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Surveillance of low-grade appendiceal mucinous neoplasms for progression to pseudomyxoma peritonei: Results from a
Enda Hannan1, Lorena Martin Roman1, Lukas O'Brien1
1The National Centre for Peritoneal Malignancy, Mater Misericordiae University Hospital, Dublin, Ireland.
Aim:
Low-grade appendiceal mucinous neoplasm (LAMN) of the appendix is a rare tumour that can progress to pseudomyxoma peritonei (PMP). There is a lack of standardization of surveillance following resection of LAMN as the progression rate to PMP is unclear. The aim of this study was to evaluate the rate of progression following resection of LAMN to PMP in a structured surveillance programme.
Method:
Data for all patients referred for LAMN surveillance from 2013 to 2021 were retrospectively collected. The surveillance regime consisted of annual CT and tumour markers for a 5-year period. Patients who progressed to PMP were identified.
Results:
Of the patients enrolled in surveillance following appendicectomy and LAMN diagnosis (65.1% female, median age 56 years), 83 had completed at least 1 year of surveillance (median follow-up 24 months). Of these, 6% (n = 5) showed disease progression during follow-up. The median time to progression was 23 months. Survival analysis revealed no statistically significant difference in progression with regards to T staging (p = 0.39), margin positivity (p = 0.11) or appendiceal perforation (p = 0.26). No patients with Tis disease developed PMP. A statistically significant difference in progression was seen in patients with M1b staging (p = 0.021) and in those with mucin beyond the right iliac fossa at diagnosis (p = 0.04).
Conclusion:
The observed progression rate justifies the necessity of postappendicectomy surveillance in patients with LAMN, with the risk of progression being highest within the first 3 years of diagnosis. The described surveillance programme allows for early detection of subclinical progression to PMP.
Insights
Surveillance after appendiceal mucinous neoplasm resection is crucial, as 6% of patients progressed to pseudomyxoma peritonei within 3 years. Early detection of subclinical progression is possible with structured monitoring.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Oncology
Background:
- Low-grade appendiceal mucinous neoplasm (LAMN) is a rare appendix tumor with potential to progress to pseudomyxoma peritonei (PMP).
- Standardized surveillance protocols post-LAMN resection are lacking due to unclear progression rates.
- This study aimed to determine LAMN progression to PMP in a structured surveillance program.
Purpose of the Study:
- To evaluate the rate of progression from LAMN to PMP following surgical resection.
- To identify risk factors associated with disease progression.
- To assess the effectiveness of a structured surveillance program for early PMP detection.
Main Methods:
- Retrospective collection of data for patients undergoing LAMN surveillance from 2013 to 2021.
- Surveillance protocol included annual CT scans and tumor marker assessments for 5 years.
- Identification of patients who progressed to PMP during the follow-up period.
Main Results:
- Of 83 patients completing at least one year of surveillance, 6% (n=5) progressed to PMP.
- Median time to progression was 23 months, with highest risk within the first 3 years.
- Progression was significantly associated with M1b staging (p=0.021) and extratumoral mucin (p=0.04), but not T staging, margin status, or perforation.
Conclusions:
- The observed progression rate supports the necessity of post-appendicectomy surveillance for LAMN patients.
- The risk of progression to PMP is highest within the first three years post-diagnosis.
- The structured surveillance program facilitates early detection of subclinical PMP progression.
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