Pseudomyxoma peritonei syndrome 12 months after an intact resection of malignant mucocele of the appendix: A case report

Mucocele of the appendix (MA) is an obstructive dilatation of the appendix caused by either a benign or malignant process. MAs are rare lesions found in only 0.2%– 0.3% of appendectomies. Malignant mucoceles are found even less frequently. It is believed that an intact mucocele presents no risk for the patient. González Moreno et al. has emphasized that peritoneal seeding should be preventable with atraumatic handling of the cancer specimen. These authors also noted that peritoneal dissemination from a MA has not been regarded as a problem with open appendectomy. The incidence of disseminated peritoneal mucinous tumors from an open appendectomy is extremely low, and no reported cases were found in the literature. We report the case of a patient with no rupture of the MA during the primary surgery who presented after 12 months of follow-up with peritoneal dissemination typical of pseudomyxoma peritonei (PP). To the best of our knowledge, no such case has previously been reported.