Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for appendiceal mucocele tumors: Five case reports and review of literature.

Abstract

Background: Appendiceal mucocele with pseudomyxoma peritonei (PMP) is a rare entity that often presents late and requires specialized management. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) has become the standard of care, yet real-world surgical experiences from diverse institutions remain limited. This case series highlights five patients presenting with appendiceal mucoceles complicated by PMP, emphasizing diagnostic challenges, operative strategies, and postoperative outcomes.

Case summary: We report five patients (aged 45-75 years) who presented with symptoms ranging from abdominal distension and umbilical nodules to incidental imaging findings. All patients underwent a comprehensive diagnostic evaluation, including laboratory testing, tumor markers, and advanced imaging. Intraoperative findings revealed mucinous dissemination consistent with PMP. CRS was performed in all cases, with the extent determined by disease burden, followed by HIPEC using mitomycin C or oxaliplatin-based regimens. Pathology demonstrated low-grade or ruptured mucinous neoplasms as the primary etiology. Postoperative recovery was generally favorable, and although three patients developed recurrence requiring repeat CRS, all achieved meaningful disease control. These cases illustrate PMP's chronic nature and the importance of multidisciplinary management.

Conclusion: CRS with HIPEC remains the most effective strategy for managing appendiceal mucocele-associated PMP.