대망에서발생한췌장외의고형가성유두종양 : 증례보고 1 이염식 이영환 강유진 전세정 박성훈 윤기중 2 고형가성유두종양 (Solid pseudopapillary tumor) 은주로췌장에서발생하는저등급의악성종양으로다른장기로전이는잘하지않아비교적예후가좋다고알려져있으며췌장이외의장기에서발생하는경우는극히드물다. 저자들은고형및출혈성낭종형태로대망에서발생하여복막에결절성파종을일으킨비전형적췌장외의고형가성유두종양의영상의학적소견을문헌고찰과함께보고하고자한다. 고형가성유두종양 (Solid pseudopapillary tumor) 은췌장내원당시시행한 CT에서불균일하게조영증강되는고형병의외분비성종양의약 1-2% 를차지하는비교적드문종양으변과저감쇠의낭성병변이혼합된 15 10 12 cm 크기의커로, 주로젊은가임기여성에서발생하며, 낮은악성도와비교다란종괴가좌상복부에서관찰되었다. 낭성병변의내부에는적좋은예후를보인다 (1, 2). 대부분의고형가성유두종양은약 5 mm 크기의작은결절성석회화가있었고, 액체-액체층췌장에서발생하며, Taizo 등의보고에의하면췌장외의장기 (fluid-fluid level) 을보여내부에출혈이동반되었을가능성에서발생한고형가성유두종양은매우드물어현재까지 5예가이있었다 (Fig. 1A). 다수의작은결절성병변들이간주위와보고되었고대망, 복막, 간등에서발생하였다 (3-7). 골반강의복막에관찰되었고, 복강내에다량의복수가고여본증례는고령남자환자의대망 (greater omentum) 과복있었다 (Fig. 1B). 주위의간이나췌장등고형장기에낭성병막에발생한고형가성유두종양으로초음파검사, 전산화단층촬변이나전이를시사할만한병변은없었고, 전이성림프절로영 (CT), 자기공명영상 (MRI) 등의영상의학소견을중심으로의심할만한림프절역시보이지않았다. 증례를보고하고자한다. CT 촬영후세포흡인검사를위해시행한초음파검사에서좌상복부에고형병변과낭성병변이혼합된커다란종괴가보였으며, 낭성병변의에코는불균일하였다 (Fig. 1C). 약 40 cc 증례보고의붉은색의혈성복수를흡인하였으나병리학적검사상종양 71세남자환자가내원약 3개월전부터발생한복부팽만과세포는검출되지않았다. 약 4개월후추적관찰중시행한불편감을주소로내원하였다. 과거력상당뇨와고혈압으로각 MRI에서좌상복부의종괴는이전에시행한 CT에비하여낭각 7년과 8년째약물치료중이었으며, 40년전에폐결핵으로성병변의크기가증가하여있었다 (Fig. 1D). 낭성병변은 T2 치료받은기왕력이있었고, 수술과관련된과거력이나특이한강조영상에서상부의고신호강도와하부의저신호강도로보가족력은없었다. 이학적검사상좌상복부에커다란종괴가촉이는액체-액체층이보였고, T1강조영상에서는전체적으로고진되었고, 혈액검사상 hemoglobin 9.3 g/dl, WBC 7100/μ 신호강도를보여낭성병변내부에혈액성분이있음을의심 L, total serum protein 6.5 g/dl, serum albumin 3.8 할수있었다 (Fig. 1E). 조영증강 T1강조영상에서는고형병 g/dl이었으며, 종양표지자중 alpha-fetoprotein(afp) 변이불균일한형태로조영증강되었다. 처음내원시시행한 0.96 ng/ml, carcinoembryonic antigen(cea) 1.41 CT에서는보이지않았으나췌장경부변연부위에약 2 cm ng/ml, cancer antigen(ca)-125 19.6 IU/mL로각각정상크기의 T1강조영상에서낮은신호강도를보이고, T2강조영상범위였고, CA 19-9은 1279 IU/mL로매우증가하여있었다. 에서높은신호강도를보이는낭성병변이있었고, 이는대망에서발생한고형가성유두종양의복막전이로인한병변으로 1 원광대학병원영상의학과추정되었다. 2 원광대학병원진단병리과병리조직학적검사를위하여대망에있는종괴의고형부위이논문은 2007년도원광대학교의교비지원에의해서수행됨. 이논문은 2009년 8월 7일접수하여 2009년 9월 7일에채택되었음. 에서복강경하종괴절제술을시행하였고복강경검사에서대망 67
이염식외 : 대망에서발생한췌장외의고형가성유두종양 의종괴와동반된파종성결절들을확인할수있었다 (Fig. 1F). 육안적병리소견상절제된병변은 2 1.5 1.5 cm 크기의난원형의백색의고형종괴였으며, 현미경병리소견상 hematoxylin-eosin 염색에서균일한원형의종양세포와풍부한미세혈관을보였으며, 종양조직내에고형혹은가성유두양형태와낭성변화를보였다 (Fig. 1G). 면역조직화학검사에서 alpha-1-antichymotrypsin, neuron-specific enolase (NSE), vimentin에양성반응을보였고, C-kit, hepatocyte, S-100 protein, calretinin, Panck, synaptypsin, chromogranin A, CD10, CD34, smooth muscle actin, HMB45, and GFAP에음성반응을보였다. 이상의병리조직검사와면역조직화학검사결과및영상의학적소견을종합하여대망에서발생하고복강내에파종된형태의고형가성유두종양으로최종진단할수있었다. 환자는진단후증상완화 A B C E D Fig. 1. A 71-year-old man with peritoneal solid pseudopapillary tumor arose at the greater omentum. A, B. Contrast enhanced axial CT images show a large complex mass at left upper quadrant, with mixed cystic and solid nature, and slight contrast enhancement of peripheral solid portion of the mass. There is no mass at pancreas. Multiple enhancing nodules at right subphrenic space with large amount of ascite. C. Abdominal US shows a complex mass with hyperechoic solid portion and anechoic cystic portions at LUQ area. D, E. MR images show that the mass has heterogeneous internal signal intensity, which indicates that the mass is more complex than suggested by the CT findings. Axial T1-weighted image (D) shows homogenous high signal intensity of the cystic mass and T2-weighted MR image (E) shows that the mass has fluid-fluid level, a finding consistent with hemorrhage. 68
F G Fig. 1. F. Photograph of peritoneal mass at greater omentum in the laparoscopic biopsy. G. Micro-photogram shows multifocal cystic changes (arrows) and scanty microvasculature in monomorphic solid pattern of tumor tissue (hematoxylin-eosin, original magnification 100). Table 1. Review of Extrapancreatic Solid Pseudopapillary Tumors Author,(ref.) Age/Sex Location Size(cm) Recurrence Prognosis Taizo,(3) 45/M Omentum 18 Liver, peritoneum 8 years 2 months; DOD Fukunaga,(4) 46/F Omentum 5-3 months alive; NED Ishikawa,(5) 13/F Mesocolon 8-3 years alive; NED Kim,(6) 41/F Liver 30-13 months alive; NED Klöppel,(7) 25/M Peritoneum 8-2years alive; NED Present case 71/M Omentum, Peritoneum 15-7months; DOD NED, no evidence of disease; DOD, died of disease 를위한보존적치료만을시행하였고약 7개월후에고형가성고형가성유두종양은임상증상은복통, 복부불편감또는복유두종양의진행에의한폐전이와동반된폐렴에의해사망하부종괴등지속적인종양의크기증가와관련된비특이적인였다. 양상을보인다. 조직학적으로외막을가지는종괴로내부의출혈정도에따라고형, 혼합형, 또는낭성병변등으로다양하게보일수있다. 점차적인퇴행성변화와가성유두양변성등다고찰른췌장의종양과는구별되는특징이있다 (10). 고형가성유두고형가성유두종양은 solid and cystic tumor, solid and 종양은췌장미부에가장흔하고, 비교적경계가명확하며주 papillary epithelial neoplasm, papillary-cystic 로내부에낭성병변을포함하는종괴로발견된다. 조영전 neoplasm, papillary cystic epithelial neoplasm, CT에서낭성병변은약 +20에서 +50 HU를보이며, 조영 papillary-cystic tumor 등의많은동의어로불리다가, 1996 후 CT에서종양의주변부의고형병변에조영증강을보인다. 년에 World Health Organization(WHO) 에서외분비췌장 MRI에서도비교적경계가좋고 T1, T2강조영상에서종괴내종양의국제조직학적분류에따라고형가성유두종양으로명부에불균일한신호강도를보이며, 특징적으로 T1강조영상에명되었다 (8). 서고신호강도, T2강조영상에서불균일한저신호강도를보이특징적으로 20-30대의젊은가임기 Asian와 African- 며이는내부의혈액성분을확인하는데도움이된다. American 여성에서빈도가높게발생하며, 비교적드문종양대부분의고형가성유두종양은췌장에서발생하며, 췌장이으로치료후좋은예후를보인다고알려져있다. 비록대다수외의장기에서발생한경우는매우드물어현재까지약 5예만의고형가성유두종양은양성경과를보이지만 Lam 등 (9) 의이보고되었다 (Table 1)(3-7). 췌장외부에서발생한증례들보고를따르면약 15% 의환자에서전이나주변장기로의침을살펴보면평균연령은 35세이며 (13~46세) 남자 2명, 여범이있었으며, 이러한악성경과를보이는경우는고령이나자 3명이었고크기는평균 14 cm(5~18 cm) 이었고발생부남자환자인경우가많았다. WHO에서는혈관이나신경또는위는각각대망 2예, 간 1예, 복막 1예그리고결장간막이 1예림프절이나간으로의침범이있을경우분명한악성종양으로였다. 오직 1예만이치료후추적기간중간과복막에재발한고형가성유두암 (solid-pseudopapillary carcinomas) 로분류고형가성유두종양에의하여사망하였고, 다른환자들은치료하였다 (8). 후평균 19개월추적검사에서재발이나전이소견이발견되 69
이염식외 : 대망에서발생한췌장외의고형가성유두종양 지않았다. 각각의증례들의영상의학적소견은비교적큰크기의종괴로주변과경계가잘지어졌고내부는낭성과고형성분이혼합된병변으로보였으며, 면역조직화학검사에서 alpha-1-antitrypsin 과비멘틴 (vimentin) 에양성반응을보였다. 본증례는 71세남자환자로췌장에서발생하는고식적인고형가성유두낭종에비하여호발연령및성별이맞지않았고췌장내부에종양이보이지않아서처음진단에서는고형가성유두종양을생각하기어려웠다. 좌상복부의위직하방대망부위에큰종괴가있었으며, 복막과대망을따라수많은작은고형종괴들이파종된형태로관찰되어복막에파종된암종이나위장관간질종양 (Gastrointestinal Stromal Tumor, 이하 GIST) 혹은비전형적인악성중피종 (Mesothelioma) 을감별진단에포함하였다. 그러나본환자의초음파, CT, MRI 등영상의학적검사소견을후향적으로분석할때, 낭성병변과고형병변이혼합된비교적경계가좋은커다란종괴였으며, 낭성병변내부에혈액성분과석회화가있었고, 조영증강영상에서주변부고형병변에조영증강되는양상을보여췌장의전형적인고형가성유두종양의소견과비교적일치하였다. 병리조직검사에서도 H-E stain에서균일한원형종양세포와풍부한미세혈관및가성유두양변성을보여췌장에서발생하는고형가성유두종양의소견과일치하였다. 기존의보고에의하면고형가성유두종양은면역조직화학검사에서 alpha-1- antitrypsin, vimentin, NSE, alpha-1-antichymotrypsin 등에양성반응을보이고 chromogranin, epithelial membrane antigen, cytokeratin등에음성반응을보인다고알려져있다 (1). 본증례의면역조직화학검사소견에서도 GIST는배제할수있었고고형가성유두종양에합당하였다. 췌장에서발생한고형가성유두종양은췌장의낭성종양인장액성낭종 (serous cystadenomas), 점액성낭성종양 (mucinous cystic neoplasm), 낭성변화를일으킨내분비종양이나췌장암등과감별이필요하다. 본증례와같이췌장외부에서발생하고복강내로파종된고형가성유두종양경우는영상소견만으로는복강이나후복강에발생한악성육종 (sarcoma) 이나폐암, 대장암, 유방암, 갑상선암등의복강내전이암, 악성위장관간질종양이나악성중피종등과도감별 이어려울것으로판단된다. 하지만, 영상의학적검사나혈액학적검사상전이암으로진단할만한원발병소를찾을수가없었고, GIST는커다란낭성병변을동반한여러개의종괴로관찰되는경우가드물고, 악성중피종역시결절성병변이나종괴보다는불분명한형태의두꺼운복막비후형태로보이는경우가많다는점이본증례와다를것으로판단된다. 결론적으로고령의남자에서대망에발생하여복막에파종된췌장외의고형가성유두종양을영상의학적소견을중심으로보고한다. 참고문헌 1. Coleman KM, Doherty MC, Bigler SA. Solid pseudopapillary tumor of the pancreas. Radiographics 2003;23:1644-1648 2. Choi JY, Kim MJ, Kim JH, Kim SH, Lim JS, Oh YT, et al. Solid pseudopapillary tumor of the pancreas: typical and atypical manifestations. AJR Am J Roentgenol 2006;187:178-186 3. Hibi T, Ojima H, Sakamoto Y, Kosuge T, Shimada K, Sano T, et al. A solid pseudopapillary tumor arising from the greater omentum followed by multiple metastases with increasing malignant potential. J Gastroenterol 2006;41:276-281 4. Fukunaga M. Pseudopapillary solid cystic tumor arising from an extrapancreatic site. Arch Pathol Lab Med 2001;125:1368-1371 5. Ishikawa O, Ishiguro S, Ohhigashi H, Sasaki Y, Yasuda T, Imaoka S, et al. Solid and papillary neoplasm arising from an ectopic pancreas in the mesocolon. Am J Gastroenterol 1990;85:597-601 6. Kim YI, Kim ST, Lee GK, Choi BI. Papillary cystic tumor of the liver. A case report with ultrastructural observation. Cancer 1990;65:2740-2746 7. Klöppel G, Maurer R, Hofmann E, Luthold K, Oscarson J, Forsby N, et al. Solid-cystic (papillary-cystic) tumours within and outside the pancreas in men: report of two patients. Virchows Arch A Pathol Anat Histopathol 1991;418:179-183 8. Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. World Health Organization: histological typing of tumours of the exocrine pancreas. 2nd. Berlin: Springer-Verlag, 1996 9. Lam KY, Lo CY, Fan ST. Pancreatic solid-cystic-papillary tumor: clinicopathologic features in eight patients from Hong Kong and review of the literature. World J Surg 1999;23:1045-1050 10. Adsay NV, Klimstra DS. Cystic forms of typically solid pancreatic tumors. Semin Diagn Pathol 2000;17:81-88 70
J Korean Soc Radiol 2010;62:67-71 The Growth of an Extrapancreatic Solid Pseudopapillary Tumor from the Greater Omentum: A Case Report 1 Yeum Sik Lee, M.D., Young Hwan Lee, M.D., Eu Gene Kang, M.D., Se Jeong Jeon, M.D., Seong Hoon Park, M.D., Ki Jung Yun, M.D. 2 1 Department of Radiology, Wonkwang University Hospital 2 Department of Pathology, Wonkwang University Hospital A solid pseudopapillary tumor is an uncommon tumor of the pancreas that rarely metastasizes to other organs and usually shows good prognosis. An extrapancreatic tumor arising from a solid pseudopapillary tumor is very rare. We report a case of an atypical extrapancreatic solid pseudopapillary tumor that arose from the great omentum and disseminated to the peritoneum, and discuss the radiologic findings, including the CT, US, and MRI. Index words : Pancreatic Neoplasms Omentum Tomography, X-Ray Computed Magnetic Resonance Imaging Address reprint requests to : Young Hwan Lee, M.D., Department of Radiology, Wonkwang University Hospital, 344-2 Shinyong-dong, Iksan, Jeonbuk 570-711, Korea. Tel. 82-63-859-1920 Fax. 82-63-851-4749 E-mail: yjyh@wonkwang.ac.kr 71