An International Registry of Peritoneal Carcinomatosis from Appendiceal Goblet Cell Carcinoma Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy

World J Surg. 2022 Jun;46(6):1336-1343. doi: 10.1007/s00268-022-06498-w. Epub 2022 Mar 13.

Abstract

Purpose: Peritoneal carcinomatosis from appendiceal goblet cell carcinoma (A-GCC) is a rare and aggressive form of appendiceal tumor. Cytoreductive surgery (CRS) and hyperthermic intra peritoneal chemotherapy (HIPEC) was reported as an interesting alternative regarding survival compared to surgery without HIPEC and/or systemic chemotherapy. Our aim was to evaluate the impact of CRS and HIPEC for patients presenting A-GCC through an international registry.

Methods: A prospective multicenter international database was retrospectively searched to identify all patients with A-GCC tumor and peritoneal metastases who underwent CRS and HIPEC through the Peritoneal Surface Oncology Group International (PSOGI). The post-operative complications, long-term results, and principal prognostic factors were analyzed.

Results: The analysis included 83 patients. After a median follow-up of 47 months, the median overall survival (OS) was 34.6 months. The 3- and 5-year OS was 48.5% and 35.7%, respectively. Patients who underwent complete macroscopic CRS had a significantly better survival than those treated with incomplete CRS. The 5-year OS was 44% and 0% for patients who underwent complete, and incomplete CRS, respectively (HR 9.65, p < 0.001). Lymph node involvement and preoperative chemotherapy were also predictive of a worse prognosis. There were 3 postoperative deaths, and 30% of the patients had major complications.

Conclusion: CRS and HIPEC may increase long-term survival in selected patients with peritoneal metastases of A-GCC origin, especially when complete CRS is achieved. Ideally, randomized control trials or more retrospective data are needed to confirm CRS and HIPEC as the gold standard in this pathology.

Publication types

  • Multicenter Study

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Appendiceal Neoplasms* / pathology
  • Appendiceal Neoplasms* / therapy
  • Carcinoma* / surgery
  • Combined Modality Therapy
  • Cytoreduction Surgical Procedures
  • Goblet Cells / pathology
  • Humans
  • Hyperthermia, Induced*
  • Hyperthermic Intraperitoneal Chemotherapy
  • Peritoneal Neoplasms* / secondary
  • Peritoneal Neoplasms* / therapy
  • Prospective Studies
  • Registries
  • Retrospective Studies
  • Survival Rate