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Clinicopathologic and Prognostic Features in Patients with Peritoneal Metastasis from Mucinous Adenocarcinoma, Adenocarcinoma with Signet Ring Cells, and Adenocarcinoid of the Appendix Treated with Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy

  • Gastrointestinal Oncology
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Tumors that show a signet ring or adenocarcinoid histomorphology have been associated with a poor prognosis. This study aimed to analyze the clinicopathologic and prognostic features in patients with peritoneal metastasis from mucinous adenocarcinoma (PMCA), adenocarcinoma with signet ring cell (PMCA-S), or adenocarcinoid (PMCA-A) of the appendix treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy.

Methods

Retrospective analysis of a prospectively maintained database for all patients treated for appendiceal adenocarcinoma from 1989 to 2012 was performed.

Results

The study cohort consisted of 494 patients including 361 patients with PMCA (73.1 %), 80 patients with PMCA-S (16.2 %), and 53 patients with PMCA-A (10.7 %). The patients comprised 273 men (55.3 %) and 221 women (44.7 %) with a mean age at presentation of 50.7 years for the PMCA-S patients, 47.3 years for the PMCA patients, and 47.5 years for the PMCA-A patients (p < 0.03). The 3- and 5-year survival rates were respectively 51 and 38 % for PMCA compared with 30 and 22 % for PMCA-S and 26 and 15 % for PMCA-A. The median survival time was 45.4 months for PMCA compared with 18.9 months for PMCA-S and 26.8 months for PMCA-A (p < 0.000). The groups did not differ significantly in the completeness of cytoreduction achieved, with 53.5 % of the PMCA patients having a CC0/1 compared with 46.2 % of the PMCA-S patients and 41.6 % of the PMCA-A patients (p < 0.20). In the multivariate analysis, the independent predictors of a reduced survival were incompleteness of cytoreduction, histomorphology of PMCA-S or PMCA-A, and distant metastasis.

Conclusion

The findings showed that PMCA-S or PMCA-A histomorphology contributes to the poor prognosis associated with peritoneal metastasis from appendiceal adenocarcinoma. The independent predictors for a poor overall survival included incompleteness of cytoreduction, PMCA-S and PMCA-A histomorphology, and distant metastasis.

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References

  1. McCusker ME, Cote TR, Clegg LX, et al. Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology, and end-results program, 1973–1998. Cancer. 2002;94:3307–12.

    Article  PubMed  Google Scholar 

  2. Nitecki SS, Wolff BG, Schlinkert R, et al. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg. 1994;219:51–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. 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.

    Article  CAS  PubMed  Google Scholar 

  4. Ronnett BM, Kurman RJ, Zahn CM, et al. Pseudomyxoma peritonei in women: a clinicopathologic analysis of 30 cases with emphasis on site of origin, prognosis, and relationship to ovarian mucinous tumors of low malignant potential. Hum Pathol. 1995;26:509–24.

    Article  CAS  PubMed  Google Scholar 

  5. Carr NJ, Sobin LH. Adenocarcinoma of the appendix. In: Bosman FT, Carneiro F, Hruban RH, Theise ND (eds) WHO Classification of Tumours of the Digestive System. International Agency for Research on Cancer (IARC), Lyon, 2010, pp 122–5.

    Google Scholar 

  6. 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;19:1390–408.

    Article  CAS  PubMed  Google Scholar 

  7. 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.

    Article  CAS  PubMed  Google Scholar 

  8. Didolkar MS, Fanous N. Adenocarcinoma of the appendix: a clinicopathologic study. Dis Colon Rectum. 1977;20:130–4.

    Article  CAS  PubMed  Google Scholar 

  9. Chang P, Attiyeh FF. Adenocarcinoma of the appendix. Dis Colon Rectum. 1981;24:176–80.

    Article  CAS  PubMed  Google Scholar 

  10. Cortina R, McCormick J, Kolm P, Perry RR. Management and prognosis of adenocarcinoma of the appendix. Dis Colon Rectum. 1995;38:848–52.

    Article  CAS  PubMed  Google Scholar 

  11. Cerame MA. A 25-year review of adenocarcinoma of the appendix: a frequently perforating carcinoma. Dis Colon Rectum. 1988;31:145–50.

    Article  CAS  PubMed  Google Scholar 

  12. Qizilbash AH. Primary adenocarcinoma of the appendix: a clinicopathological study of 11 cases. Arch Pathol Lab Med. 1975;99:556–62.

    CAS  Google Scholar 

  13. Butler JA, Houshiar A, Lin F, et al. Goblet cell carcinoid of the appendix. Am J Surg. 1994;168:685–7.

    Article  CAS  PubMed  Google Scholar 

  14. Ratzenhofer M, Aubock L. The amphicrine (endo-exocrine) cells in the human gut, with a short reference to amphicrine neoplasias. Acta Morphol Acad Sci Hung. 1980;28:37–58.

    CAS  PubMed  Google Scholar 

  15. Warkel RL, Cooper PH, Helwig EB. Adenocarcinoid, a mucin-producing carcinoid tumor of the appendix: a study of 39 cases. Cancer. 1978;42:2781–93.

    Article  CAS  PubMed  Google Scholar 

  16. Klein HZ. Mucinous carcinoid tumor of the vermiform appendix. Cancer. 1974;33:770–7.

    Article  CAS  PubMed  Google Scholar 

  17. Gallegos NC, Milroy C, Linehan IP, et al. Crypt cell carcinoma of the appendix. Eur J Surg Oncol. 1992;18:386–7.

    CAS  PubMed  Google Scholar 

  18. Isaacson P. Crypt cell carcinoma of the appendix (so-called adenocarcinoid tumor). Am J Surg Pathol. 1981;5:213–24.

    Article  CAS  PubMed  Google Scholar 

  19. Haqqani MT, Williams G. Mucin-producing carcinoid tumours of the vermiform appendix. J Clin Pathol. 1977;30:473–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Watson PH, Alguacil-Garcia A. Mixed crypt cell carcinoma: a clinicopathological study of the so-called “goblet cell carcinoid.” Virchows Arch A Pathol Anat Histopathol. 1987;412:175–82.

    Article  CAS  PubMed  Google Scholar 

  21. Edmonds P, Merino MJ, LiVolsi VA, Duray PH. Adenocarcinoid (mucinous carcinoid) of the appendix. Gastroenterology. 1984;86:302–9.

    CAS  PubMed  Google Scholar 

  22. Sugarbaker PH. Dissection by electrocautery with a ball tip. J Surg Oncol. 1994;56:246–8.

    Article  CAS  PubMed  Google Scholar 

  23. Jacquet P, Sugarbaker PH. Current methodologies for clinical assessment of patients with peritoneal carcinomatosis. J Exp Clin Cancer Res. 1996;15:49–58.

    Google Scholar 

  24. Sugarbaker PH. An overview of peritonectomy, visceral resections, and perioperative chemotherapy for peritoneal surface malignancy. In: Sugarbaker PH (ed) Cytoreductive Surgery and Perioperative Chemotherapy for Peritoneal Surface Malignancy: Textbook and Video Atlas. Cine-Med Publishing, Woodbury, CT, 2012, pp 1–30.

    Google Scholar 

  25. Mohamed F, Sugarbaker PH. Intraperitoneal taxanes. Surg Oncol Clin North Am. 2003;12:825–33.

    Article  Google Scholar 

  26. Sugarbaker PH. An instrument to provide containment of intraoperative intraperitoneal chemotherapy with optimized distribution. J Surg Oncol. 2005;92:142–6.

    Article  PubMed  Google Scholar 

  27. Sugarbaker P, Bijelic L, Chang et al. Neoadjuvant FOLFOX chemotherapy in 34 consecutive patients with mucinous peritoneal carcinomatosis of appendiceal origin. J Surg Oncol. 2010;102:576–81.

    Article  PubMed  Google Scholar 

  28. Bijelic L, Kumar A, Stuart A, et al. Systemic chemotherapy prior to cytoreductive surgery and HIPEC for carcinomatosis from appendix cancer: impact on perioperative outcomes and short-term survival. Gastroenterol Res Pract. 2012;2012:163284.

    PubMed  PubMed Central  Google Scholar 

  29. Murphy EM, Farquharson SM, Moran BJ. Management of an unexpected appendiceal neoplasm. Br J Surg. 2006;93:783–92.

    Article  CAS  PubMed  Google Scholar 

  30. 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. Ann Surg Oncol. 2007;15:526–34.

    Article  PubMed  Google Scholar 

  31. Smeenk R, Verwaal V, Antonini N, et al. Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg. 2007;245:104–9.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Jimenez W, Sardi A, Nieroda C, et al. Predictive and prognostic survival factors in peritoneal carcinomatosis from appendiceal cancer after cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2014;21:4218–25.

    Article  PubMed  Google Scholar 

  33. Glehen O, Mohamed F, Sugarbaker P. Incomplete cytoreduction in 174 patients with periotoneal carcinomatosis from appendiceal malignancy. Ann Surg. 2004;240:278–85.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Baumgartner J, Tobin L, Heavey S, et al. Predictors of progression in high-grade appendiceal or colorectal peritoneal carcinomatosis after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2015;22:1716–21.

    Article  PubMed  Google Scholar 

  35. Ko YH, Park SH, Jung CK, et al. Clinical characteristics and prognostic factors for primary appendiceal carcinoma. Asia Pac J Clin Oncol. 2010;6:19–27.

    Article  PubMed  Google Scholar 

  36. Misdraji J, Young RH. Primary epithelial neoplasms and other epithelial lesions of the appendix (excluding carcinoid tumors). Semin Diagn Pathol. 2004;21:120–33.

    Article  PubMed  Google Scholar 

  37. Park IJ, Yu CS, Kim HC, et al. Clinical features and prognostic factors in primary adenocarcinoma of the appendix. Korean J Gastroenterol. 2004;43:29–34.

    PubMed  Google Scholar 

  38. McGory ML, Maggard MA, Kang H, et al. Malignancies of the appendix: beyond case series reports. Dis Colon Rectum. 2005;48:2264–71.

    Article  PubMed  Google Scholar 

  39. Turaga K, Pappas S, Gamblin C. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol. 2012;19:1379–85.

    Article  PubMed  Google Scholar 

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Correspondence to Chukwuemeka Ihemelandu MD, FACS.

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Ihemelandu, C., Sugarbaker, P.H. Clinicopathologic and Prognostic Features in Patients with Peritoneal Metastasis from Mucinous Adenocarcinoma, Adenocarcinoma with Signet Ring Cells, and Adenocarcinoid of the Appendix Treated with Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy. Ann Surg Oncol 23, 1474–1480 (2016). https://doi.org/10.1245/s10434-015-4995-0

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