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Appendicular mucocele: two case reports and literature review
Abstract:
The classification of mucinous tumors of the vermiform appendix is quite controversial, and includes a spectrum of neoplastic lesions ranging from benign proliferations, intraluminal, to invasive adenocarcinomas. Among the complications of appendicular mucinous neoplasms we should mention the "pseudomyxoma peritonei", a condition caused by cancerous cells (mucinous adenocarcinoma) that produce abundant mucin or gelationous ascites. Mucinous neoplasms of the appendix are rare diseases of unknown etiology. The diagnosis is difficult because of poorly specific clinical, biochemical and imaging parameters, and their detection can be occasional. Most of the reported cases involving women of reproductive age (with a history of endometriosis, abdominal surgery or pelvic inflammatory disease). The definitive diagnosis requires histology and immunohistochemistry. Cytoredutive surgery combined with hyperthermic intraperitoneal chemoterapy (HIPEC) is now considered the best treatment for this disease. We present two cases treated with surgery and HIPEC.
Insights
This study addresses controversial appendiceal mucinous tumors, a rare condition potentially leading to pseudomyxoma peritonei. Treatment involves cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC).
Area of Science:
- Gastroenterology
- Oncology
- Surgical Pathology
Background:
- Mucinous tumors of the vermiform appendix present a controversial classification, encompassing a range from benign to invasive adenocarcinomas.
- These rare neoplasms are of unknown etiology and can lead to pseudomyxoma peritonei, characterized by abundant mucin production.
Observation:
- Diagnosis is challenging due to nonspecific clinical, biochemical, and imaging findings, often leading to incidental detection.
- A majority of reported cases involve women of reproductive age, with potential links to endometriosis, prior abdominal surgery, or pelvic inflammatory disease.
Findings:
- Definitive diagnosis relies on histological examination and immunohistochemistry.
- Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the established optimal treatment approach.
Implications:
- The study highlights the diagnostic difficulties and emphasizes the importance of histology and immunohistochemistry for accurate classification.
- It underscores the efficacy of cytoreductive surgery and HIPEC as the current standard of care for appendiceal mucinous neoplasms and pseudomyxoma peritonei.

