Long term outcomes associated with the use of perioperative systemic chemotherapy on low grade appendiceal mucinous neoplasms with pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

Samantha M Ruff ¹, Gyulnara Kasumova ², Manoj Palavalli ¹

¹Department of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center/James Comprehensive Cancer Center, Columbus, OH, United States.
²Division of Surgical Oncology, Department of Surgery, Central Maine Medical Center, Lewiston, ME, United States.
³Department of Surgical Oncology, University of California Irvine Medical Center, Irvine, CA, United States.
⁴Department of Surgical Oncology, University of Utah, Salt Lake City, UT, United States.
⁵Department of Surgical Oncology, Medical College of Wisconsin, Milwaukee, WI, United States.
⁶Department of Surgical Oncology, University of Cincinnati, Cincinnati, OH, United States.
⁷Department of Surgical Oncology, University of California San Diego, San Diego, CA, United States.
⁸Department of Surgical Oncology, MD Anderson Cancer Center, Houston, TX, United States.
⁹Department of Surgical Oncology, University of Wisconsin Medical Center, Madison, WI, United States.
¹⁰Department of Surgical Oncology, Emory University Hospital, Atlanta, GA, United States.
¹¹Department of Surgery, Mayo Clinic, Rochester, MN, United States.
¹²Department of Surgical Oncology, The Johns Hopkins University, Baltimore, MD, United States.
¹³Department of Surgical Oncology, City of Hope Cancer Center, Duarte, CA, United States.
¹⁴Department of Surgical Oncology, Moffitt Cancer Center, Tampa, FL, United States.
¹⁵Department of Surgery, Division of Surgical Oncology, University of Texas Southwestern Medical Center, Dallas, TX, United States.

⁰Department of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center/James Comprehensive Cancer Center, Columbus, OH, United States.

Frontiers in Oncology +

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January 24, 2025

View abstract on PubMed

Summary

Systemic chemotherapy combined with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS+HIPEC) was associated with worse outcomes for low-grade appendiceal mucinous neoplasms (LAMN). These findings suggest systemic chemotherapy should not be routinely recommended for LAMN patients undergoing CRS+HIPEC.

Area Of Science

Oncology
Surgical Oncology
Gastrointestinal Oncology

Background

Low-grade appendiceal mucinous neoplasms (LAMN) are indolent tumors with low invasive potential, but can present as pseudomyxoma peritonei.
Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is a standard treatment that improves survival for LAMN.
The role of systemic chemotherapy in conjunction with CRS+HIPEC for LAMN remains poorly defined.

Purpose Of The Study

To evaluate the impact of systemic chemotherapy on overall survival (OS) and recurrence-free survival (RFS) in patients with LAMN undergoing CRS+HIPEC.
To compare outcomes between LAMN patients who received CRS+HIPEC with and without systemic chemotherapy.

Main Methods

A multicenter retrospective cohort study was conducted using data from the US HIPEC Collaborative.
Patients with LAMN who underwent CRS+HIPEC were analyzed.
Overall survival (OS) and recurrence-free survival (RFS) were compared between patients who received systemic chemotherapy and those who did not, using Kaplan-Meier analysis and Cox regression.

Main Results

Of 529 LAMN patients, 63 (11.9%) received systemic chemotherapy alongside CRS+HIPEC.
Patients receiving systemic chemotherapy had a higher disease burden (mean PCI: 18.8 vs 14.3).
Patients treated with CRS+HIPEC alone demonstrated significantly better OS (104.3 months) and RFS (84.9 months) compared to those who received systemic chemotherapy (OS: 70.2 months, RFS: 38 months; p<0.001).
Factors associated with worse OS included higher pre-operative CEA levels, higher completeness of cytoreduction scores (CCR2, CCR3), and treatment with systemic chemotherapy (HR 4.196, p=0.045).

Conclusions

Perioperative systemic chemotherapy was associated with worse long-term outcomes in LAMN patients undergoing CRS+HIPEC.
Systemic chemotherapy may lead to patient deconditioning, potentially contributing to poorer outcomes.
Systemic chemotherapy should not be recommended for LAMN patients undergoing CRS+HIPEC outside of clinical trials.

Keywords:

HIPEC LAMN chemotherapy cytoreductive surgery low grade appendiceal mucinous neoplasm

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