Perforated Carcinoid Tumor of the Appendix: Need for Guidelines for Management With Respect to Prognostic Factors

Carcinoid tumors, one of the most common malignant lesions involving the appendix, are typically found incidentally during routine appendectomies. While up to 20% of acute appendicitis cases present with perforation, the incidence of perforation among patients with undiagnosed carcinoid tumors of the appendix is unknown. In addition, there is no consensus on the management of carcinoid tumors in the perforated appendix or its impact on prognosis. We present a case of a 42-year-old woman presented with perforated appendicitis. Final pathology demonstrated the presence of a 1.1 cm, well-differentiated grade 1 neuroendocrine tumor at the tip of the appendix extending into the subserosa, without evidence for lymphovascular invasion. Given the depth of tumor invasion and the relatively young age of the patient, the decision was made to perform an interval completion right hemicolectomy for lymph node sampling. Only a few cases have been reported in the available literature, and it remains unclear whether appendiceal perforation represents an independent negative prognostic factor for patient survival. Additional data from cohort studies are needed to determine the true incidence, prognosis, and optimal management of newly diagnosed carcinoid tumors in the perforated appendix. Furthermore, clear consensus guidelines are needed to identify the subgroup of patients who would benefit from interval or primary right hemicolectomy.