Annals of Surgical Oncology

, Volume 26, Issue 2, pp 473–481 | Cite as

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

  • Carlos Munoz-Zuluaga
  • Armando SardiEmail author
  • Mary Caitlin King
  • Carol Nieroda
  • Michelle Sittig
  • Ryan MacDonald
  • Vadim Gushchin
Gastrointestinal Oncology



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.


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


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).


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.



Carlos Munoz-Zuluaga, Armando Sardi, Mary Caitlin King, Carol Nieroda, Michelle Sittig, Ryan MacDonald, and Vadim Gushchin declare that there are no conflicts of interest regarding the publication of this paper.


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Copyright information

© Society of Surgical Oncology 2018

Authors and Affiliations

  1. 1.The Institute for Cancer CareMercy Medical CenterBaltimoreUSA
  2. 2.Center for Clinical ExcellenceMercy Medical CenterBaltimoreUSA

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