Outcomes in Peritoneal Dissemination from Signet Ring Cell Carcinoma of the Appendix Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy

Ann Surg Oncol. 2019 Feb;26(2):473-481. doi: 10.1245/s10434-018-7007-3. Epub 2018 Dec 6.

Abstract

Background: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is standard treatment for peritoneal dissemination from appendiceal cancer (AC); however, its role in high-grade histopathologic subtypes (high-grade mucinous carcinoma peritonei [HGMCP] and HGMCP with signet ring cells [HGMCP-S]) is controversial due to their aggressive behavior. This study analyzed clinical outcomes of high-grade AC after CRS/HIPEC.

Methods: A prospective database of CRS/HIPEC procedures for HGMCP performed from 1998-2017 was reviewed. Perioperative variables and survival were analyzed.

Results: Eighty-six HGMCP and 65 HGMCP-S were identified. HGMCP had more positive tumor markers (TM) (CEA/CA-125/CA-19-9) than HGMCP-S (63% vs 40%, p = 0.005). HGMCP had higher Peritoneal Cancer Index (32 vs 26, p = 0.097) and was less likely to have positive lymph nodes (LN) than HGMCP-S (28% vs 69%, p = < 0.001). Complete cytoreduction was achieved in 84% and 83%, respectively. PFS at 3- and 5-years was 59% and 48% for HGMCP vs 31% and 14% for HGMCP-S. Median PFS was 4.3 and 1.6 years, respectively (p < 0.001). OS at 3- and 5-years was 84% and 64% in HGMCP vs 38% and 25% in HGMCP-S. Median OS was 7.5 and 2.2 years, respectively (p < 0.001). LN negative HGMCP-S had longer median PFS and OS than LN positive HGMCP-S (PFS: 3.4 vs 1.5 years, p = 0.03; OS: 5.6 vs 2.1 months, p = 0.021).

Conclusions: The aggressive histology of HGMCP-S is associated with poor OS, has fewer abnormal TM, and is more likely to have positive LN. However, CRS/HIPEC can achieve a 5-year survival of 25%, which may improve to 51% with negative LN.

MeSH terms

  • Adult
  • Aged
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Appendiceal Neoplasms / mortality*
  • Appendiceal Neoplasms / pathology
  • Appendiceal Neoplasms / therapy
  • Carcinoma, Signet Ring Cell / mortality*
  • Carcinoma, Signet Ring Cell / pathology
  • Carcinoma, Signet Ring Cell / therapy
  • Chemotherapy, Cancer, Regional Perfusion / mortality*
  • Combined Modality Therapy
  • Cytoreduction Surgical Procedures / mortality*
  • Female
  • Follow-Up Studies
  • Humans
  • Hyperthermia, Induced / mortality*
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local / mortality*
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / therapy
  • Peritoneal Neoplasms / mortality*
  • Peritoneal Neoplasms / pathology
  • Peritoneal Neoplasms / therapy
  • Prognosis
  • Prospective Studies
  • Retrospective Studies
  • Survival Rate
  • Young Adult