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Outcomes in Peritoneal Carcinomatosis from Appendiceal Goblet Cell Carcinoma Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC)

  • Peritoneal Surface Malignancy
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Abstract

Background

Appendiceal goblet cell adenocarcinoma (GCA) is often misclassified and mistreated due to mixed histologic features. In general, cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is standard of care for peritoneal carcinomatosis (PC) from mucinous appendiceal tumors; however, in PC from GCA, data are limited and the role of CRS/HIPEC is controversial. We report outcomes in PC from appendiceal GCA treated with CRS/HIPEC.

Patients and Methods

A prospective institutional database of 391 CRS/HIPEC patients with appendiceal carcinomatosis from 1998 to 2018 was reviewed. Twenty-seven patients with GCA were identified. Perioperative variables were described. Survival was estimated using the Kaplan–Meier method.

Results

GCA occurred in 7% (27/391) of appendiceal CRS/HIPEC patients. Seven (26%) cases were aborted. Two patients underwent a second CRS/HIPEC for peritoneal recurrence. Median age at diagnosis was 53 years (range 39–72 years), and 12 (60%) were female. All underwent previous surgery. Seven (35%) had prior chemotherapy and received a median of 5 cycles (range 3–8). Median PCI was 6 (range 1–39). Complete cytoreduction was achieved in 95% (19/20). Grade III complications occurred in three (15%) patients, and no perioperative deaths occurred. Median follow-up was 97 months. Overall survival at 1, 3 and 5 years was 100%, 74% and 67%, respectively. Progression-free survival at 1, 3, and 5 years was 94%, 67% and 59%, respectively.

Conclusion

CRS/HIPEC should be considered as the main treatment option for patients with PC from appendiceal GCA. When performed at a CRS/HIPEC specialty center, 5-year OS of 67% can be achieved.

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References

  1. Shaib W, Krishna K, Kim S, et al. Appendiceal neuroendocrine, goblet and signet-ring cell tumors: a spectrum of diseases with different patterns of presentation and outcome. Cancer Res Treat Off J Korean Cancer Assoc. Apr 2016;48(2):596–604.

    Google Scholar 

  2. Clift AK, Frilling A. Neuroendocrine, goblet cell and mixed adeno-neuroendocrine tumours of the appendix: updates, clinical applications and the future. Exp Rev Gastroent Hepatol. Mar 2017;11(3):237–47.

    CAS  Google Scholar 

  3. Clift AK, Kornasiewicz O, Drymousis P, et al. Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management. Endocr Connect. Feb 2018;7(2):268–77.

    Article  Google Scholar 

  4. Wen KW, Hale G, Shafizadeh N, Hosseini M, Huang A, Kakar S. Appendiceal goblet cell carcinoid: common errors in staging and clinical interpretation with a proposal for an improved terminology. Hum Pathol. Jul 2017;65:187–93.

    Article  Google Scholar 

  5. Chetty RDP, Klimstra DS, Henson DE, Albores-Saavedra J. Combined classical carcinoid and goblet cell carcinoid tumor: a new morphologic variant of carcinoid tumor of the appendix. Am J Surg Pathol. Aug 2010;34:1163–7.

    Article  Google Scholar 

  6. Burke AP, Sobin LH, Federspiel BH, Shekitka KM, Helwig EB. Goblet cell carcinoids and related tumors of the vermiform appendix. Am J Clin Pathol. Jul 1990;94(1):27–35.

    Article  CAS  Google Scholar 

  7. Taggart MW, Abraham SC, Overman MJ, Mansfield PF, Rashid A. Goblet cell carcinoid tumor, mixed goblet cell carcinoid-adenocarcinoma, and adenocarcinoma of the appendix: comparison of clinicopathologic features and prognosis. Arch Pathol Lab Med. Jun 2015;139(6):782–90.

    Article  Google Scholar 

  8. Pham TH, Wolff B, Abraham SC, Drelichman E. Surgical and chemotherapy treatment outcomes of goblet cell carcinoid: a tertiary cancer center experience. Ann Surg Oncol. Mar 2006;13(3):370–6.

    Article  Google Scholar 

  9. Madsen AH, Ladekarl M, Villadsen GE, et al. Effects of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of goblet cell carcinoma: a prospective cohort study. Ann Surg Oncol. Feb 2018;25(2):422–30.

    Article  Google Scholar 

  10. Shankar S, Ledakis P, El Halabi H, Gushchin V, Sardi A. Neoplasms of the appendix: current treatment guidelines. Hematol Oncol Clin North Am. Dec 2012;26(6):1261–90.

    Article  Google Scholar 

  11. Sugarbaker P. technical handbook for the integration of cytoreductive surgery and perioperative intraperitoneal chemotherapy into the surgical management of gastrointestinal and gynecologic malignancy. 4th ed. Washington, D.C.: Foundation for Applied Research in Gastrointestinal Oncology; 2005.

    Google Scholar 

  12. Clavien PA, Barkun J, de Oliveira ML, et al. The Clavien–Dindo classification of surgical complications: five-year experience. Ann Surg. Aug 2009;250(2):187–96.

    Article  Google Scholar 

  13. Network NCC. Colon cancer (2.2019). 2019. www.nccn.org/store/login/login.aspx? https://www.nccn.org/professionals/physician_gls/pdf/colon.pdf. Accessed 5 Aug 2019.

  14. Subbuswamy SG, Gibbs NM, Ross CF, Morson BC. Goblet cell carcinoid of the appendix. Cancer. Aug 1974;34(2):338–44.

    Article  CAS  Google Scholar 

  15. Yozu M, Johncilla ME, Srivastava A, et al. Histologic and outcome study supports reclassifying appendiceal goblet cell carcinoids as goblet cell adenocarcinomas, and grading and staging similarly to colonic adenocarcinomas. Am J Surg Pathol. Jul 2018;42(7):898–910.

    Article  Google Scholar 

  16. van Eeden S, Offerhaus GJ, Hart AA, et al. Goblet cell carcinoid of the appendix: a specific type of carcinoma. Histopathology. Dec 2007;51(6):763–73.

    Article  Google Scholar 

  17. Rossi RE, Luong TV, Caplin ME, et al. Goblet cell appendiceal tumors–management dilemmas and long-term outcomes. Surg Oncol. Mar 2015;24(1):47–53.

    Article  Google Scholar 

  18. Liu E, Telem DA, Warner RRP, Dikman A, Divino CM. The role of Ki-67 in predicting biological behavior of goblet cell carcinoid tumor in appendix. Am J Surg. 2011;202:400–3.

    Article  CAS  Google Scholar 

  19. Tang LH, Shia J, Soslow RA, et al. Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix. Am J Surg Pathol. Oct 2008;32(10):1429–43.

    Article  Google Scholar 

  20. Pape UF, Perren A, Niederle B, et al. ENETS consensus guidelines for the management of patients with neuroendocrine neoplasms from the jejuno-ileum and the appendix including goblet cell carcinomas. Neuroendocrinology. 2012;95(2):135–56.

    Article  CAS  Google Scholar 

  21. 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. Dec 2014;21(13):4218–25.

    Article  Google Scholar 

  22. Sardi A, Sipok A, Díaz-Montes T, Sittig M, Nieroda C, Studeman K, Gushchin V. Outcomes of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in the treatment of primary peritoneal carcinoma. J Peritoneum (Other Serosal Surfaces). Aug 2017;2(2):2170–7.

    Google Scholar 

  23. Aydin N, Sardi A, Milovanov V, et al. Outcomes of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal mesothelioma: experience of a peritoneal surface malignancy center. Am Surg. Dec 2015;81(12):1253–9.

    PubMed  Google Scholar 

  24. Mahteme H, Sugarbaker PH. Treatment of peritoneal carcinomatosis from adenocarcinoid of appendiceal origin. Br J Surg. Sep 2004;91(9):1168–73.

    Article  CAS  Google Scholar 

  25. Randle RW, Griffith KF, Fino NF, et al. Appendiceal goblet cell carcinomatosis treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Surg Res. Jun 15 2015;196(2):229–34.

    Article  Google Scholar 

  26. McConnell YJ, Mack LA, Gui X, et al. Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy: an emerging treatment option for advanced goblet cell tumors of the appendix. Ann Surg Oncol. Jun 2014;21(6):1975–82.

    Article  Google Scholar 

  27. Sugarbaker PH. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in the management of gastrointestinal cancers with peritoneal metastases: progress toward a new standard of care. Cancer Treat Rev. Jul 2016;48:42–9.

    Article  Google Scholar 

  28. Goere D, Souadka A, Faron M, et al. Extent of colorectal peritoneal carcinomatosis: attempt to define a threshold above which HIPEC does not offer survival benefit: a comparative study. Ann Surg Oncol. Sep 2015;22(9):2958–64.

    Article  Google Scholar 

  29. Elias D, Mariani A, Cloutier AS, et al. Modified selection criteria for complete cytoreductive surgery plus HIPEC based on peritoneal cancer index and small bowel involvement for peritoneal carcinomatosis of colorectal origin. Eur J Surg Oncol. Nov 2014;40(11):1467–73.

    Article  CAS  Google Scholar 

  30. Halabi HE, Gushchin V, Francis J, et al. Prognostic significance of lymph node metastases in patients with high-grade appendiceal cancer. Ann Surg Oncol. January 01 2012;19(1):122–5.

    Article  Google Scholar 

  31. Ledakis P, King MC, Munoz C, Gushchin V, Sittig M, Sardi A. Colorectal/gastric/other digestive peritoneal metastases: C73 preoperative systemic chemotherapy in patients with PMCA of appendiceal origin treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Pleura Peritoneum. 2018;3(s1):A162.

    Article  Google Scholar 

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Correspondence to Armando Sardi MD, FACS.

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Zambrano-Vera, K., Sardi, A., Munoz-Zuluaga, C. et al. Outcomes in Peritoneal Carcinomatosis from Appendiceal Goblet Cell Carcinoma Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC). Ann Surg Oncol 27, 179–187 (2020). https://doi.org/10.1245/s10434-019-07932-5

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  • DOI: https://doi.org/10.1245/s10434-019-07932-5

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