Korean Journal of Clinical Oncology 2016;12:119-123 https://doi.org/10.14216/kjco.16020 pissn 1738-8082 eissn 2288-4084 Original Article 충수돌기에서기인한고등급복막가성점액종의반복적인용적축소수술의임상적고찰 박정현 1, 송인호 1, 이동운 1, 권윤혜 1, 김정기 2, 문상희 1, 박지원 1,3, 유승범 1,3, 정승용 1,3, 박규주 1,3 1 서울대학교의과대학서울대병원외과학교실, 2 국립중앙의료원소화기센터외과, 3 서울대학교암병원대장암센터 Surgical treatment for high-grade pseudomyxoma peritonei originated from appendix: Analysis of clinical outcomes of repeated debulking surgery Jung Hyun Park 1, Inho Song 1, Dong Woon Lee 1, Yoon-Hye Kwon 1, Jeong-Ki Kim 2, Sang Hui Moon 1, Ji Won Park 1, Seung-Bum Ryoo 1, Seung-Yong Jeong 1,3, Kyu Joo Park 1,3 1 Department of Surgery, Seoul National University College of Medicine, Seoul; 2 Department of Surgery, National Medical Center, Seoul; 3 Colorectal Cancer Center, Seoul National University Cancer Hospital, Seoul, Korea Purpose: To evaluate the effect of repeated debulking surgery for high-grade pseudomyxoma peritonei (PMP) originating from the appendix. Methods: Between January 1998 and December 2014, fifty patients, who underwent debulking surgery for high-grade PMP originating from the appendix, were obtained from a prospectively collected database and retrospectively analyzed. Two groups according to the number of operations were divided and analyzed. Results: A total of 118 operations were performed. Thirty-one patients received more than two operations. The median interval between operations was 18.2 months (range, 2 170 months). Complications developed after 26 operations (22.0%), including ileus (n=10), intra-abdominal fluid collection (n=7), surgical site infection (n=5), and others. There were two mortalities within 30 days after operation. Between two groups of patients who received one operation only and patients who received more than two operations, transfusion, diversion operation, and postoperative complication rate showed statistically significant differences. Two groups of patients had no differences in overall survival rates. Conclusion: Our results indicate that the number of operations does not affect the survival rate of high-grade appendiceal PMP, in which repeated debulking surgery is vital to relieve symptoms of the tumor burden. Keywords: Pseudomyxoma peritonei, Cytoreduction surgical procedures, Treatment, Recurrence 서론 복막가성점액종 (pseudomyxoma peritonei, PMP) 은복강내점액성종양이침착되는질환으로 [1], 연간발생률이백만명당 1 2 명인매 Received: Oct 12, 2016 Accepted: Nov 16, 2016 Correspondence to: Kyu Joo Park Department of Surgery, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea Tel: +82-2-2072-2901, Fax: +82-2-7966-3975 E-mail: kjparkmd@plaza.snu.ac.kr Copyright Korean Society of Surgical Oncology This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 우드문질환이다 [2]. 질병의원인은대부분충수돌기에서유래하는것으로알려져있으며 [3,4], 치료는보존적치료 [5] 부터종양의제거후전신항암치료 [6,7] 까지다양한방법이시도되어왔으나생존율의향상은명확하지않다. 근래에 Sugarbaker 에의해세포감퇴수술 (cytoreductive surgery, CRS) 과동시에복강내온열항암화학요법 (hyperthermic intraperitoneal chemotherapy, HIPEC) 이소개된이후그결과가생존율상승에효과적이라고보고된바있다 [8]. 그러나고등급복막가성점액종으로국한하여살펴보면종양의광범위한주변장기로의침투로잔여종양을 2.5 mm 이내로제거가불가능한경우에해당술식의적용은제한이있다 [9]. 결국진행된고등급복막가성점액종환자에서는반복적용적축소수술이생존율의증가를기대할수있는치료법이될수있으나이에대한수술후임상적예후에대한근거는빈약하다 [10]. www.kjco.org 119
따라서이연구는충수돌기에서기인한고등급복막가성점액종환자의반복적용적축소수술에영향을미치는인자에대한분석및용적축소수술에따른생존율을도출함으로써반복적용적축소수술의임상적의미에대해평가하고자한다. 방법 1998 년 1 월부터 2014 년 12 월까지충수돌기에서기인한고등급복막가성점액종으로수술을받은환자 50 명을대상으로전향적으로모집된의무기록을후향적분석하였다. 본연구는임상시험계획심사위원회 (Institutional Review Board) 승인을받았다 ( 승인번호 : 1610-037-797). 연구를위해수집한인자는다음과같다. 첫번째수술시행당시연령, 성별, 첫번째수술당시체질량지수 (body mass index), 수술전혈액내종양표지자수치 ( 암종배아항원 : carcinoembryonic antigen [CEA]; carbohydrate antigen 19-9 [CA 19-9]), 첫번째수술의방법, 수술횟수, 첫수술당시의복막종양지수 (peritoneal carcinomatosis index, PCI), 수술시간, 수술중실혈량, 수혈여부, 병리조직학적등급, 수술후합병증, 수술후사망, 수술후전신항암화학요법의유무, 수술후마지막관찰기간, 사망여부등을전자의무기록을통해수집하였다. 본연구에속한환자들은최소한번이상본원에서수술적치료를받았으며, 모든수술에서원발부위의절제혹은모든종양의절제가불가능한경우최대한의용적축소수술을시행하였다. 첫번째수술의목적은암종의완벽한절제혹은이미완벽한절제가불가능할경우에는장기의기능보존과복부팽만, 호흡곤란등의복강내종양의크기효과에따른증상을완화시키기위한것이었다. 소장간막을침윤한복막가성점액종에대하여국소적일경우소장절제및문합을시행하였고, 이미광범위하게퍼져있어종양제거의의의가없을경우에는장관연결성유지에초점을맞추어점액성종양을최대한제거하는수술을시행하였다. 기본적으로넓게퍼져있는복막가성점액종에대해복막절제술을시행하지않았으나, 국소적으로복막에종양이있는경우에는그부분의복막만절제하는것을원칙으로하였다. 수술후경과관찰중종양의부피증가로인하여호흡곤란, 일상생활이불가능할정도의복부팽만이나타나거나암종의소장및대장의침투로기계적장폐색등의증상이발생하는경우재수술을진행하였다. 수술후합병증은수술일 30 일이내발생한것으로정의하였으며장마비는수술후 5 일이후까지구역, 구토등의증상의발현으로보존적인치료를시행하거나혹은식이진행이되지않으면서영상학적검사에서장마비에부합한소견을보이는경우로정의하였다. 창상합병증에는창상의재봉합술을시행한경우도포함하였으며그외복강내농양또는체액축적등이조사되었다. 수술후사망은수술후같은입원기간내의사망을모두포함하였다. 종양의부피와분포정도는수술시소견에따라기록된복막종양지수로측정되었다 [11]. 수술을한번만받은환자와두번이상받은환자를두군으로구분하여위의인자들과연관성의차이에대한분석을시행하였다. 수술의횟수에따라나눈두군의비교는비연속변수의경우카 이제곱검정으로분석하였고연속변수중정규분포를따를경우독립 T 검정을정규분포를따르지않을경우비모수검정인 Mann-Whitney 검정을하였다. 두군의전체생존율은카플란 - 마이어생존분석을이용하여산출하였으며 P- 값은로그 - 랭크분석을이용하였다. 통계학적분석을위해 SPSS ver. 21.0 (IBM Co., Somers, NY, USA) 을사용하였으며, P 값이 0.05 미만인경우통계학적으로유의하다고정의하였다. 결과 임상적특성 환자들의임상병리학적특성은 Table 1 에나타내었다. 평균추적기간은 64.0 개월 ( 범위, 6.8 213.6 개월 ) 이었고, 평균연령은 57.5 세였다. 성별로는여자 33 명, 남자 17 명으로여자에서높은빈도를보였다. 한번의수술만받은환자는 19 명이었으며두번이상의수술을받은환자는 31 명이었다. 수술사이기간의중앙값은 18.2 개월 ( 범위, Table 1. Clinical characteristics of high-grade appendiceal PMP patients (n=50) Characteristics Mean or no. Age (yr) 57.5± 11.7 Sex (female) 33 (66.0) Body mass index (kg/m 2 ) 21.0± 3.4 Preoperative tumor marker CEA (ng/ml) 17.8 (1.3 403.0) CA 19-9 (U/mL) 52.0 (5.0 5,070.0) First operation Right hemicolectomy 32 (64.0) Total colectomy 6 (12.0) Omentectomy 5 (10.0) TAH with BSO 7 (14.0) No. of operations 1 19 (38.0) 2 12 (24.0) 3 8 (16.0) 4 4 (8.0) 5 7 (14.0) Diversion operation Ileostomy 17 (34.0) Colostomy 4 (8.0) PCI 27.0 (6.0 36.0) Operation time (min) 155 (45 330) Intraoperative blood loss (ml) 535 (50 4,200) Transfusion 27 (54.0) Systemic chemotherapy 44 (88.0) Complications 15 (30.0) Values are presented as mean± standard deviation or absolute number (%). PMP, pseudomyxoma peritonei; CEA, carcinoembryonic antigen; TAH, total abdominal hysterectomy; BSO, bilateral salpingo-oophorectomy; PCI, peritoneal carcinomatosis index. 120 Korean Journal of Clinical Oncology
Jung Hyun Park et al. Repeated debulking surgery for high-grade appendiceal pseudomyxoma peritonei 2 170 개월 ) 로측정되었으며첫번째수술과두번째수술사이기간은 31.8 개월, 두번째수술과세번째수술사이기간은 21.0 개월, 세번째수술과네번째수술사이기간은 13.2 개월로, 수술사이기간은수술횟수가누적될수록짧아지는경향을보였다. 첫번째수술은우측결장절제술 (32 예, 64.0%) 을가장흔히받았으며, 전결장절제술, 자궁및양측난소절제술, 대망절제술순서로시행되었다. 장루형성술은총 21 예 (42.0%) 에서시행되었으며, 회장루가 17 예 (34.0%), 결장루가 4 예 (8.0%) 로분포하였다. PCI 의중간값은 27.0 ( 범위, 6.0 36.0) 으로측정되었다. 수술시간의중간값은 155 분 ( 범위, 45 330 분 ) 이었으며, 수술중실혈량의중간값은 535 ml ( 범위, 50 4,200 ml) 이었고, 27 예 (54.0%) 에서수혈이시행되었다. 전신항암화학요법은모두 44 예 (88.0%) 에서시행되었다. 전신항암요법은대부분플루오로피리미딘기반의항암치료를시행했으며 (41 예, 93.2%) 용법은다음과같았다. 플루오로피리미딘단독투약이 8 예, 옥살리플라틴과혼합하여투약이 16 예, 시스플라틴과혼합하여투약이 14 예, 이리노테칸과혼합하여투약이 2 예가있었다. 6 명의환자에서전신항암화학요법이시행되지않았는데, 이는환자의거부또는전신적건강상태의저하등의이유로전신항암화학요법이제한된다고판단한경우였다. 본연구에포함된모든환자에서복강내항암화학요법은시행되지않았다. 수술후이환율과사망률 총 118 건의수술중 26 예 ( 환자수 = 15, 22.0%) 에서수술후합병증이발생하였으며, 장마비가 10 예, 복강내체액축적이 7 예, 수술부위감염이 5 예, 문합부누출이 4 예, 폐렴이 2 예등이었다. 수술부위감염 5 예중 2 명의환자에서수술부위재봉합을시행하였다. 문합부누출 4 예의경우, 모두복강내체액축적이동반되었다. 5 명의환자는복강내체액축적과장마비가동시에발생하였으며, 2 명의환자는폐렴과장마비가동시에발생하였다. 복강내체액축적과수술부위감염, 장마비와수술부위감염이동시에발생한환자가각 1 명씩있었으며, 흉수, 복강내체액축적및장마비가동시에발생한환자가 1 명있었다. Table 2 에합병증에대해정리하였다. 수술후사 Table 2. Postoperative morbidity after all operations (n=118 a) ) Morbidity (n= 19) 2 Operations (n= 99) P-value Total 2 (10.5) 24 (24.2 b) ) 0.239 Postoperative ileus 1 (5.3) 9 (9.1) Intra-abdominal fluid collection 0 7 (7.1) Anastomosis leakage 0 4 c) (4.0) Surgical site infection 1 (5.3) 4 (4.0) Pneumonia 0 2 (2.0) Pleural effusion 0 1 (1.0) Postoperative peritonitis 0 1 (1.0) Data are expressed as absolute number (%) a) Number of all operations. b) Number of complications divided by number of total operations of the group. c) Four anastomosis leakages occurred with intra-abdominal fluid collection. 망률은 1.7% (2 예 ) 로나타났으며. 모두급성호흡곤란증후군으로사망하였다. 첫번째와다섯번째수술후폐렴이발생한후급성호흡곤란증후군으로진행되어사망하였으며수술후 117 일과 26 일째사망하였다. 수술횟수에따른두군의비교 수술을한번받은환자들과두번이상받은환자들을두군으로구분하여다른인자와의관계에대하여 Table 3 에정리하였다. 나이, 성별, 수술전종양표지자 (CEA, CA19-9), PCI, 수술시간, 수술중실혈량, 전신항암치료의여부, 대장절제유무는재수술에연관성을보이지않았다. 두군에서수혈여부 (OR, 9.74; P= 0.002), 장루형성유무 (OR, 7.69; P = 0.043) 와수술후합병증유무 (OR, 6.14; P = 0.026) 가유의한차이를보였다. 수술을한번받은환자들과두번이상받은환자들간의전체생존율은차이가없었다. 수술을한번받은환자군의 5 년생존율은 49.3%, 10 년생존율은 39.4% 였고, 두번이상받은환자군의 5 년생존율은 56.4%, 10 년생존율은 22.6% 였다 (Log rank P= 0.946) (Fig. 1). 고찰 본연구에서는충수돌기에서기인한고등급복막가성점액종환자에서수술후임상결과를분석하였다. 평균환자의나이는 57.5 ± 11.7 세이고상대적으로여성에서많이발생하였다. PCI 의중간값은 27.0 (6.0 36.0) 이며, 두번이상수술을시행한환자는 31 명 (62%) 이 Table 3. Clinical characteristics of PMP patients according to number of operations Characteristics (n= 19) 2 Operations (n= 31) P-value Age (yr) 58.9± 10.9 56.6± 12.3 0.623 Sex (female) 12 (63.2) 21 (67.7) 0.370 BMI (kg/m 2 ) 21.4± 2.6 20.8± 3.7 0.355 Preoperative tumor marker a) CEA 5 (ng/ml) 11 (61.1) 23 (74.2) 0.356 CA 19-9 37 (U/mL) 7 (46.7) 15 (62.5) 0.481 PCI 27.0 (6.0 36.0) 27.0 (6.0 36.0) 0.423 Operative time (min) 120 (60 330) 170 (45 330) 0.114 Intraoperative blood loss (ml) 400 (50 4,200) 650 (50 2,200) 0.400 Transfusion 5 (26.3) 22 (71.0) 0.002 Systemic chemotherapy 15 (78.9) 29 (93.5) 0.123 Stoma formation 4 (21.1) 17 (54.8) 0.043 Colon resection 16 (84.2) 22 (71.0) 0.332 Complication b) 2 (10.5) 13 (41.9) 0.026 Data are expressed as mean ± SD or absolute number (%). PMP, pseudomyxoma peritonei; BMI, body mass index; PCI, peritoneal carcinomatosis index; CEA, carcinoembryonic antigen. a) CEA, ng/ml; CA 19-9, U/mL. b) Complication only included events after first operation. www.kjco.org 121
1.0 0.8 0.6 0.4 0.2 0 5-year OS: 56.4% 5-year OS: 49.3% 0 50 100 150 200 250 Time (mo) Number of operations 2 Operation P = 0.946 Fig. 1. Overall survival of patients with high-grade PMP from appendiceal origin. PMP, pseudomyxoma peritonei; OS, overall survival. 었다. 수술후합병증은 26 예 (22.0%) 에서나타났다. 수술을한번시행한군과두번이상시행한군을비교분석하였을때재수술과관련된인자는수혈, 장루형성여부, 합병증발생여부가있었다. 생존분석결과, 두군의전체생존율의차이는보이지않았다. 기존연구들에의하면재수술에영향을미치는인자는잔여종양의유무, 합병증발생여부가보고된바있다 [12,13]. 본연구에서수술횟수에따라재수술을받은환자들과받지않은환자들로나누어분석을시행한결과수술후합병증발생유무가재수술과연관성이있다고분석되었다. 이는반복된수술로합병증이누적되어유의하게분석된결과로고려된다. 수혈및장루형성여부가재수술과통계적으로관련이있다고분석된것은이인자들이재수술에영향을미치는것이아니라수술이반복됨에따라수술의난이도가상승하여수혈의필요성과장루형성빈도가높아지는것을의미한다. 그외일반적으로종양의예후와관련이있다고알려진 CEA, CA 19-9 [14], PCI [15], 그리고전신항암요법 [6,16] 여부는재수술과관련성을보이지않았다. 이는본연구에서포함된모든환자들은이미 1 차혹은 2 차병원에서충수돌기와관련된수술을시행하고점액종의광범위한복막전이가존재한상태로본병원에방문하였거나첫방문에서부터주변으로의복막전이가존재한상태로용적축소수술을받았기때문에특이성이떨어지는것으로판단된다. 그러나잔여종양의유무그리고첫수술시복강내상태에대한정확한정보는 PCI 를제외한기록을의무기록에서수집할수없어추가적인분석은제한되었다. 고등급과저등급복막가성점액종의특성에대해보고된바에의하면고등급복막가성점액종의경우림프의전이가많으나장기침범의경우저등급복막가성점액종과비교할때통계학적차이가없었다 [17]. 결국충수돌기에서기인한고등급복막가성점액종의경우림프절절제술이예후에영향을미칠가능성을예측할수있다. 본연구에서분석한결과는대장절제와동시에림프절절제를구획으로시행한환자들과단순대망절제술혹은자궁및양측난소절제술을시행한환자들간의재수술에관하여연관성을보이지않았다 (P = 0.332). 세포감퇴수술의정의는 2.5 mm 이상의종양을모두제거하는것을목적으로하며 [9], 용적축소수술은종양및추가적인절제를통해장관연결성을확보하고최대한종양을제거하여종양의양적효과를감소시켜전체적인종양의부담을줄이는것이목표인수술방법으로두가지수술의목적이다르다고볼수있다. 최근들어복막가성점액종에서 CRS 와 HIPEC 이이상적인치료법으로대두되었지만, 근치적절제로인한높은합병증발생률 [6], 복강내직접적인항암제주입으로인해발생할수있는심각한문제점 [18] 등아직까지적용하기에어려운점이많다. 진행된복막가성점액종환자에서 CRS 를할수없을경우, 반복적용적축소수술이중요한치료방법이될수있다. 반복적용적축소수술을시행한선행연구결과, 5 년생존율은 15% 에서 65% 로다양하였고, 질환의병리조직학적등급에따라 5 년전체생존율의유의한차이가있었다 [16]. 생존율에영향을미치는인자에는수술전종양표지자, 종양등급도등이제시되었다 [19]. 본연구에서반복적용적축소수술의횟수에따른전체생존율비교분석을하였으나그차이는보이지않았다. 수술을한번받은환자군의 5 년생존율은 49.3%, 10 년생존율은 39.4% 였고, 두번이상받은환자군의 5 년생존율은 56.4%, 10 년생존율은 22.6% 였다 (P = 0.946). 하지만, 본연구에포함된환자들이복막가성점액종이진행되어전신항암치료가효과를보이지않고 [20] 재수술이불가피한상황에서수술했다는점을고려할때, 반복적용적축소수술이시행된군이한번수술받은환자군과동일한생존기간을보장한다는점에서종양의부피증가로인한호흡곤란, 일상생활이불가능할정도의복부팽만, 그리고종양의장관침투로인한기계적장폐색등의수술적적응증을보이는환자에서반복적이고적극적인수술적제거가안전하고유용한치료의한방안임을제시한다. 고등급복막가성점액종의경우기존연구에서보고된합병증은출혈, 복강내농양, 상처감염, 장마비, 요도감염등이알려져있다 [21]. 본연구에서도총합병증은 22.0% 로관찰되었다. 각환자에서수술횟수가증가할수록주로복강내체액축적, 문합부누출, 수술후복막염, 폐렴등이증가하는추세였다. 반복된용적축소수술에따른심한유착으로수술시제한이있었으나, 종양의특성상점액성물질은다른암종수술에비하여상대적으로유착정도가덜하였으며전문적술기에능한한명의집도의에의해모든수술이진행되었다. 모든의사에게적용될수는없는결과인점이제한점이지만, 복막가성점액종의특성상적극적인수술이중요하다는점에서의의가있다. 한번수술한군과두번이상수술한군으로나누어비교하였을경우, 두번이상수술한환자군에서합병증발생률이유의하게높았지만 (OR, 6.14; P= 0.026), 합병증의유무는생존율에영향을미치지않았다 (P = 0.946). 이는수술이반복됨에따라합병증이누적되어통계적으로유의하게분석된것으로판단할수있으며, 적극적인수술전후환자관리로단기간내합병증을극복하면장기간생존율의향상도기대해볼수있을것이다. 122 Korean Journal of Clinical Oncology
Jung Hyun Park et al. Repeated debulking surgery for high-grade appendiceal pseudomyxoma peritonei 본연구의장점은충수돌기에서기인한고등급복막가성점액종만을대상으로하여그임상적경과에대해자세히알수있으며, 해당질환의반복적인용적축소수술의예후에대해명확히제시하고있다. 제한점으로첫째, 후향적연구이다. 이와관련된자료의미비및수집관련오류가존재한다. 둘째, 분석된환자의수가적어질환의실질적인임상경과를대변한다고볼수없다. 마지막으로 3 차병원에서시행한연구로고도로진행된환자가편중되어일반적인의료현장을대변하지못하는오류의발생이가능하다. 결론적으로충수돌기에서기인한고등급복막가성점액종의반복적인수술횟수는수혈, 장루형성여부및합병증발생여부와관련되었으나, 수술을반복적으로시행하는군과수술을한번만시행한군의생존율의차이는보이지않았다. 이는생명을위협하는증상이발생할경우적극적인반복적용적축소수술을시행하는것이치료의중요한방법임을제시한다. CONFLICT OF INTEREST No potential conflict of interest relevant to this article was reported. REFERENCES 1. Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol 2008;34:196-201. 2. Sugarbaker PH. Pseudomyxoma peritonei: a cancer whose biology is characterized by a redistribution phenomenon. Ann Surg 1994; 219:109-11. 3. Nakakura EK. Pseudomyxoma peritonei: more questions than answers. J Clin Oncol 2012;30:2429-30. 4. Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, et al. Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Clin Oncol 2012;30:2449-56. 5. Friedland JS, Allardice JT, Wyatt AP. Pseudomyxoma peritonei. J R Soc Med 1986;79:480-2. 6. Gough DB, Donohue JH, Schutt AJ, Gonchoroff N, Goellner JR, Wilson TO, et al. Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach. Ann Surg 1994;219: 112-9. 7. Mann WJ Jr, Wagner J, Chumas J, Chalas E. The management of pseudomyxoma peritonei. Cancer 1990;66:1636-40. 8. Esquivel J, Sugarbaker PH. Clinical presentation of the Pseudomyxoma peritonei syndrome. Br J Surg 2000;87:1414-8. 9. Sugarbaker PH. Cytoreductive surgery and perioperative intraperitoneal chemotherapy: a new standard of care for appendiceal mucinous tumors with peritoneal dissemination. Clin Colon Rectal Surg 2005;18:204-14. 10. Moran BJ. Commentary on Survival of patients with pseudomyxoma peritonei treated by serial debulking. Colorectal Dis 2010; 12:872-3. 11. Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res 1996;82:359-74. 12. Jarvinen P, Jarvinen HJ, Lepisto A. Survival of patients with pseudomyxoma peritonei treated by serial debulking. Colorectal Dis 2010;12:868-72. 13. Andreasson H, Graf W, Nygren P, Glimelius B, Mahteme H. Outcome differences between debulking surgery and cytoreductive surgery in patients with Pseudomyxoma peritonei. Eur J Surg Oncol 2012;38:962-8. 14. Carmignani CP, Hampton R, Sugarbaker CE, Chang D, Sugarbaker PH. Utility of CEA and CA 19-9 tumor markers in diagnosis and prognostic assessment of mucinous epithelial cancers of the appendix. J Surg Oncol 2004;87:162-6. 15. Chua TC, Al-Zahrani A, Saxena A, Glenn D, Liauw W, Zhao J, et al. Determining the association between preoperative computed tomography findings and postoperative outcomes after cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol 2011;18:1582-9. 16. Miner TJ, Shia J, Jaques DP, Klimstra DS, Brennan MF, Coit DG. Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy. Ann Surg 2005;241:300-8. 17. Carr NJ, Finch J, Ilesley IC, Chandrakumaran K, Mohamed F, Mirnezami A, et al. Pathology and prognosis in pseudomyxoma peritonei: a review of 274 cases. J Clin Pathol 2012;65:919-23. 18. Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FA. Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg 2007;245:104-9. 19. Elias D, Honore C, Ciuchendea R, Billard V, Raynard B, Lo Dico R, et al. Peritoneal pseudomyxoma: results of a systematic policy of complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Br J Surg 2008;95:1164-71. 20. Rubino MS, Abdel-Misih RZ, Bennett JJ, Petrelli NJ. Peritoneal surface malignancies and regional treatment: a review of the literature. Surg Oncol 2012;21:87-94. 21. Dayal S, Taflampas P, Riss S, Chandrakumaran K, Cecil TD, Mohamed F, et al. Complete cytoreduction for pseudomyxoma peritonei is optimal but maximal tumor debulking may be beneficial in patients in whom complete tumor removal cannot be achieved. Dis Colon Rectum 2013;56:1366-72. www.kjco.org 123