The surgical technique has been described in detail previously and is based on the Sugarbaker principles of peritonectomy with a few modifications. Large tumour nodules in the small bowel must be resected and all visible tumors must be removed to maximize the benefits of peri-operative intraperitoneal chemotherapy. Small cancer deposits on the visceral peritoneum, especially on the surface of tubular structures, are individually electroevaporated. This fundamental technique requires the removal and stripping of all tumour tissues involving the parietal and visceral peritoneum. We here review the application of peritonectomy and hyperthermic intraperitoneal chemotherapy in the treatment of PMP. These methods can achieve satisfactory results in patients with PMP. This technique has been defined as hyperthermic intraperitoneal chemotherapy (HIPEC). The additional effects of hyperthermia, through the use of a special pump, increase local tissue drug concentration and consequently antiblastic drug activity. Surgery is followed by local drug administration aimed at eliminating microscopic and/or minimal residual disease left in the abdominal cavity following surgical manipulations. The natural history of this disease has been drastically modified since the introduction of a new surgical approach, proposed by Sugarbaker who defines it as a peritonectomy procedure consisting of the complete removal of the tumor. The traditional approach to PMP is based on repeated surgical debulking procedures, often associated with intraperitoneal or systemic chemotherapy. The modalities of dissemination are strongly influenced by the histopathology of the primary tumor. The characteristic PMP dissemination within the peritoneal cavity is defined by Sugarbaker as a complete redistribution phenomenon, indicating a complete and sequential invasion of the peritoneal cavity with large tumor volume localization at predetermined anatomical sites and minimal invasion at other sites. Surprisingly, signs and symptoms of cancer such as cachexia are rare. Localized masses are frequently present in PMP of appendiceal origin. Inflammatory changes associated with peritoneal tumour implants can lead to fistula formation and adhesions, which in turn can cause intermittent or chronic partial bowel obstruction. The main complaints are abdominal pain and distension besides a host of non-specific symptoms. Acute presentation during advanced stage of the disease is common. Clinically, although painless, deterioration of general health begins long before diagnosis. However, the appendix is the primary source of most PMPs, which may then spread to other sites like the ovaries. More women appear to suffer from this condition then men. A large proportion of ovarian tumours are secondary to appendiceal tumours. The cases of ovarian origin outnumber those of appendiceal origin. PMP is an indolent disease, which is most prevalent in women aged between 50 and 70 years. With the progress of immunohistochemistry, PMP should be considered as a border line malignant disease because of its inevitable persistence and progression. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is characterised by the production of copious amounts of mucinous fluid resulting in a “jelly belly”. Pseudomyxoma peritonei (PMP) is a rare disease.
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