Skip to main content

Advertisement

Log in

Aggressive Management of Peritoneal Carcinomatosis from Mucinous Appendiceal Neoplasms

  • Regional Cancer Therapies
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Peritoneal carcinomatosis (PC) in the setting of mucinous appendiceal neoplasms is characterized by the intraperitoneal accumulation of mucinous ascites and mucin-secreting epithelial cells that leads to progressive compression of intra-abdominal organs, morbidity, and eventual death. We assessed postoperative and oncologic outcomes after aggressive surgical management by experienced surgeons.

Methods

We analyzed clinicopathologic, perioperative, and oncologic outcome data in 282 patients with PC from appendiceal adenocarcinomas between 2001 and 2010 from a prospective database. Kaplan–Meier survival curves and multivariate Cox-regression models were used to identify prognostic factors affecting oncologic outcomes.

Results

Adequate cytoreduction was achieved in 82% of patients (completeness of cytoreduction score (CC)-0: 49%; CC-1: 33%). Median simplified peritoneal cancer index (SPCI), operative time, and estimated blood loss were 14 (range, 0–21), 483.5 min (range, 46–1,402), and 800 ml (range, 0–14,000), respectively. Pathology assessment demonstrated high-grade tumors in 36% of patients and lymph node involvement in 23% of patients. Major postoperative morbidity occurred in 70 (25%) patients. Median overall survival was 6.72 years (95% confidence interval (CI), 4.17 years not reached), with 5 year overall survival probability of 52.7% (95% CI, 42.4, 62%). In a multivariate Cox-regression model, tumor grade, age, preoperative SPCI and chemo-naïve status at surgery were joint significant predictors of overall survival. Tumor grade, postoperative CC-score, prior chemotherapy, and preoperative SPCI were joint significant predictors of time to progression.

Conclusions

Aggressive management of PC from mucinous appendiceal neoplasms, by experienced surgeons, to achieve complete cytoreduction provides long-term survival with low major morbidity.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7(1):69–76.

    Article  PubMed  Google Scholar 

  2. Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM. Disseminated 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. 1995;19(12):1390–408.

    Article  PubMed  CAS  Google Scholar 

  3. Misdraji J, Yantiss RK, Graeme-Cook FM, Balis UJ, Young RH. Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases. Am J Surg Pathol. 2003;27(8):1089–103.

    Article  PubMed  Google Scholar 

  4. Bradley RF, Stewart JH, Russell GB, Levine EA, Geisinger KR. Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review. Am J Surg Pathol. 2006;30(5):551–9.

    Article  PubMed  Google Scholar 

  5. Maheshwari V, Tsung A, Lin Y, Zeh HJ 3rd, Finkelstein SD, Bartlett DL. Analysis of loss of heterozygosity for tumor-suppressor genes can accurately classify and predict the clinical behavior of mucinous tumors arising from the appendix. Ann Surg Oncol. 2006;13(12):1610–6.

    Article  PubMed  Google Scholar 

  6. Yan TD, Black D, Savady R, Sugarbaker PH. A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol. 2007;14(2):484–92.

    Article  PubMed  Google Scholar 

  7. Glehen O, Mohamed F, Gilly FN. Peritoneal carcinomatosis from digestive tract cancer: new management by cytoreductive surgery and intraperitoneal chemohyperthermia. Lancet Oncol. 2004;5(4):219–28.

    Article  PubMed  Google Scholar 

  8. Loungnarath R, Causeret S, Bossard N, Faheez M, Sayag-Beaujard AC, Brigand C, et al. Cytoreductive surgery with intraperitoneal chemohyperthermia for the treatment of pseudomyxoma peritonei: a prospective study. Dis Colon Rectum. 2005;48(7):1372–9.

    Article  PubMed  Google Scholar 

  9. Guner Z, Schmidt U, Dahlke MH, Schlitt HJ, Klempnauer J, Piso P. Cytoreductive surgery and intraperitoneal chemotherapy for pseudomyxoma peritonei. Int J Colorectal Dis. 2005;20(2):155–60.

    Article  PubMed  CAS  Google Scholar 

  10. Sugarbaker PH, Alderman R, Edwards G, Marquardt CE, Gushchin V, Esquivel J, et al. Prospective morbidity and mortality assessment of cytoreductive surgery plus perioperative intraperitoneal chemotherapy to treat peritoneal dissemination of appendiceal mucinous malignancy. Ann Surg Oncol. 2006;13(5):635–44.

    Article  PubMed  Google Scholar 

  11. 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(1):104–9.

    Article  PubMed  Google Scholar 

  12. Stewart JH, Shen P, Russell GB, Bradley RF, Hundley JC, Loggie BL, et al. Appendiceal neoplasms with peritoneal dissemination: outcomes after cytoreductive surgery and intraperitoneal hyperthermic chemotherapy. Ann Surg Oncol. 2006;13(5):624–34.

    Article  PubMed  Google Scholar 

  13. Baratti D, Kusamura S, Nonaka D, Langer M, Andreola S, Favaro M, et al. Pseudomyxoma peritonei: clinical pathological and biological prognostic factors in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Ann Surg Oncol. 2008;15(2):526–34.

    Article  PubMed  Google Scholar 

  14. Yan TD, Links M, Xu ZY, Kam PC, Glenn D, Morris DL. Cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei from appendiceal mucinous neoplasms. Br J Surg. 2006;93(10):1270–6.

    Article  PubMed  CAS  Google Scholar 

  15. Portilla AG, Shigeki K, Dario B, Marcello D. The intraoperative staging systems in the management of peritoneal surface malignancy. J Surg Oncol. 2008;98(4):228–31.

    Article  PubMed  Google Scholar 

  16. Bao P, Bartlett D. Surgical techniques in visceral resection and peritonectomy procedures. Cancer J. 2009;15(3):204–11.

    Article  PubMed  Google Scholar 

  17. Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res. 1996;82:359–74.

    Article  PubMed  CAS  Google Scholar 

  18. Gusani NJ, Cho SW, Colovos C, Seo S, Franko J, Richard SD, et al. Aggressive surgical management of peritoneal carcinomatosis with low mortality in a high-volume tertiary cancer center. Ann Surg Oncol. 2008;15(3):754–63.

    Article  PubMed  Google Scholar 

  19. Dindo D, Demartines N, Clavien PA. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg. 2004;240(2):205–13.

    Article  PubMed  Google Scholar 

  20. 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(2):300–8.

    Article  PubMed  Google Scholar 

  21. 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(2):112–9.

    Article  PubMed  CAS  Google Scholar 

  22. Smith JW, Kemeny N, Caldwell C, Banner P, Sigurdson E, Huvos A. Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience. Cancer. 1992;70(2):396–401.

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of interests

None.

Author information

Authors and Affiliations

Authors

Corresponding authors

Correspondence to David L. Bartlett MD or Haroon A. Choudry MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Austin, F., Mavanur, A., Sathaiah, M. et al. Aggressive Management of Peritoneal Carcinomatosis from Mucinous Appendiceal Neoplasms. Ann Surg Oncol 19, 1386–1393 (2012). https://doi.org/10.1245/s10434-012-2241-6

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1245/s10434-012-2241-6

Keywords

Navigation