Case Report Ewha Med J 2012;35(1):49-53 pissn 2234-3180 / eissn 2234-2591 복강경수술로절제한소장의장간막유건종 1 예 노경태ㆍ성순희 1 ㆍ이령아 이화여자대학교의학전문대학원외과학교실, 1 병리학교실 A Case of Ileal Mesenteric Desmoid Tumor Resected by Laparoscopic Surgery Kyoung Tae Noh, Sun Hee Sung 1, Ryung-Ah Lee Departments of Surgery, 1 Pathology, Ewha Womans University School of Medicine, Seoul, Korea Desmoid tumor is rare neoplasm originated from fibrous sheath or musculoaponeurotic structure. It is classified as benign tumor histologically, but clinically, it has malignant characteristics due to its infiltrative growth to adjacent organ and frequent local recurrence. Especially, mesenteric desmoid tumor shows poor prognosis because of its symptoms of pain, intestinal obstruction, ureter obstruction and fistula formation and high frequency of recurrence. We experienced a case of mesenteric desmoid tumor in a 64-year-old woman with a painless abdominal mass. Laparoscopic exploration was performed and 10 cm sized mesenteric mass was identified, which resected widely and the diagnosis was confirmed with desmoid tumor by pathologic report. We reviewed the feature of the mesenteric desmoid tumor, that is, pathophysiology, clinical presentations, diagnosis, treatment and prognosis. (Ewha Med J 2012;35(1):49-53) Key Words: Desmoid tumor; Mesentery; Laparoscopy 서론 유건종 (desmoid tumor) 은근초나근건막에서유래하는드문종양으로, 조직학적으로는양성이나주변조직으로의침윤과빈번한국소재발로인하여임상적으로는악성의성질을보인다 [1]. 유건종은발생부위및유전질환동반여부에따라복강외종양, 복강내종양, 다발성종양, 가족성-다발성종양, 가드너씨증후군의 5가지로나눌수있다 [2]. 특히, 장간막에서발생한유건종은주위조직으로침윤이나종괴로인한압박에의해통증, 장폐쇄, 요로폐쇄및누공을형성 Received: August 23, 2011, Accepted: October 28, 2011 Corresponding author: Ryung-Ah Lee, Department of Surgery, Ewha Womans University School of Medicine, 1071 Anyangcheon-ro, Yangcheon-gu, Seoul 158-710, Korea Tel: 82-2-2650-2659, Fax: 82-2-2644-7984 E-mail: ralee@ewha.ac.kr 하며, 절제가불완전한경우 70% 에달하는재발률을보이기때문에예후가매우불량하다 [3,4]. 우리는뚜렷한유전질환이없는 65세여성에서소장의장간막에서기원한복강내유건종을경험하여문헌고찰과함께보고하는바이다. 증례 65세여자환자가 2달전부터우하복부에촉지되는무통성의종괴로우측난소종양의심하에본원산부인과에내원하였다. 환자는 35년전난관결찰술을시행한것외에는특이병력이없었고특이한가족력및유전성증후군을의심할만한소견도보이지않았다. 내원시환자는병색을보이지않고활력징후도안정되어있었으나신체검사에서복부에압통없는 8.0 cm의종물이촉진되었고이어시행한질식초음파에서우측난소주변으로 7.6 cm 크기의종괴가관찰되 49
Ewha Med J Vol. 35, No. 1, 2012 Fig. 1. Abdominal CT findings. 10 6.5 cm sized solid mass is visible in right lower quadrant. The tumor originates from small bowel mesentery. (A) Horizontal view. (B) Coronal view. Fig. 2. Gross pathologic finding. 9.5 9 5 cm sized well demarcated mass is identified in the small bowel mesentery. Retraction diverticulum is visible on ileal mucosa. 근육층을침범하고있었고균일한핵을가진방추형의섬유모세포들이미만성으로관찰되었으며섬유모세포사이에는교원질이풍부하게관찰되었다 (Fig. 3). 또한종괴에서비정형유사분열은관찰되지않았고회장절단면에종괴의침윤은없었다. 면역조직화학염색에서는 PAS, CD34, S-100, HMB-45, CD99에음성을보였고 MIB-1, c-kit, actin, desmin에약양성을보였으며 β-catenin에는핵과세포질모두양성을보였다 (Fig. 4). 이상과같은육안병리및현미경조직검사, 면역조직화학염색결과에따라이종괴는유건종으로확진될수있었다. 고찰 어추가적인검사및수술을위해입원하였다. 입원하여시행한혈액검사에서는특이소견을보이지않았으나복부전산화단층촬영에서 10.0 cm 크기의회장장간막에서기원한것으로추정되는종괴가관찰되었고유건종또는악성평활근육종이의심되었다 (Fig. 1). 양측난소및자궁은정상소견을보였다. 환자는수술을위해외과로전과되었고복강경탐색술이시행되었다. 수술소견에서 10 cm의타원형의딱딱한종괴가회맹판막에서약 15 cm 떨어진부분의회장의직하부장간막에서관찰되었고주변장기로의침습은보이지않았다. 수술은복강경을이용하여약 40 cm 의회장을장간막과함께절제하였다. 육안병리검사에서 9.5 9 5 cm의경계가확실한구형의종괴가회장장간막에서관찰되었고회장의점막에는종괴에의한견인게실 (retraction diverticulum) 이보였다. 종괴의절제면은점액성이포함된어두운노란색을띄고있었다 (Fig. 2). 현미경조직검사에서종괴는소장 유건종은 1832년 John MacFarlane이복부종물로서처음보고하였고 Muller가힘줄을의미하는그리스어인 desmos 에서유래한 desmoid 라는용어를 1838년처음사용한후주로근초나근건막구조에서유래한양성연부조직종양으로보고되어왔다 [5]. 유건종의발생장소는그기원이근골격계및간질조직인만큼신체의어느부위에도발생할수있다. 하지만주로발생하는장소는어깨와골반그리고복벽이며복강내에서는장간막에서주로발생한다 [6]. 복강내장간막에서발생한장간막유건종은연평균백만명당 2 4명으로그빈도가매우드물고모든연령에서발생가능하나, 주로 20 40대에서많이볼수있다 [7]. 장간막유건종의주된발생장소는소장장간막, 특히회장장간막으로드물게는위-간장간막, 결장간막및위-비장인대에서도발생한다 [8]. 본증례의환자역시회장장간막에발생한종괴를볼수있었다. 장간막유건종이생기는병리기전은아직밝혀지지않았으나가드너씨증후군과관련된유전자적영향, 50 THE EWHA MEDICAL JOURNAL
Kyoung Tae Noh, et al: A Case of Ileal Mesenteric Desmoid Tumor Resected by Laparoscopic Surgery Fig. 3. Micropathologic finding. (A) The tumor infiltrates into the muscle layers of small bowel (H&E stain, 40). (B) Fibroblastic spindle cells are arranged orderly in collagenous or myxoid matrix (H&E stain, 100). Fig. 4. Immunohistochemical stain for β-catenin shows nuclear and cytoplasmic positivity in tumor cells (Immunohistochemical stain, 400). 복강내수술과같은외상에의한섬유아세포의이상증식, 고에스트로겐혈증에의한호르몬의영향, 임신, 크론병등이그유발인자로알려져있다 [9]. 장간막유건종은대부분의환자가무통성복부종괴소견을보이나, 종괴가주위장기를압박할정도로커지게되면장관폐색또는요관폐색을유발하여복통, 오심, 구토, 체중감소, 변비, 뇨실금, 야뇨증등의증상을보이며, 드물게발열과급성기반응성단백의증가를보이며염증반응을일으키기도한다 [8,10,11]. 최근에국내에서복강내거대농양으로발현한유건종에대한증례가발표되기도하였다 [12]. 본증례의 환자에게서는우측하복부에무통성의종괴가촉진된것외에다른증상은보이지않았다. 장간막유건종의진단은증상, 진찰소견, 과거력및병력조사와함께대장내시경과같은가드너씨증후군에대한기초검사가요구되며영상검사소견이도움이되나확진은조직검사에의해가능하다 [6]. 이종양의진단에도움을주는영상검사에는초음파, 복부전산화단층촬영및자기공명영상등이있다. 초음파에서종괴는균질한저에코, 고형체를보이고낭포형태가없는것이특징이다. 복부전산화단층촬영은종괴의위치와성상을알수있는가장좋은방법으로콜라겐과세포질의비율에따라다양하게나타나지만근육과비슷한밀도의균질한종괴를보이고출혈, 괴사및석회화가없는섬유성병변의특징을보인다. 자기공명영상에서는 T1-강조영상에서균질한강도로나타나며 T2-강조영상에서는불균일하게높은신호를나타내는데이것은세포질이나교원질의비율이다양하기때문이다 [13]. 유건종의조직학적소견은동일한모양의잘분화된섬유아세포들과기둥모양의전형적인방추상섬유세포들사이에많은교원질이존재하는것이특징적이며염증세포도나타날수있다. 유사분열은볼수있으나부정형의유사분열은볼수없다 [14,15]. 면역조직화학염색은장간막유건종의조직학적특성및발생위치가유사한위장관기질종양, 경화성장간막염, 염증성근육섬유세포종양, 흉막외고립섬유성종양등과의감별에중요한역할을한다 [16]. Monihan 등 [17] 은장간막유건종에서 S100 THE EWHA MEDICAL JOURNAL 51
Ewha Med J Vol. 35, No. 1, 2012 단백과 CD34에음성을나타내는것이유건종임을확인하는데유용하다고하였고 Mongomery 등 [18] 은 β-catenin이종양세포의핵에양성을보이는경우장간막유건종으로진단할수있다고하였다. 본증례는면역조직화학염색에서 S100 단백및 CD34에음성이고 β-catenin염색에서양성을보여유건종임을확진할수있었다. 장간막유건종의치료방법은수술, 방사선치료, 약물및호르몬치료등이있으나최선의치료방법은침범된조직의넓은변연부를포함한광범위외과적절제술이다 [19]. 보통유건종은복강내수술과같은외상과연관되거나거대종물또는종괴로인한장관폐쇄등의증상으로발견되어복강경수술이시도된예는매우적으나본증례의경우종괴가크기에비해주변장기로의침범이없어복강경수술로완전절제되었다. 완전절제하기에크기가너무크거나생명기관과연관되어수술적절제가어려운경우방사선치료 (4,000 6,000 rad), 항암치료 (dactinomycin, vincristine, cyclophosphamide 등 ), 호르몬치료 (tamoxifen, testolactone) 를하거나그외 indomethacin이나 antiprostaglandin제제, ascorbic acid, theophilline 등을사용할수있다고보고되고있으나그효과는입증되지못했다 [14,19]. 장간막유건종은수술후국소재발률이높기때문에치료후경과관찰이필수적이다. 특히가드너씨증후군환자에서발생한경우거의 100% 에서재발을하며높은사망률을보인다. 그러나가드너씨증후군이없는경우재발은최고 60% 까지일어나며, 이들은대개불충분한종양의제거와관계있어, 만일종양을충분히제거한다면재발률을 4% 까지낮출수있다. 본증례의환자의경우종양의완전절제가이루어졌으며따라서재발없는경과를기대하고있다. 참고문헌 1. Kulaylat MN, Karakousis CP, Keaney CM, McCorvey D, Bem J, Ambrus Sr JL. Desmoid tumour: a pleomorphic lesion. Eur J Surg Oncol 1999;25:487-497. 2. Kawashima A, Goldman SM, Fishman EK, Kuhlman JE, Onitsuka H, Fukuya T, et al. CT of intraabdominal desmoid tumors: is the tumor different in patients with Gardner's disease? AJR Am J Roentgenol 1994;162: 339-342. 3. Park BH, Kim HJ, Chang YW, Kim KJ, Lee DK, Dong SH, et al. Desmoid tumor and duodenal adenoma in a patient with familial adenomatous polyposis: a case report. Korean J Gastrointest Endosc 2001;23:32-35. 4. Lee HS, Jeon HM, Ok ST, Kim JS, Lee EJ, Kim JS. Unresectable desmoid tumor developing after surgery of F.A.P case report. J Korean Soc Coloproctol 1998;14: 323-329. 5. Shiu MH, Weinstein L, Hajdu SI, Brennan MF. Malignant soft-tissue tumors of the anterior abdominal wall. Am J Surg 1989;158:446-451. 6. Venkat D, Levine E, Wise WE. Abdominal pain and colonic obstruction from an intra-abdominal desmoid tumor. Gastroenterol Hepatol (N Y) 2010;6:662-665. 7. Mecrow IK, Miller V, Lendon M, Doig CM. Mesenteric fibromatosis presenting with ascites in childhood. J Pediatr Gastroenterol Nutr 1990;11:118-122. 8. Bansal M, Shindelman LE, Geller SA, Gordon RE, Schwarz R. Mesenteric fibromatosis. Mt Sinai J Med 1983;50:527-530. 9. Lee JC, Thomas JM, Phillips S, Fisher C, Moskovic E. Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol 2006;186:247-254. 10. Klein WA, Miller HH, Anderson M, DeCosse JJ. The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis. Cancer 1987;60:2863-2868. 11. Murayama T, Imoto S, Ito M, Matsushita K, Matozaki S, Nakagawa T, et al. Mesenteric fibromatosis presenting as fever of unknown origin. Am J Gastroenterol 1992;87:1503-1505. 12. Yu YH, Son BK, Jun DW, Kim SH, Jo YJ, Park YS, et al. A case of desmoid tumor presenting as intra-abdominal abscess. Korean J Gastroenterol 2009;53:315-319. 13. Forte MD, Brant WE. Spontaneous isolated mesenteric fibromatosis: report of a case. Dis Colon Rectum 1988; 31:315-317. 14. Koppikar MG, Vaze AM, Patel MS, Phadke PP, Chitale AR, Bapat RD. Mesenteric fibromatosis. J Postgrad Med 1980;26:196-198. 15. Stout AP, Raffaele L. Tumors of the soft tissues. Washington, DC: Armed Forces Institute of Pathology; 1967. 16. Moon HH, Yang SI, Yoon KY, Jang HK, Seo KW, Lee SH, et al. Jejunal mesenteric fibromatosis. J Korean Surg Soc 2010;78:320-324. 17. Monihan JM, Carr NJ, Sobin LH. CD34 immunoexpression in stromal tumours of the gastrointestinal tract and in mesenteric fibromatoses. Histopathology 1994; 25:469-473. 18. Montgomery E, Torbenson MS, Kaushal M, Fisher C, 52 THE EWHA MEDICAL JOURNAL
Kyoung Tae Noh, et al: A Case of Ileal Mesenteric Desmoid Tumor Resected by Laparoscopic Surgery Abraham SC. Beta-catenin immunohistochemistry separates mesenteric fibromatosis from gastrointestinal stro al tumor and sclerosing mesenteritis. Am J Surg Pathol 2002;26:1296-1301. 19. Nam KH, Kweon BC, Lee HK, Lee DW, Woo CK, Park JS, et al. A case of mesenteric fibromatosis after appendectomy. Korean J Med 1998;54:577-581. THE EWHA MEDICAL JOURNAL 53