Progression of perforated cystadenoma of the appendix to pseudomyxoma peritonei over 18 years. A case report

INTRODUCTION AND IMPORTANCE: In the past, mucinous appendiceal neoplasms (MAN) greater than 2 cm in diameter were treated by a right colon resection. New data shows that treatment options are to be determined by the histopathologic grade of the appendiceal tumor and the condition of the wall of the appendix (intact vs. breached). CASE PRESENTATION: A 39-year-old woman had an incidental diagnosis of a low-grade appendiceal mucinous neoplasm (LAMN) at the time of a hysterectomy. The appendiceal tumor had small quantities of mucus surrounding an enlarged appendix. No tumor cells were seen in the mucus by histologic study. The patient was placed in follow-up. Eighteen years later she required treatment for advanced pseudomyxoma peritonei. CLINICAL DISCUSSION: When 5 different histopathologic types of MAN are considered with an intact vs. perforated wall of the appendix, four different treatment options develop. With LAMN and well or moderately differentiated mucinous appendiceal adenocarcinoma (MACA), the patient does not require operative intervention if the wall of the appendix is intact. If mucus or mucus plus tumor cells are identified outside the appendix an intervention is indicated. In patients, as the one presented, in whom only small amounts of mucus are outside the appendix, surveillance may be recommended. CONCLUSIONS: In patients with a diagnosed low-grade MAN, dissemination to regional lymph nodes is rare. Dissemination to the peritoneal space places the patient at risk to develop pseudomyxoma peritonei. As this case report illustrates, if surveillance is recommended, long-term follow-up is required.