Approach to pseudomyxoma peritonei

Abstract

Pseudomyxoma peritonei (PMP) is a mucinous tumour of the appendix that spreads into the peritoneal cavity in the form of gelatinous deposits. The incidence of PMP is believed to be approximately 1-3 out of a million per year. Nonetheless, due to its indolent nature, it is usually discovered at an advanced stage and severely impacts quality of life. Curative treatment for PMP is complete cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). An extensive literature review was conducted searching EMBASE, MEDLINE, PubMed, and Google Scholar databases for PMP in aims to delineate a clinical approach to diagnosis and treatment. Literature was limited to the years 2007-2018. We found the 5-year overall survival with CRS and HIPEC estimated to be between 23%-82% and rates of major complications as high as 24%. Therefore, it is important to appropriately stage and select patients that should undergo CRS with HIPEC. Modalities like MDCT radiological scores have been shown to have sensitivity and specificity of 94% and 81%, respectively, in being able to predict resectability and survival. Despite treatment, the disease often recurs. Tumor markers have significant potential for establishing prognosis pre-operatively, and this paper will review the most recent evidence in support of them.

Keywords: Appendix; Cytoreductive surgery; Hyperthermic intraperitoneal chemotherapy; Mucocele; Pseudomyxoma peritonei.

Figure 1

Mucinous ascites on computed tomography[24]. This computed tomography scan demonstrates mucinous ascites. We also see characteristic findings of pseudomyxoma peritonei: Scalloping of the liver and spleen.

Figure 2

Histological classification of pseudomyxoma peritonei[25]. This figure highlights some of the characteristic findings in the different histological types of pseudomyxoma peritonei. A, B: Disseminated peritoneal adenomucinous (DPAM) is demonstrated in (A and B) with paucicellular mucin pools (A) and scant strips of low-grade neoplastic epithelium (B); C, D: Peritoneal mucinous carcinamatosis-intermediate (PMCA-I) is demonstrated in (C and D). PMCA-I is less cellular than PMCA, but the degree of atypia exceeds that of DPAM (D); E, F: Highlight PMCA, with mucin cells with abundant epithelium (E) and malignant cytological features (F).

Figure 3

Computed tomography of pseudomyxoma peritonei[26]. This is an axial computed tomography scan. A: Cystic accumulations of mucus (arrows) surrounded by calcified rims; B: A coronal reconstruction representing cystic accumulations in the upper abdomen and the liver.

Figure 4

Intraoperative pictures of cytoreductive surgery[11]. This is a figure depicting various stages of cytoreductive surgery. A: A view of a pseudomyxoma patient’s abdominal cavity immediately after laparotomy; B: Stripping of the right anterior peritoneum; C: Depicts stripping of the right subphrenic peritoneum; D: An image of the resected terminal ileum, colon, and spleen affected by pseudomyxoma peritonei.

Figure 5

Hyperthermic intraperitoneal chemotherapy after complete cytoreductive surgery[27]. This figure demonstrates the hyperthermic intraperitoneal chemotherapy (HIPEC) setup after cytoreductive surgery has been completed. A: HIPEC administered using an open technique; B: After placement of tubes, drains and temperature probes, the skin edges are elevated onto the rim of the self-retaining retractor using a running suture. A plastic sheet covers the abdomen to prevent splashing and loss of chemotherapy agent. A slit in the sheet allows the surgeon to access the abdominal cavity. Continuous mixing by the surgeon ensures all abdominal surfaces are uniformly coated with doses of heated chemotherapy.