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Prognostic significance of acellular mucin in patients undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) for appendiceal neoplasms

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Abstract

Introduction

Appendiceal neoplasms have a propensity for peritoneal dissemination. The standard of care for select individuals is CRS/HIPEC. In the current 8th AJCC Staging system, a finding of only intraperitoneal acellular mucin (M1a) is classified as Stage IVa. There is concern that the current AJCC system may over-stage patients.

Methods

This was a single-institution retrospective review of 164 cases of mucinous appendiceal neoplasm. Patients undergoing CRS/HIPEC with M1a disease were compared to patients with peritoneal deposits containing tumor cells (well-differentiated adenocarcinoma; low-grade mucinous carcinoma peritonei—M1b,G1). Overall and recurrence-free survival were assessed.

Results

Median age was 51 years, 70% were female, and 75% White. Sixty-four patients had M1a disease and 100 M1b,G1 disease. M1a disease had a lower median PCI score (11 vs. 20, p = .0001) and a higher rate of complete CRS (62% vs. 50%, p = .021). Median follow-up was 7.6 years (IQR 5.6–10.5 years). For M1a disease, there were no recurrences and only one patient died during the study interval. In comparison, for M1b disease, 66/100 (66%) recurred with a 5-year RFS of 40.5% (HR 8.0, 95% CI 4.9–15.1, p < .0001), and 31/100 (31%) died with a 5-year OS of 84.8% (HR 4.5, 95% CI 2.2–9.2, p < .0001).

Conclusions

Acellular mucin (M1a disease) after CRS/HIPEC for appendiceal neoplasm is associated with longer OS and RFS compared to M1b, G1 disease. Current AJCC staging does not accurately reflect the differing outcomes of these two patient populations. The presence of acellular mucin in the peritoneal cavity should not be perceived as a metastatic equivalent.

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Data availability

Patient data for this study is maintained in a prospective clinical database supported by the University of Texas MD Anderson Cancer Center and managed by the institutional review board (IRB). Inquires for access to this information should be made to Dr. Christopher Scally. All software used for this analysis are available to the public for purchase.

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Funding

Conrad Family Appendiceal Cancer Research Fund.

William Payne Family Appendiceal Cancer Research Fund.

Bidnick Family Appendiceal Cancer Research Fund.

Schroeder Family Appendiceal Cancer Research Fund.

National Cancer Institute (L30 CA171000 and K22 CA234406 to J.P.S.

Cancer Prevention & Research Institute of Texas (RR180035 to J.P.S.)

Cancer Center Support Grant P30 CA016672).

Sun and Do Lee GI Research and Care Fund.

Dwyer Endowment for Surgical Oncology.

Author information

Authors and Affiliations

  1. Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1484, Houston, TX, 77030-4009, USA

    Derek J. Erstad, Kristen A. Robinson, Karen Beaty, Safia Rafeeq, Yi-Ju Chiang, Paul. F. Mansfield, Keith F. Fournier & Christopher P. Scally

  2. Department of Gastrointestinal Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Kanwal Raghav, John P. Shen & Michael J. Overman

  3. Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Wai Chin Foo & Melissa W. Taggart

  4. Department of Surgery, Maine Medical Center, Portland, MN, USA

    Richard E. Royal

Authors
  1. Derek J. Erstad
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Contributions

DJE wrote the main manuscript. DJE, KAR, KB, SR, YJC, and CPS performed chart reviews and data analysis for manuscript generation. KR, JPS, MJO, WCF, MWT, PFM, and RER provided expert opinion and manuscript review. CPS and KF contributed to study design, data analysis, and manuscript generation.

Corresponding author

Correspondence to Derek J. Erstad.

Ethics declarations

Ethics approval

This retrospective study was reviewed and approved by the MD Anderson Cancer Center Institutional Review Board prior to initiation.

Study and publication consent

All patients included in this study provided signed consent for inclusion of their health information into a prospective clinical database maintained at the University of Texas MD Anderson Cancer Center. Consent includes publication of deidentified information for studies derived from this prospective database. All patient information for this study was derived from this database and has been deindentified.

Competing interests

The authors declare no competing interests.

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Keith F. Fournier and Christopher P. Scally share co-senior authorship.

Synopsis:

We evaluated patients with appendiceal neoplasm and peritoneal dissemination in order to determine the prognostic significance of disseminated acellular mucin vs. cellular mucin. Among a cohort of 64 patients with only acellular mucin (AJCC Stage IVa, M1a disease), we detected zero clinically evident recurrences after cytoreductive surgery and HIPEC.

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Erstad, D.J., Robinson, K.A., Beaty, K. et al. Prognostic significance of acellular mucin in patients undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) for appendiceal neoplasms. Langenbecks Arch Surg 408, 110 (2023). https://doi.org/10.1007/s00423-022-02732-0

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