Durable Response after Repeat Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in a Patient with Extensive Mucinous Adenocarcinoma of the Appendix

Abstract

Mucinous adenocarcinoma of the appendix is a rare form of lower gastrointestinal (GI) tract cancer. These cancers have a high tendency to progress towards peritoneal metastasis and their response to systemic treatment is typically low. Together, cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have become an established form of therapy used to prolong the survival of patients with this disease. Repeat CRS and HIPEC have been shown to be feasible in selected patients with GI peritoneal carcinomatosis (PC), among which those with appendix cancer receive the greatest benefit. The peritoneal cancer index (PCI) and completeness of cytoreduction have been shown to be important predictors of outcomes. However, repeat cytoreduction in patients with a high-volume peritoneal tumor burden (peritoneal cancer index (PCI) > 30) is not typically performed due to concerns regarding morbidity and mortality. Herein, we describe a case of repeat CRS and HIPEC for extensive appendiceal mucinous peritoneal carcinomatosis after initial incomplete cytoreduction and durable remission of 28 months without adjuvant chemotherapy. In appendiceal mucinous cancers, repeat CRS can achieve a durable response despite an initial failed CRS and high-volume disease.

The Vermiform Appendix and Its Pathologies

“…Controversies related to this therapy come from the different results obtained by the studies. Studies with positive results come in support of the use of HIPEC [145,147,[150][151][152]179]. On the other hand, several recent phase III randomized trials show that HIPEC does not benefit disease progression-free survival or overall survival.…”

“…For mucinous adenocarcinomas, which are invasive mucinous neoplasms, the standard surgical treatment includes right hemicolectomy, although its effectiveness is not entirely accepted [143] due to the high recurrence rate. Better results, in terms of disease remission and prolongation of the disease-free period, are achieved by chemotherapy [144] and mainly by the combination of repeated cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, even in the presence of a large initial volume [145].…”

“…Right hemicolectomy [143] Chemotherapy [144] Cytoreductive surgery + hyperthermic intraperitoneal chemotherapy [145] Goblet cell adenocarcinomas without carcinomatosis Right hemicolectomy [146] Goblet cell adenocarcinomas with carcinomatosis Cytoreductive surgery + hyperthermic intraperitoneal chemotherapy [147] Goblet cell adenocarcinomas, stages III, IV Cytoreductive surgery + adjuvant chemotherapy [148] Nonmucinous appendiceal adenocarcinomas/signet ring adenocarcinomas without metastases Right hemicolectomy [149] Nonmucinous appendiceal adenocarcinomas/signet ring adenocarcinomas with metastases Right hemicolectomy [149] + standard systemic chemotherapy (fluorouracil, platinum, and irinotecan [153]) + cytoreductive surgery/hyperthermic intraperitoneal chemotherapy [150][151][152] Localized nonmucinous appendiceal adenocarcinomas/signet ring adenocarcinomas without node involvement Right hemicolectomy [149] + adjuvant chemotherapy (fluoropyrimidine/oxaliplatin) [153] Neuroendocrine neoplasms < 2 cm Appendectomy [155] Neuroendocrine neoplasms > 2 cm Right hemicolectomy [155] Resecable metastatic neuroendocrine neoplasms Cytoreductive surgery [155,157] Non…”