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Appendiceal mucoceles and pseudomyxoma peritonei
S Landen1, C Bertrand, G J Maddern
1Digestive Surgery Unit, Jolimont Hospital, Haine St. Paul, Belgium.
Abstract:
Mucoceles of the appendix and associated pseudomyxoma peritonei are a heterogeneous group comprising various histopathologic lesions with differing prognoses. Between 1983 and 1990, we treated eight patients with appendiceal mucocele, three cystadenomas and five cystadenocarcinomas, three of which had accompanying pseudomyxoma peritonei. All patients were more than 50 years of age. Women outnumbered men by seven to one. Preoperative diagnosis was acute appendicitis or appendiceal abscess in all instances of mucocele unaccompanied by pseudomyxoma peritonei. Ultrasound of the abdomen, together with paracentesis, diagnosed pseudomyxoma peritonei in two of three patients. Elevated carcinoembryonic antigen levels were found in six of the eight patients and monitoring of this parameter was useful in the early detection of the two recurrences observed. The three patients with cystadenomas remain free of disease after appendectomy. Of the five patients treated for cystadenocarcinoma by right colectomy, two underwent reoperation after recurrence of disease. One patient died 41 months later of intestinal obstruction caused by pseudomyxoma peritonei. Pseudomyxoma peritonei significantly decreases survival of patients with appendiceal mucocele. In these patients, aggressive initial surgical management, repeated if need be, is indicated.
Insights
Appendiceal mucoceles, including cystadenomas and cystadenocarcinomas, have varied outcomes. Pseudomyxoma peritonei significantly worsens survival, necessitating aggressive surgical management for appendiceal mucocele patients.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Pathology
Background:
- Appendiceal mucoceles represent a spectrum of appendiceal neoplasms.
- These lesions can range from benign cystadenomas to malignant cystadenocarcinomas.
- Associated pseudomyxoma peritonei presents unique diagnostic and management challenges.
Purpose of the Study:
- To analyze the clinical presentation, diagnosis, and outcomes of patients with appendiceal mucoceles.
- To evaluate the impact of pseudomyxoma peritonei on patient survival.
- To determine optimal surgical management strategies for appendiceal mucoceles.
Main Methods:
- Retrospective review of eight patients treated for appendiceal mucocele between 1983 and 1990.
- Histopathologic classification into cystadenoma and cystadenocarcinoma.
- Correlation of clinical data, diagnostic imaging (ultrasound), tumor markers (CEA), and surgical outcomes.
Main Results:
- Five cystadenocarcinomas and three cystadenomas were identified; three cases had pseudomyxoma peritonei.
- Pseudomyxoma peritonei was diagnosed preoperatively in two of three patients using ultrasound and paracentesis.
- Elevated carcinoembryonic antigen (CEA) levels aided in recurrence detection.
- Cystadenoma patients remained disease-free post-appendectomy.
- Cystadenocarcinoma patients treated with right colectomy experienced recurrences, with one death due to pseudomyxoma peritonei complications.
Conclusions:
- Appendiceal mucoceles have diverse histopathology and prognoses.
- Pseudomyxoma peritonei is a critical factor negatively impacting survival.
- Aggressive and potentially repeated surgical intervention is indicated for appendiceal mucocele with pseudomyxoma peritonei.