, 200). ( 세로 ) 8.0 cm ( 높이 ) 의크기에무게는 870 gm이었으며, 캡슐에둘러싸여있었다 (Fig. 2). 병리조직검사결과핵다형성 (nuclear pleomorphism) 의정도가심하였으며, 종양세포의세포질에서담즙이관찰되어, Edmondson and Steiner 분류상 Grade IV에해당하는간세포암종으로진단할수있었다 (Fig. 3) [8]. 간세포암종의육안적분류는 Eggel의분류에따라괴상형 (massive), 결절형 (nodular), 미만형 (diffuse) 으로분류하는것이널리이용된다 [9]. 유경성간세포암종이란용어는 1897년 Roux가처음으로기술하였으며, 목의유무에관계없이간으로부터돌출된간세포암종으로정의하였다 [1]. 이후 1934년 Goldberg와 Wallenstein이 1.5 cm의목을가지고있고좌엽하면부터돌출된간세포암종을처음으로증례보고하였다 [10]. 유경성간세포암종은돌출된간세포암종이목이관찰되는제1형과이번증례처럼목없이간표면에붙어외성장의형태를띄는제2형으로나눌수있다 [11]. Goldberg 등은유경성간세포암종은선천적으로 Glisson s capsule에서잘못위치된소엽에서생기는것으로판단되는것으로보고하였고 [10], Miyoshi 등역시유경성간세포암종은부엽 (accessory lobe) 에서생기는것으로판단하였다 [12]. 부엽은간우엽에서종종관찰되고, 또한혈관과담관을가진목에의해연결되는경우가흔하다. 부엽은해부학적으로우엽의하면에서흔히관찰되나간의모든엽에서관찰될수있다 [12]. 유경성간세포암종은이소성간조직 (ectopic liver tissue) 에서도또한생길수있으며, 이소성간조직은간의삼각인대 (triangular ligament), 비장, 제대와 (umbilical fossa), 복강동맥총 (celiac axis) 옆의

4 - 하동천외 6 인. 거대유경성간세포암종 1 예 - 후복막강, 장막, 담낭등의다양한위치에서관찰될수있다 [13]. 일본에서는유경성간세포암종이전체간세포암종의 0.24% 에서 3.00% 정도를차지한다는보고가있었다 [3]. 대만에서는 432명의간세포암종환자중에서 18명 (4.2%) 이유경성간세포암종이었다는보고가있으며, 이중 11명 (61%) 의환자에서조직학적분화도가좋은 (well differentiated type) 특징을보이고, 총생존율 (overall survival) 에서유경성간세포암종의평균생존율 (meal survival) 이 94개월로, 그렇지않은일반간세포암종의평균생존율인 54.5개월보다유의하게높았다고보고하였다. 이는유경성간세포암종의경우에는돌출종괴이기때문에간으로의성장이제한되는효과가있고, 일반간세포암종에비하여혈관침범이나타나는경우가적었기때문이라고한다 [2]. 혈관침범이외과적절제이후의재발에중요한위험인자임을고려할때유경성간세포암종의환자에서생존율이더좋은것으로설명하고있는보고도있다 [14]. 일반적으로간세포암종에서는종양크기가클수록나쁜예후를보이지만 [15], 상기참고문헌에따르면 [2,14] 유경성간세포암종에서는종양크기가크다고하더라도절제가능한경계를얻을수있는경우가많은것이생존율을증가시켰을것이라고판단된다. 혈청 α-fp는종종간세포암종과복부의다른종양과감별하는데널리이용되는데, 일본의보고에따르면간세포암종에서의 α-fp 양성비율이 68% 였고, 이중유경성간세포암종에서의 α-fp 양성비율이 66% 로유의한차이가없었다는보고도있지만 [16,17], 일본의또다른연구에서는 7명의유경성간세포암종환자중에서 6명의환자가 α-fp 음성을보였다는보고도있어 [18], 유경성간세포암종을진단하는초기검사에는 α-fp와더불어영상학적진단이매우중요할것으로판단된다. 유경성간세포암종은후복막의침범을나타내는경우, 우측부신종양으로오인되는증례보고가소수있다 [19]. 하지만, 저자들은본증례와같이위저부옆의종괴로나타나는경우를경험하였고, 본증례에서는복부전산화단층촬영에서종괴와간의경계가비교적명확히관찰되었으며, 음주력, 만성간염등의간세포암종의위험인자가없는환자였기때문에추정진단에서간세포암종의가능성을염두하지못한경우로수술을하고나서야진단할수있었던증례였다. 유경성간세포암종은외과적절제가일반적인간세포암종보다용이하게때문에적절한절제가이뤄진경우는더 나은예후를보이지만, 그렇지못했을경우에는빠른성장을보이는경향이있기때문에예후가나쁠수있다 [20]. 따라서초기의적절한진단과치료가이뤄져야할것이고, 간외부의복강내종괴에대해서도간세포암종의가능성을배제하여서는안되겠다. 요 유경성간세포암종은간세포암중에서매우드문형태이다. 국내에서의유경성간세포암종의증례보고는매우드물었고, 대만과일본등의증례가소수보고되어있다. 저자들은위저부옆의거대종괴에대해위장관기질종양으로의심하고진단겸치료를위해수술을시행하였던경우로, 수술후목이없는거대한외성장형태의유경성간세포암종으로진단되었다. 유경성간세포암종은일반적간세포암종에비해절제가능한경우가많지만, 빠른성장을보이기때문에빨리진단을하는것이예후에매우중요하다. 이러한이유로종괴가간이외의위치에있다고하더라도, 초기추정진단시간세포암종의가능성을배제하여서는안될것이다. 약 중심단어 : 유경성간세포암종 ; 위장관기질종양 REFERENCES 1. Roux C. Un cas de cancer primitif du foie avec pericholecystite calcule-use, perforation intestinale: hemostase hepatigue. Rev Med Suisse Romande 1897;17: Yeh CN, Lee WC, Jeng LB, Chen MF. Pedunculated hepatocellular carcinoma: clinicopathologic study of 18 surgically resected cases. World J Surg 2002;26: Horie Y, Katoh S, Yoshida H, Imaoka T, Suou T, Hirayama C. Pedunculated hepatocellular carcinoma: report of three cases and review of literature. Cancer 1983;51: Parkin DM, Whelan SL, Ferlay J, Thomas DB, Teppo L. Cancer Incidence in Five Continents. Vol. 8. Lyon, France: IARC Sci Publ, Ministry Health and Welfare annual report of Korea Central Cancer Registry Park JB, Moon SK. A case report of pedunculated hepatocellular carcinoms. Korean J Pathol 1991;25: Bae HH, Hwang JH, Kim KH, et al. A case of pedunculated hepatocellular carcinoma. J Korean Cancer Assoc 1994;26: Edmondson HA, Steiner PE. Primary carcinoma of the liver: a

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김범수

김범수 Analysis of Outcomes after Resection of Sarcomatous Hepatocellular Carcinoma Purpose: Sarcomatous hepatocellular carcinoma (HCC) is rare. Therefore, the clinicopathologic characteristics and prognosis