Perforated Goblet Cell Carcinoid Tumors of the Appendix: Navigating the Management Conundrum

Abstract

Appendix is the most common site of occurrence for a goblet cell carcinoid tumor. A diagnosis of an appendiceal goblet cell carcinoid is made in retrospect the majority of the time. These tumors are best treated with a right hemicolectomy and adjuvant therapies tailored according to the presence or absence of residual disease. Presentation as a perforated appendix is seen in 16% of these tumors. The natural history and the ideal management strategy in such a scenario are not well described. In those with peritoneal spread cytoreductive surgery with HIPEC (hyperthermic intraperitoneal chemotherapy) offers the best disease-free and progression-free survival. Close follow-up with cross-sectional imaging helps in identifying recurrences at the earliest. Multimodality management involving patient participation in every aspect of care accomplishes high-value care in the treatment of these tumors.