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Treatment Variation and Long-Term Outcomes of Low-Grade Appendiceal Neoplasms

  • Peritoneal Surface Malignancy
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Abstract

Background

Heterogenous nomenclature describing appendiceal neoplasms has added to uncertainty around their appropriate treatment. Although a recent consensus has established the term low-grade appendiceal neoplasm (LAMN), we hypothesize that significant variation remains in the treatment of LAMNs.

Methods

We retrospectively reviewed our prospectively maintained appendiceal registry, identifying patients with LAMNs from 2009 to 2019. We assessed variability in treatment, including whether patients underwent colectomy, spread of disease at presentation, and long-term outcomes.

Results

Of 136 patients with LAMNs, 88 (35%) presented with localized disease and 48 (35%) with disseminated peritoneal disease. Median follow-up was 2.9 years (IQR 1.9–4.4), and 120 (88%) patients underwent pre-referral surgery. Among 26 pre-referral colectomy patients, 23 (88%) were performed for perceived oncologic need/nodal evaluation; no nodal metastases were identified. In patients with resected LAMNs without radiographic evidence of disseminated disease, 41 (47%) underwent second look diagnostic laparoscopy (DL) to evaluate for occult metastases. No peritoneal metastases were identified. Patients with disseminated disease were treated with cytoreductive surgery/heated intraperitoneal chemotherapy (CRS/HIPEC). For patients undergoing CRS/HIPEC, 5-year recurrence-free survival was 94% (95% CI 81–98%). For patients with localized disease, 5-year RFS was 98% (95% CI 85–99%).

Conclusions

Significant variation exists in treatment patterns for LAMNs, particularly prior to referral to a high-volume center. Patients frequently underwent colectomy without apparent oncologic benefit. In the current era of high-quality cross sectional imaging, routine use of DL has low yield and is not recommended. Recurrence in this population is rare, and low-intensity surveillance can be offered. Overall prognosis is excellent, even with peritoneal disease.

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Acknowledgement

We thank the Bidnick Appendix Cancer Research Fund, the Schroeder Appendix Cancer Research Fund, the Volz Appendix Cancer Research Fund, the David Dwyer Research Fund, and the Sunny and Do Lee Research Fund.

Funding

The authors have no competing interests to report.

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Authors

Contributions

MGW and CPS were responsible for study conception and design; MT, WCF, PM, KFF, and CPS were responsible for data collection; MGW, NB, BAH, PFM, CFF, and CPS were responsible for analysis and interpretation of results; and MGW, NB, and CPS were responsible for draft manuscript preparation. All authors reviewed the results and approved the final version of the manuscript

Corresponding author

Correspondence to Christopher P. Scally MD, MS.

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White, M.G., Bhutiani, N., Helmink, B.A. et al. Treatment Variation and Long-Term Outcomes of Low-Grade Appendiceal Neoplasms. Ann Surg Oncol 30, 8138–8143 (2023). https://doi.org/10.1245/s10434-023-13501-8

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  • DOI: https://doi.org/10.1245/s10434-023-13501-8

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