Pseudomyxoma peritonei is a rare entity manifested by implants of a mucinous gelatinous material arising from either the appendix or ovary and involves the peritoneal cavity, peritoneum and omentum. Preoperative diagnosis is feasible by means of radiographic studies. The main step in the treatment is debulking surgery with appendectomy, bilateral oophorectomy and omentectomy. Adjuvant radiotherapy and chemotherapy has been used. Long term survival is around 54% at five years. Here we present a case of a ruptured mucocele of the appendix and pseudomyxoma who presents as massive increase in the abdominal girth of two years evolution.
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Long term outcomes associated with the use of perioperative systemic chemotherapy on low grade appendiceal mucinous neoplasms with pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
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Department of Surgery, Division of Surgical Oncology, University of Texas Southwestern Medical Center, Dallas, TX, United States.
Introduction: Low grade appendiceal mucinous neoplasms (LAMN) are indolent tumors that lack invasive potential but may present as pseudomyxoma peritonei. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) significantly improves both overall and recurrence free survival. While systemic chemotherapy is generally considered ineffective for LAMN, little literature is available to support this notion.
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