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.
Similar content being viewed by others
References
Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, Gonzalez-Moreno S, Taflampas P, Chapman S, Moran BJ, Peritoneal Surface Oncology Group I. A consensus for classification and pathologic reporting of pseudomyxoma peritonei and associated appendiceal neoplasia: the results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process. Am J Surg Pathol. 2016;40:14–26.
Fournier K, Rafeeq S, Taggart M, Kanaby P, Ning J, Chen HC, Overman M, Raghav K, Eng C, Mansfield P, Royal R. Low-grade appendiceal mucinous neoplasm of uncertain malignant potential (LAMN-UMP): prognostic factors and implications for treatment and follow-up. Ann Surg Oncol. 2017;24:187–93.
Chicago Consensus Working G. The Chicago consensus on peritoneal surface malignancies: management of appendiceal neoplasms. Ann Surg Oncol. 2020;27:1753–60.
Glasgow SC, Gaertner W, Stewart D, Davids J, Alavi K, Paquette IM, Steele SR, Feingold DL. The American Society of Colon and Rectal Surgeons, clinical practice guidelines for the management of appendiceal neoplasms. Dis Colon Rectum. 2019;62:1425–38.
Istl AC, Gage MM, Esquivel J, Ahuja N, Greer JB, Johnston FM. Management of low-grade appendiceal mucinous neoplasms (LAMN): an international survey of surgeons performing CRS and HIPEC. Ann Surg Oncol. 2021;28:3831–7.
Gupta AR, Brajcich BC, Yang AD, Bentrem DJ, Merkow RP. Necessity of posttreatment surveillance for low-grade appendiceal mucinous neoplasms. J Surg Oncol. 2021;124:1115–20.
Li X, Zhou J, Dong M, Yang L. Management and prognosis of low-grade appendiceal mucinous neoplasms: a clinicopathologic analysis of 50 cases. Eur J Surg Oncol: J Eur Soc Surg Oncol Brit Assoc Surg Oncol. 2018;44:1640–5.
Bhutiani N, Mercer MK, Bachman KC, Heidrich SR, Martin RCG 2nd, Scoggins CR, McMasters KM, Ajkay N. Evaluating the effect of margin consensus guideline publication on operative patterns and financial impact of breast cancer operation. J Am Coll Surg. 2018;227:6–11.
Rajeev R, Klooster B, Turaga KK. Impact of surgical volume of centers on post-operative outcomes from cytoreductive surgery and hyperthermic intra-peritoneal chemoperfusion. J Gastrointest Oncol. 2016;7:122–8.
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.
Author information
Authors and Affiliations
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
Ethics declarations
DISCLOSURE
The authors have no competing interests to report
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Supplementary Information
Below is the link to the electronic supplementary material.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1245/s10434-023-13501-8