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

Q: Why is systemic chemotherapy often less effective in mucinous adenocarcinoma of the appendix with peritoneal carcinomatosis?

The article notes that these cancers have a high tendency toward peritoneal metastasis and typically show low response to systemic treatment.

Q: What treatment combination is described as the standard for peritoneal metastases in this setting?

Cytoreductive surgery (CRS) to remove visible disease followed by hyperthermic intraperitoneal chemoperfusion (HIPEC) to address microscopic disease is described as standard treatment.

Q: What outcome was achieved after repeat CRS and HIPEC in the reported case?

Repeat CRS and HIPEC resulted in durable remission lasting 28 months without adjuvant chemotherapy, despite an initial incomplete cytoreduction and high-volume disease.

Sep 28, 2026

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Mucinous adenocarcinoma of the appendix is a rare lower gastrointestinal cancer that commonly progresses to peritoneal metastasis and shows limited response to systemic therapy. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is an established approach to prolong survival in peritoneal carcinomatosis. Repeat CRS and HIPEC are feasible for selected patients, though repeat cytoreduction with high tumor burden is usually avoided due to morbidity and mortality concerns. This report describes repeat CRS and HIPEC for extensive appendiceal mucinous peritoneal carcinomatosis after initial incomplete cytoreduction, achieving durable remission for 28 months without adjuvant chemotherapy. It suggests repeat CRS can yield a durable response despite prior CRS failure and high-volume disease.

LanguageEnglish

CategoryHealthcare

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Pages7

Reading Time18 min

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Abstract

Introduction

Case

diseases Case Report Durable Response after Repeat Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) ina Patient with Extensive Mucinous Adenocarcinoma of the Appendix Dalia Kaakour 1, Garrett Ward 2, Maheswari Senthil 3,† and Farshid Dayyani 1, *,† Citation: Kaakour, D.; Ward, G.; Senthil, M.; Dayyani, F. Durable Response after Repeat Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in a Patient with Extensive Mucinous Adenocarcinoma of the Appendix. Diseases 2023, 11, 60 . [https://doi.org/10.3390/](https://doi.org/10.3390/)[ ](https://doi.org/10.3390/)diseases11020060 Academic Editor: Carlo Catassi Received: 31 January 2023 Revised: 17 March 2023 Accepted: 28 March 2023 Published: 6 April 2023 Copyright: © 2023 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ([https://](https://)[ ](https://)[creativecommons.org/licenses/by/](creativecommons.org/licenses/by/)[ ](creativecommons.org/licenses/by/)[4.0/](4.0/)) . 1 Department of Medicine, Division of Hematology and Oncology, University of California Irvine, Orange, CA 92868, USA 2 Department of Radiological Sciences, Division of Abdominal Imaging, University of California Irvine, Orange, CA 92868, USA 3 Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, CA 92868, USA * Correspondence: [fdayyani@hs.uci.edu](fdayyani@hs.uci.edu)[ ](fdayyani@hs.uci.edu)† These authors contributed equally to this work. 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. Keywords: mucinous adenocarcinoma of the appendix; peritoneal carcinomatosis; cytoreduction and HIPEC 1. Introduction Mucinous adenocarcinoma (MAC) of the appendix is a relatively uncommon form of GI malignancy and accounts for <0.5% of all gastrointestinal neoplasms [1] . The incidence of MAC of the appendix is rapidly increasing [2], and patients often present with a more advanced stage, typically due to metastasis in the peritoneal cavity. The extent of surgical resection for localized appendiceal mucinous adenocarcinoma is slightly controversial. However, generally speaking, an appendectomy alone may be adequate for low-grade disease if negative margins are achieved [3,4] . Right hemicolectomy is considered the standard-of-care therapy for high-grade disease due to the risk of regional lymph node spread, which is reported in 17–72% of cases [4] . Usually, peritoneal metastases of MAC remain isolated to the peritoneal cavity, rendering these patients appropriate candidates for aggressive locoregional therapies [5] . The combination of

FAQs

Why is systemic chemotherapy often less effective in mucinous adenocarcinoma of the appendix with peritoneal carcinomatosis?

The article notes that these cancers have a high tendency toward peritoneal metastasis and typically show low response to systemic treatment.

What treatment combination is described as the standard for peritoneal metastases in this setting?

Cytoreductive surgery (CRS) to remove visible disease followed by hyperthermic intraperitoneal chemoperfusion (HIPEC) to address microscopic disease is described as standard treatment.

What outcome was achieved after repeat CRS and HIPEC in the reported case?

Repeat CRS and HIPEC resulted in durable remission lasting 28 months without adjuvant chemotherapy, despite an initial incomplete cytoreduction and high-volume disease.

Sep 28, 2026

Uploaded by Aria