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[Appendiceal mucoceles. Pathophysiology and therapeutic indications]
M Scotté1, A Laquerrière, Y Riff
1Service de Chirurgie Générale et Digestive, Hôpital Charles-Nicolle, Rouen.
Abstract:
Appendiceal mucoceles (AM) are rare lesions of the appendix, characterized by an accumulation of mucus. Two main pathogenic mechanisms may be invoked to explain their development. Firstly, AM are secondary to an obstruction of the appendiceal lumen for a wide variety of reasons. Secondly, they may be due to tumours of the appendix, whether malignant (cystadenocarcinomas) or benign (cystadenomas), responsible for a hypersecretion of mucus. Intraperitoneal mucinous effusion (IME) develops when appendiceal perforation occurs, especially with malignant AM. We found 13 retention AM and in most a definite obstructive lesion was present. There were 3 malignant AM, all associated with a neoplastic IME. While they are frequently described in the literature, no cystadenoma was observed in these series. Clinical symptoms are often confusing, but paraclinical investigations may lead to preoperative diagnosis. Appendectomy is the treatment of retention AM and cystadenoma. Their prognosis is related to other associated diseases, namely ovarian and colorectal tumours, but is otherwise good. Cystadenocarcinomas require a right hemicolectomy with evacuation of IME but their prognosis is poor.
Insights
Appendiceal mucoceles (AM) are rare appendix lesions caused by obstruction or tumors. Treatment varies by type, with retention AMs treated by appendectomy and malignant forms requiring hemicolectomy.
Area of Science:
- Gastroenterology
- Surgical Pathology
- Oncology
Background:
- Appendiceal mucoceles (AM) are uncommon appendix lesions characterized by mucus accumulation.
- Pathogenesis involves appendiceal lumen obstruction or neoplastic (benign/malignant) or non-neoplastic hypersecretion.
- Perforation, particularly of malignant AM, can lead to intraperitoneal mucinous effusion (IME).
Purpose of the Study:
- To investigate the pathogenic mechanisms, clinical presentation, and treatment outcomes of appendiceal mucoceles.
- To differentiate between retention AM and neoplastic AM.
- To evaluate the prognostic factors associated with different types of AM.
Main Methods:
- Retrospective analysis of 13 cases of appendiceal mucoceles.
- Review of clinical symptoms, paraclinical investigations, and surgical findings.
- Histopathological examination to determine the nature of the AM and associated lesions.
Main Results:
- 13 retention AMs were identified, mostly associated with obstructive lesions.
- 3 malignant AMs (cystadenocarcinomas) were found, all linked to neoplastic IME.
- No benign cystadenomas were observed in this series.
- Preoperative diagnosis was achievable through paraclinical investigations despite confusing clinical symptoms.
Conclusions:
- Retention AMs, often caused by obstruction, are treated with appendectomy with a good prognosis.
- Malignant AMs (cystadenocarcinomas) necessitate right hemicolectomy and have a poor prognosis.
- Appendiceal mucocele management and prognosis depend on the underlying cause and presence of malignancy.