Purpose
Epithelial appendiceal tumors are uncommon but can present as an emergency simulating appendicitis, or unexpectedly at laparotomy, laparoscopy, or on cross-sectional imaging. Occult rupture with features of pseudomyxoma peritonei may be encountered. We report the operative findings, pathologic assessment, and early outcomes in 123 consecutive patients with a perforated appendiceal neoplasm presenting as pseudomyxoma peritonei.
Methods
From March 1994 to March 2004, 292 patients were referred to a peritoneal malignancy surgical treatment center. Complete tumor removal (cytoreduction) was attempted in selected patients and, if achieved, surgery was combined with intraoperative, intraperitoneal mitomycin C (10 mg/m2).
Results
In total, 123 patients (52 males; 41 percent) underwent laparotomy for a perforated appendiceal malignancy presenting as pseudomyxoma peritonei. The median age was 52 (range 30–77) years. Complete cytoreduction was achieved in 83 of 123 patients (67 percent), major palliative resection in 34 patients (28 percent), and 6 patients (5 percent) were inoperable. Postoperative mortality was 6 of 123 patients (5 percent). Kaplan-Meier analysis of the 83 patients who had complete tumor removal predicted 75 percent disease-free survival at five years.
Conclusions
A perforated appendiceal epithelial tumor most frequently presents as pseudomyxoma peritonei. This treatment strategy, involving surgery and intraperitoneal chemotherapy, can result in good outcomes in this rare and otherwise fatal disease.
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References
GT Deans RA Spence (1995) ArticleTitleNeoplastic lesions of the appendix Br J Surg 82 299–306 Occurrence Handle7795991 Occurrence Handle1:STN:280:DyaK2Mzhs1eluw%3D%3D
DD Esmer-Sanches JL Martinez-Ordaz P Roman-Zepeda P Sanchez-Fernandez E Medina-Gonzalez (2004) ArticleTitleAppendiceal tumors. Clinicopathologic review of 5,307 appendectomies Cir Cir 72 375–378
SJ Connor GB Hanna FA Frizelle (1998) ArticleTitleAppendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies Dis Colon Rectum 41 75–80 Occurrence Handle9510314 Occurrence Handle10.1007/BF02236899 Occurrence Handle1:STN:280:DyaK1c7mvFWgtg%3D%3D
R Werth (1884) ArticleTitleKlinische and Anastomische Untersuchungen Zur Lehre von der Bauchgeschwullsten und der laparotomy Arch Gynecol Obstet 84 100–118
BJ Moran TD Cecil (2003) ArticleTitleThe etiology, clinical presentation, and management of pseudomyxoma peritonei Surg Oncol Clin North Am 12 585–603 Occurrence Handle10.1016/S1055-3207(03)00026-7
NJ Carr LH Sobin (1995) ArticleTitleEpithelial noncarcinoid tumors and tumor-like lesions of the appendix Cancer 76 2383–2384 Occurrence Handle8635047 Occurrence Handle10.1002/1097-0142(19951201)76:11<2383::AID-CNCR2820761130>3.0.CO;2-6 Occurrence Handle1:STN:280:DyaK283itlaqtA%3D%3D
BM Ronnett CM Zahn RJ Kurman ME Kass PH Sugarbaker BJ Shmookler (1995) ArticleTitleDisseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to “pseudomyxoma peritonei Am J Surg Pathol 19 1390–1408 Occurrence Handle7503361 Occurrence Handle1:STN:280:DyaK28%2FotlKktQ%3D%3D Occurrence Handle10.1097/00000478-199512000-00006
J Misdraji RK Yantiss FM Graeme-Cook UJ Balis RH Young (2003) ArticleTitleAppendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases Am J Surg Pathol 27 1089–1103 Occurrence Handle12883241 Occurrence Handle10.1097/00000478-200308000-00006
J Misdraji RH Young (2004) ArticleTitlePrimary epithelial neoplasms and other epithelial lesions of the appendix (excluding carcinoid tumours) Semin Diagn Pathol 21 120–133 Occurrence Handle15807472 Occurrence Handle10.1053/j.semdp.2004.11.005
PH Sugarbaker BM Ronnett A Archer et al. (1996) ArticleTitlePseudomyxoma peritonei syndrome Adv Surg 30 233–280 Occurrence Handle8960339 Occurrence Handle1:STN:280:DyaK2s7hvFGitA%3D%3D
J Esquivel PH Sugarbaker (2000) ArticleTitleClinical presentation of the pseudomyxoma peritonei syndrome Br J Surg 87 1414–1418 Occurrence Handle11044169 Occurrence Handle10.1046/j.1365-2168.2000.01553.x Occurrence Handle1:STN:280:DC%2BD3crgtVGktQ%3D%3D
F Consorti A Lorenzotti G Midiri M Paola ParticleDi (2000) ArticleTitlePrognostic significance of mucinous carcinoma of colon and rectum: a prospective case-control study J Surg Oncol 73 70–74 Occurrence Handle10694641 Occurrence Handle10.1002/(SICI)1096-9098(200002)73:2<70::AID-JSO3>3.0.CO;2-J Occurrence Handle1:STN:280:DC%2BD3c7mtVOkuw%3D%3D
H Ito RT Osteen R Bleday MJ Zinner SW Ashley EE Whang (2004) ArticleTitleAppendiceal adenocarcinoma: long-term outcomes after surgical therapy Dis Colon Rectum 47 474–480 Occurrence Handle14978617 Occurrence Handle10.1007/s10350-003-0077-7
VN Papadopoulos A Michalopoulos A Netta et al. (2004) ArticleTitlePrognostic significance of mucinous component in colorectal carcinoma Tech Coloproct 8 IssueIDSuppl 1 S123–S125 Occurrence Handle10.1007/s10151-004-0131-z
DB Gough JH Donohue AJ Schutt et al. (1994) ArticleTitlePseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach Am Surg 219 112–119 Occurrence Handle1:STN:280:DyaK2c7nsFKgsw%3D%3D
TJ Miner J Shia DP Jaques DS Klimstra MF Brennan DG Coit (2005) ArticleTitleLong-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy Ann Surg 241 300–308 Occurrence Handle15650641 Occurrence Handle10.1097/01.sla.0000152015.76731.1f
PH Sugarbaker BW Zhu GB Sese B Shmookler (1993) ArticleTitlePeritoneal carcinomatosis from appendiceal cancer: results in 69 patients treated by cytoreductive surgery and intraperitoneal chemotherapy Dis Colon Rectum 36 323–329 Occurrence Handle8458256 Occurrence Handle10.1007/BF02053933 Occurrence Handle1:STN:280:DyaK3s3gvFSrsQ%3D%3D
PH Sugarbaker D Chang (1999) ArticleTitleResults of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy Ann Surg Oncol 6 727–731 Occurrence Handle10622499 Occurrence Handle10.1007/s10434-999-0727-7 Occurrence Handle1:STN:280:DC%2BD3c%2Fos1aisw%3D%3D
AJ Witkamp E Bree Particlede MM Kaag et al. (2001) ArticleTitleExtensive cytoreductive surgery followed by intra-operative hyperthermic intraperitoneal chemotherapy with mitomycin-C in patients with peritoneal carcinomatosis of colorectal origin Eur J Cancer 37 979–984 Occurrence Handle11334722 Occurrence Handle10.1016/S0959-8049(01)00058-2 Occurrence Handle1:CAS:528:DC%2BD3MXjtFCns7k%3D
M Deraco D Baratti MG Inglese et al. (2004) ArticleTitlePeritonectomy and intraperitoneal hyperthermic perfusion (IPHP): a strategy that has confirmed its efficacy in patients with pseudomyxoma peritonei Ann Surg Oncol 11 393–398 Occurrence Handle15070599 Occurrence Handle10.1245/ASO.2004.07.002
P Shen J Hawksworth J Lovato et al. (2004) ArticleTitleCytoreductive surgery and intraperitoneal hyperthermic chemotherapy with mitomycin C for peritoneal carcinomatosis from nonappendiceal colorectal carcinoma Ann Surg Oncol 11 178–186 Occurrence Handle14761921 Occurrence Handle10.1245/ASO.2004.05.009
Z Guner U Schmidt MH Dahlke HJ Schlitt J Klempnauer P Piso (2005) ArticleTitleCytoreductive surgery and intraperitoneal chemotherapy for pseudomyxoma peritonei Int J Colorectal Dis 20 155–160 Occurrence Handle15503065 Occurrence Handle10.1007/s00384-004-0648-7 Occurrence Handle1:STN:280:DC%2BD2M%2FlvVKitg%3D%3D
BJ Moran A Mukherjee R Sexton (2006) ArticleTitleOperability and early outcome in 100 consecutive laparotomies for peritoneal malignancy Br J Surg 93 100–104 Occurrence Handle16302177 Occurrence Handle10.1002/bjs.5210 Occurrence Handle1:STN:280:DC%2BD2MnptVajtw%3D%3D
TV Sulkin H O’Neill AI Amin B Moran (2002) ArticleTitleCT in pseudomyxoma peritonei: a review of 17 cases Clin Radiol 57 608–613 Occurrence Handle12096860 Occurrence Handle10.1053/crad.2002.0942 Occurrence Handle1:STN:280:DC%2BD38zlsVejsw%3D%3D
F Alexander-Sefre K Chandrakumaran S Banerjee R Sexton JM Thomas B Moran (2005) ArticleTitleElevated tumour markers prior to complete tumour removal in patients with pseudomyxoma peritonei predict early recurrence Colorectal Dis 7 382–386 Occurrence Handle15932563 Occurrence Handle10.1111/j.1463-1318.2005.00773.x Occurrence Handle1:STN:280:DC%2BD2M3oslOqtA%3D%3D
AD Stephens R Alderman D Chang et al. (1999) ArticleTitleMorbidity and mortality analysis of 200 treatments with cytoreductive surgery and hyperthermic intraoperative intraperitoneal chemotherapy using the coliseum technique Ann Surg Oncol 6 790–796 Occurrence Handle10622509 Occurrence Handle10.1007/s10434-999-0790-0 Occurrence Handle1:STN:280:DC%2BD3c%2Fos1ahsw%3D%3D
J Bryant AJ Clegg MK Sidhu H Brodin P Royle P Davidson (2005) ArticleTitleSystematic review of the Sugarbaker procedure for pseudomyxoma peritonei Br J Surg 92 153–158 Occurrence Handle15685704 Occurrence Handle10.1002/bjs.4862 Occurrence Handle1:STN:280:DC%2BD2M7hslyisA%3D%3D
R Loungnarath S Causeret N Bossard et al. (2005) ArticleTitleCytoreductive surgery with intraperitoneal chemohyperthermia for the treatment of pseudomyxoma peritonei: a prospective study Dis Colon Rectum 48 1372–1379 Occurrence Handle15909071 Occurrence Handle10.1007/s10350-005-0045-5
O Glehen F Mohamed PH Sugarbaker (2004) ArticleTitleIncomplete cytoreduction in 174 patients with peritoneal carcinomatosis from appendiceal malignancy Ann Surg 240 278–285 Occurrence Handle15273552 Occurrence Handle10.1097/01.sla.0000133183.15705.71
Farquharson SM, Murphy E, Sexton R, Cecil TD, Moran BJ. What happens to patients following surgery for peritoneal malignancy with incomplete tumour removal? Analysis of 100 consecutive laparotomies. The Surgeon, J Roy Coll Surg Ed & Ireland 2005;92(Suppl):7.
F Mohamed S Gething M Haiba EA Brun PH Sugarbaker (2004) ArticleTitleClinically aggressive pseudomyxoma peritonei: a variant of a histologically indolent process J Surg Oncol 86 10–15 Occurrence Handle15048674 Occurrence Handle10.1002/jso.20038
BJ Moran (2006) ArticleTitleEstablishment of a peritoneal malignancy treatment centre in the United Kingdom Eur J Surg Oncol 32 614–618 Occurrence Handle16678994 Occurrence Handle10.1016/j.ejso.2006.03.005
P Piso H Bektas U Werner et al. (2001) ArticleTitleImproved prognosis following peritonectomy procedures and hyperthermic intraperitoneal chemotherapy for peritoneal carcinomatosis from appendiceal carcinoma Eur J Surg Oncol 27 286–290 Occurrence Handle11373107 Occurrence Handle10.1053/ejso.2000.1095 Occurrence Handle1:STN:280:DC%2BD3M3ptVWlsw%3D%3D
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Murphy, E.M., Sexton, R. & Moran, B.J. Early Results of Surgery in 123 Patients with Pseudomyxoma Peritonei from a Perforated Appendiceal Neoplasm. Dis Colon Rectum 50, 37–42 (2007). https://doi.org/10.1007/s10350-006-0741-9
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DOI: https://doi.org/10.1007/s10350-006-0741-9