Background. Carcinoid tumors are the most common malignant lesions arising from Appendix and are mostly found incidentally during surgery for appendicitis. Perforation of Appendix occurs in 10-20 % of cases with appendicitis. Currently, no guidelines exist for the treatment of perforated carcinoids of Appendix.
Methods. A systematic literature search was performed to identify relevant articles on classical carcinoid or goblet cell carcinoid of Appendix in an attempt to evaluate the impact of perforation on management and prognosis. All articles on carcinoids reporting perforation of Appendix were included.
Results. In total, 23 articles on carcinoid of Appendix with an associated perforation were found. Perforation was never investigated or mentioned as a possible negative factor on recurrence or prognosis. Among a total of 103 patients with classical carcinoids and associated perforation, no peritoneal recurrence or death was described, although follow-up data were often unspecified or scarce. Among a total of 18 goblet cell carcinoids with perforation, metastatic spread to the peritoneum was described in one case and two tumor-related deaths occurred among these cases. No specific relation to perforation could be distilled.
Conclusions. The best available evidence suggests that perforation has no influence on prognosis of classical appendiceal carcinoids. In contrast, peritoneal carcinomatosis is much more common in goblet cell carcinoids but the true impact of perforation remains unclear. Careful follow-up should therefore be considered in these cases.