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

Ann Surg Oncol. 2020 Jan;27(1):179-187. doi: 10.1245/s10434-019-07932-5. Epub 2019 Oct 23.


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.

MeSH terms

  • Adenocarcinoma / mortality
  • Adenocarcinoma / secondary
  • Adenocarcinoma / therapy*
  • Adult
  • Aged
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Appendiceal Neoplasms / mortality
  • Appendiceal Neoplasms / pathology
  • Appendiceal Neoplasms / therapy*
  • Carcinoid Tumor / mortality
  • Carcinoid Tumor / secondary
  • Carcinoid Tumor / therapy*
  • Chemotherapy, Adjuvant
  • Combined Modality Therapy
  • Cytoreduction Surgical Procedures*
  • Female
  • Follow-Up Studies
  • Goblet Cells / pathology
  • Humans
  • Hyperthermia, Induced*
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local
  • Peritoneal Neoplasms / mortality
  • Peritoneal Neoplasms / secondary
  • Peritoneal Neoplasms / therapy*
  • Retrospective Studies
  • Survival Rate
  • Treatment Outcome