Comparative study of mucinous and non-mucinous appendiceal neoplasms with peritoneal dissemination treated by cyoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC)

Eur J Surg Oncol. 2021 May;47(5):1132-1139. doi: 10.1016/j.ejso.2020.08.017. Epub 2020 Aug 25.


Background: Appendiceal non-mucinous neoplasms (AnMN) are rare and poorly understood malignancies with no standard treatment. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is largely used to treat peritoneal disseminations from appendiceal mucinous neoplasms (AMN), but its role with AnMN is unclear.

Methods: A prospective database of 315 patients with advanced appendiceal primaries undergoing CRS/HIPEC during 1996-2020 was reviewed. Baseline characteristics, operative and long-term outcomes of AnMN were compared with those of AMN. AMN were categorized according to PSOGI classification into high-grade, low-grade, and acellular mucin (AC), based on peritoneal disease histology.

Results: Twenty-three patients (7.3%) with goblet cell carcinoma (GCC; n = 9), intestinal-type adenocarcinoma (ITAC; n = 12), and mixed adeno-neuroendocrine carcinoma (MANEC; n = 2) were identified. AnMN patients were more likely to be males (P = 0.006), have preoperative systemic chemotherapy (P = 0.001), grossly incomplete CRS (P = 0.001), and nodal metastases (P = 0.001), but not systemic relapse after CRS/HIPEC (P = 0.133). Median follow-up was 25.1 months (range 0.8-77.3) for AnMN, and 80.9 months (range 0.1-279.2) for AMN. Median overall survival was 24.0 months for AnMN, 66.2 months for high-grade AMN (P = 0.015), 160.0 months for low-grade ANM (P = 0.001), and not reached for AC (P = 0.001). Among AnMN patients, median survival was 23.4 months for GCC, 38.7 months for ITAC, 20.3 months for MANEC (P = 0.855). In the overall series, histological subtype (P = 0.001), incomplete cytoreduction (P = 0.001), and positive lymph-nodes (P = 0.003) correlated with poorer survival at multivariate analysis.

Conclusions: AnMN share with AMN a predominant local-regional dissemination pattern, but prognosis after CRS/HIPEC is worse. This strategy needs to be carefully considered for AnMN.

Keywords: Appendiceal intestinal-type adenocarcinoma; Appendiceal mucinous neoplasm; Cytoreductive surgery; Goblet cell carcinoma; Hyperthermic intraperitoneal chemotherapy (HIPEC); Mixed adeno-neuroendocrine carcinoma; Pseudomyxoma peritonei.

Publication types

  • Comparative Study

MeSH terms

  • Adenocarcinoma, Mucinous / pathology
  • Adenocarcinoma, Mucinous / therapy*
  • Aged
  • Appendiceal Neoplasms / pathology
  • Appendiceal Neoplasms / therapy*
  • Carcinoid Tumor / pathology
  • Carcinoid Tumor / therapy*
  • Cytoreduction Surgical Procedures*
  • Female
  • Humans
  • Hyperthermic Intraperitoneal Chemotherapy*
  • Male
  • Middle Aged
  • Neoplasm Grading
  • Neuroendocrine Tumors / pathology
  • Neuroendocrine Tumors / therapy*
  • Prognosis
  • Prospective Studies