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Clinicopathological Characteristics of Primary Appendiceal Mucinous Neoplasm and Recurrence After Radical Resection
Zaibiao Wang1, Manman Yin2, Jiayun Shao3,4
1Department of General Surgery, Bozhou Hospital Affiliated to Anhui Medical University, Bozhou, China.
Objective:
Appendiceal mucinous neoplasm (AMN) is a rare obstructive dilatation of the appendix caused by an intraluminal accumulation of mucoid material, showing an insidious onset and few specific clinical manifestations. The purpose of the study is to analyze clinicopathological characteristics of primary AMN and recurrence after radical resection.
Methods:
A total of 50 patients were included in the retrospective cohort study of AMN. Patient data, such as demographics, tumor characteristics, surgical management, preoperative serum carcinoembryonic antigen (CEA), and carcinoembryonic antigen 19-9 (CA19-9) levels, were collected. All patients were followed-up with interval CT scans until the end of December 2021, with overall survival (OS) and progression-free survival (PFS) being calculated.
Results:
All patients were confirmed as AMN by pathological diagnosis after surgery, including 28 cases (56.00%) of low-grade AMN (LAMN) and 22 cases (44.00%) of non-LAMN. Among 50 patients with AMN, there were 12 cases (24.00%) complicated with pseudomyxoma peritonei (PMP). Higher proportions of patients with pTis, pT3, pT4a, ruptured at presentation, and PMP were found in patients with non-LAMN patients than LAMN (p < 0.05). There was a remarkable difference about preoperative serum CA19-9 levels between patients with LAMN and non-LAMN (p = 0.044). Patients complicated with PMP had a higher proportion of patients with ruptured at presentation than those who were not (p < 0.001). The patients with PMP had increased tumor size compared with those without PMP (p = 0.031). Remarkable differences were observed in terms of preoperative serum CA19-9 (p = 0.009) levels between patients with PMP and without PMP. We performed a multivariate analysis of the presence or absence of PMP and found that ruptured at presentation was found to be a risk factor for PMP in patients with AMN (p = 0.003). The PFS in the patients with PMP and those without was 33.33% (4/12) and 2.63% (1/38), showing a significant difference (P = 0.002).
Conclusion:
The study demonstrates that ruptured at presentation and PMP may influence the prognosis and survival of patients with AMN.
Insights
Appendiceal mucinous neoplasm (AMN) rupture at presentation and pseudomyxoma peritonei (PMP) significantly impact patient prognosis. Early detection and management of these factors are crucial for improving survival rates in AMN patients.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Pathology
Background:
- Appendiceal mucinous neoplasm (AMN) is a rare condition causing appendix dilatation due to mucoid material.
- AMN presents insidiously with non-specific clinical manifestations, complicating early diagnosis.
- Understanding AMN's clinicopathological features is vital for effective management and predicting outcomes.
Purpose of the Study:
- To analyze the clinicopathological characteristics of primary appendiceal mucinous neoplasm (AMN).
- To investigate recurrence patterns and prognostic factors following radical resection of AMN.
- To identify risk factors associated with pseudomyxoma peritonei (PMP) in AMN patients.
Main Methods:
- Retrospective cohort study of 50 patients diagnosed with AMN.
- Collection of patient data including demographics, tumor characteristics, and preoperative serum markers (CEA, CA19-9).
- Follow-up with interval CT scans and calculation of overall survival (OS) and progression-free survival (PFS).
Main Results:
- Of 50 AMN patients, 56% had low-grade AMN (LAMN) and 44% had non-LAMN. 24% developed pseudomyxoma peritonei (PMP).
- Non-LAMN cases showed higher rates of advanced tumor stage, rupture at presentation, and PMP compared to LAMN (p < 0.05).
- Rupture at presentation was a significant risk factor for PMP (p = 0.003), and PMP was associated with significantly worse PFS (33.33% vs 2.63%, p = 0.002).
Conclusions:
- Rupture at presentation and the development of PMP are critical factors influencing the prognosis and survival of AMN patients.
- These findings underscore the importance of recognizing rupture and PMP for risk stratification and treatment planning in AMN.
- Further research into the management of AMN with PMP is warranted to improve patient outcomes.
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