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Long-Term Outcomes after Surgery for Appendiceal Mucinous Tumours
Audrius Dulskas1, Tomas Poskus2, Eligijus Poskus2
1Department of Abdominal and Oncological Surgery, National Cancer Institute, Vilnius, Lithuania.
Background:
Appendiceal mucinous tumour (AMT) is a very uncommon disease. We aimed to evaluate preoperative and postoperative characteristics as well as long-term outcomes of patients with appendiceal mucinous adenomas and peritoneal pseudomyxoma (PMP).
Methods:
A consecutive cohort of patients diagnosed with AMT were included in the study. Tumour recurrence and the development of PMP was diagnosed based on the patients' complaints, physical examination, imaging studies, and biopsy results. The calculation of survival rates was performed using the Kaplan-Meier method.
Results:
Between January 2003 and February 2013, 25 patients had AMT. 15 patients underwent appendectomy only (60%), 5 patients (20%) right hemicolectomy, 4 patients (16%) cytoreductive surgery (CRS) ± hyperthermic intraperitoneal chemotherapy (HIPEC), and 1 patient (4%) appendectomy with caecal resection. Another 2 patients underwent CRS and HIPEC 4 days and 3 years, respectively, following initial appendectomy. In the histopathologic examination, adenoma was diagnosed in 12 patients (48%); among these, low-grade PMP was found in 1 patient (4%). Low-grade appendiceal mucinous adenocarcinoma (AMCa) was diagnosed in 11 patients (44%); among these, low-grade PMP was found in seven cases (28%) and high-grade AMCa was diagnosed in 2 patients (8%). 2 of 8 PMP patients did not undergo CRS ± HIPEC because of comorbidities. The 5-year overall survival was 100% for the adenomas and 62% for PMP patients.
Conclusion:
AMT is a very rare entity with a considerably good outcome even in disseminated disease. CRS and HIPEC is a gold standard treatment for PMP with excellent prognosis. However, our results could still be improved in the future.
Insights
Appendiceal mucinous tumors (AMT) are rare, but outcomes are good, even with spread. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) offer excellent prognosis for peritoneal pseudomyxoma (PMP).
Area of Science:
- Gastroenterology
- Surgical Oncology
- Oncology
Background:
- Appendiceal mucinous tumors (AMT) represent a rare clinical entity.
- Evaluating patient characteristics and long-term outcomes for appendiceal mucinous adenomas and peritoneal pseudomyxoma (PMP) is crucial.
Purpose of the Study:
- To assess preoperative and postoperative factors influencing outcomes in AMT patients.
- To determine the long-term prognosis for patients with appendiceal mucinous adenomas and PMP.
Main Methods:
- A cohort of 25 patients diagnosed with AMT between 2003 and 2013 was analyzed.
- Tumor recurrence and PMP development were diagnosed through clinical evaluation, imaging, and biopsy.
- Survival rates were calculated using the Kaplan-Meier method.
Main Results:
- Histopathology revealed adenoma in 48% of patients, with low-grade PMP in one case (4%).
- Appendiceal mucinous adenocarcinoma (AMCa) was diagnosed in 44% of patients, with PMP in 28% and high-grade AMCa in 8%.
- Five-year survival was 100% for adenomas and 62% for PMP patients; 2 PMP patients did not receive CRS ± HIPEC due to comorbidities.
Conclusions:
- Appendiceal mucinous tumors (AMT) are rare but generally have favorable outcomes, even in advanced stages.
- Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) represent the gold standard for PMP treatment, yielding excellent prognoses.
- While outcomes are promising, further improvements in treatment strategies are anticipated.
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