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Annals of Surgical Oncology

, Volume 21, Issue 1, pp 225–231 | Cite as

Mucinous Tumor of the Appendix with Limited Peritoneal Spread: Is There a Role for Expectant Observation?

  • Francis S. W. Zih
  • Nathalie Wong-Chong
  • Claire Hummel
  • Jennifer Petronis
  • Tony Panzarella
  • Aaron Pollett
  • Andrea J. McCart
  • Carol J. Swallow
Gastrointestinal Oncology

Abstract

Background

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is now the standard management for mucinous tumors of appendiceal origin at many centers. We examined the role of expectant observation (EO) in patients who had undergone an initial resection at the time of referral to our center and who had limited residual disease.

Methods

We performed a retrospective review of patients referred to Mount Sinai/Princess Margaret Hospitals, Toronto, for consideration of surgical management of peritoneal malignancy between January 1998 and December 2009. One hundred and three patients with primary mucinous appendiceal malignancy were identified. EO, consisting of regularly scheduled imaging and clinical review, was selected for asymptomatic patients with low-grade tumor and no/limited disease on imaging. Overall survival (OS) and progression-free survival (PFS) were determined.

Results

Management consisted of supportive care in 7 patients, systemic chemotherapy in 7, referral for CRS with HIPEC in 8, CRS without HIPEC at our center in 51, and EO in 30. In the CRS group, 5-year OS was 74 % and PFS was 56 %; both OS and PFS were predicted by extent of residual disease after cytoreduction (p = 0.014 and p = 0.011, respectively). In the EO group, 5-year OS and PFS were 95 and 82 %, respectively. Two patients in the EO group subsequently underwent CRS for progression on imaging.

Conclusions

In well-selected patients who have undergone initial resection for low-grade mucinous tumor of the appendix with limited peritoneal spread, a formal program of observation can result in excellent 5-year OS and PFS. Longer-term follow-up will help define the benefits and risks of this approach.

Keywords

Overall Survival Hyperthermic Intraperitoneal Chemotherapy Early Postoperative Intraperitoneal Chemotherapy Peritoneal Carcinomatosis Index Appendiceal Mucinous Neoplasm 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

DISCLOSURE

The authors declare no conflict of interest to disclose.

References

  1. 1.
    Moran BJ, Cecil TD. The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin North Am. 2003;12:585.CrossRefGoogle Scholar
  2. 2.
    Sugarbaker P. The natural history, gross pathology, and histopathology of appendiceal epithelial neoplasms. Eur J Surg Oncol. 2006;32:644–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Järvinen P, Järvinen H, Lepistö A. Survival of patients with pseudomyxoma peritonei treated by serial debulking. Colorectal Dis. 2009;12:868–72.PubMedCrossRefGoogle Scholar
  4. 4.
    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.PubMedCrossRefGoogle Scholar
  5. 5.
    Chua TC, Yan TD, Smigielski ME, et al. Long-term survival in patients with pseudomyxoma peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy: 10 years of experience from a single institution. Ann Surg Oncol. 2009;16:1903–11.PubMedCrossRefGoogle Scholar
  6. 6.
    Cioppa T, Vaira M, Bing C, D’Amico S, Bruscino A, De Simone M. Cytoreduction and hyperthermic intraperitoneal chemotherapy in the treatment of peritoneal carcinomatosis from pseudomyxoma peritonei. World J Gastroenterol. 2008;14:6817.PubMedCrossRefGoogle Scholar
  7. 7.
    Elias D, Gilly F, Quenet F, et al. Pseudomyxoma peritonei: a French multicentric study of 301 patients treated with cytoreductive surgery and intraperitoneal chemotherapy. Eur J Surg Oncol. 2010;36:456–62.PubMedCrossRefGoogle Scholar
  8. 8.
    Marcotte E, Sideris L, Drolet P, et al. Hyperthermic intraperitoneal chemotherapy with oxaliplatin for peritoneal carcinomatosis arising from appendix: preliminary results of a survival analysis. Ann Surg Oncol. 2008;15:2701–8.PubMedCrossRefGoogle Scholar
  9. 9.
    Sideris L, Mitchell A, Drolet P, Leblanc G, Leclerc YE, Dubé P. Surgical cytoreduction and intraperitoneal chemotherapy for peritoneal carcinomatosis arising from the appendix. Can J Surg. 2009;52:135.PubMedCentralPubMedGoogle Scholar
  10. 10.
    Chua TC, Moran BJ, Sugarbaker PH, 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.PubMedCrossRefGoogle Scholar
  11. 11.
    Saxena A, Yan TD, Chua TC, Morris DL. Critical assessment of risk factors for complications after cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol. 2010;17:1291–301.PubMedCrossRefGoogle Scholar
  12. 12.
    Sugarbaker PH, Alderman R, Edwards G, 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;13:635–44.PubMedCrossRefGoogle Scholar
  13. 13.
    Panarelli NC, Yantiss RK. Mucinous neoplasms of the appendix and peritoneum. Arch Pathol Lab Med. 2011;135:1261–8.PubMedCrossRefGoogle Scholar
  14. 14.
    Edge SB, Compton CC. The American Joint Committee on Cancer: the 7th edition of the AJCC cancer staging manual and the future of TNM. Ann Surg Oncol. 2010;17:1471–4.PubMedCrossRefGoogle Scholar
  15. 15.
    Bosman FT, Carneiro F, Hruban RH, Theise N. WHO classification of tumours of the digestive system. Lyon: World Health Organization; 2010.Google Scholar
  16. 16.
    Dindo D, Demartines N, Clavien PA. Classification of surgical complications: a new proposal with evaluation in a cohort of 6,336 patients and results of a survey. Ann Surg. 2004;240:205.PubMedCrossRefGoogle Scholar
  17. 17.
    Chua TC, Liauw W, Zhao J, Morris DL. Upfront compared to delayed cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei is associated with considerably lower perioperative morbidity and recurrence rate. Ann Surg. 2011;253:769.PubMedCrossRefGoogle Scholar
  18. 18.
    Chua TC, Al-Zahrani A, Saxena A, Liauw W, Zhao J, Morris DL. Secondary cytoreduction and perioperative intraperitoneal chemotherapy after initial debulking of pseudomyxoma peritonei: a study of timing and the impact of malignant dedifferentiation. J Am Coll Surg. 2010;211:526–35.PubMedCrossRefGoogle Scholar
  19. 19.
    Baratti D, Kusamura S, Nonaka D, 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;15:526–34.PubMedCrossRefGoogle Scholar
  20. 20.
    Ronnett BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH. Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis. Cancer. 2001;92:85–91.PubMedCrossRefGoogle Scholar
  21. 21.
    Omohwo C, Nieroda CA, Studeman KD, et al. Complete cytoreduction offers longterm survival in patients with peritoneal carcinomatosis from appendiceal tumors of unfavorable histology. J Am Coll Surg. 2009;209:308–12.PubMedCrossRefGoogle Scholar
  22. 22.
    McDonald J, O’Dwyer S, Rout S, et al. Classification of and cytoreductive surgery for low-grade appendiceal mucinous neoplasms. Br J Surg. 2012;99(7):987–92.PubMedCrossRefGoogle Scholar
  23. 23.
    Klaver Y, Hendriks T, Lomme R, Rutten H, Bleichrodt R, De Hingh I. Intraoperative hyperthermic intraperitoneal chemotherapy after cytoreductive surgery for peritoneal carcinomatosis in an experimental model. Br J Surg. 2010;97:1874–80.PubMedCrossRefGoogle Scholar
  24. 24.
    Pelz JO, Doerfer J, Decker M, Dimmler A, Hohenberger W, Meyer T. Hyperthermic intraperitoneal chemoperfusion (HIPEC) decrease wound strength of colonic anastomosis in a rat model. Int J Colorectal Dis. 2007;22:941–7.PubMedCrossRefGoogle Scholar
  25. 25.
    Aarts F, Bleichrodt R, de Man B, Lomme R, Boerman O, Hendriks T. The effects of adjuvant experimental radioimmunotherapy and hyperthermic intraperitoneal chemotherapy on intestinal and abdominal healing after cytoreductive surgery for peritoneal carcinomatosis in the rat. Ann Surg Oncol. 2008;15:3299–307.PubMedCrossRefGoogle Scholar

Copyright information

© Society of Surgical Oncology 2013

Authors and Affiliations

  • Francis S. W. Zih
    • 1
  • Nathalie Wong-Chong
    • 1
  • Claire Hummel
    • 1
  • Jennifer Petronis
    • 2
  • Tony Panzarella
    • 3
  • Aaron Pollett
    • 4
  • Andrea J. McCart
    • 1
  • Carol J. Swallow
    • 1
  1. 1.Department of Surgical OncologyMount Sinai and Princess Margaret HospitalsTorontoCanada
  2. 2.Department of Radiation OncologyPrincess Margaret HospitalTorontoCanada
  3. 3.Division of BiostatisticsPrincess Margaret HospitalTorontoCanada
  4. 4.Department of Pathology and Laboratory MedicineMount Sinai HospitalTorontoCanada

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