Analysis of Clinical Outcomes of Pseudomyxoma Peritonei from Appendicular Origin Following Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy-A Retrospective Study from INDEPSO

Abstract

To evaluate the clinical outcomes of patients of pseudomyxoma peritonei of appendiceal origin undergoing cytoreductive surgery and HIPEC. Data collected from members, an independent collaborative group of Indian surgeons specializing in the management of peritoneal surface malignancy (INDEPSO), was analyzed retrospectively. Clinicopathological and perioperative outcomes of patients treated for pseudomyxoma peritonei (PMP) of appendicular origin were evaluated. Ninety-one patients were diagnosed with pseudomyxoma peritonei of appendicular origin between March 2013 and December 2017. The median age was 53 years and 60% were females. The median PCI was 27 [range 3-39] and a CC-0/1 resection was achieved in 83.5% patients. The most common histological grade was low-grade PMP, seen in 71.4% cases. The overall rate of grades 3-4 morbidity was 33% (30/91) and the 90-day mortality rate reported was 6.5%. Pulmonary complications and systemic sepsis emerged as the most significant factors affecting morbidity, mortality, and failure to rescue. At a median follow-up of 24 months, the median OS was not reached and the median PFS was 53 months. On univariate and multivariate analysis, high-grade histology, prior chemotherapy, debulking surgery alone without HIPEC, and high PCI > 10 were predictors of poor progression-free survival. The survival and morbidity results of pseudomyxoma peritonei from appendicular origin following cytoreductive surgery and hyperthermic intraperitoneal chemotherapy are encouraging. With further awareness and understanding of the disease, and improvement in surgical expertise and learning curve, there is scope for further reduction in morbidity and better improvement in survival.

Indications for Hyperthermic Intraperitoneal Chemotherapy with Cytoreductive Surgery: A Clinical Practice Guideline

“…The retrospective study by Sinukumar et al 32 showed that the use of hipec was not associated with os, but was independently associated with increased pfs (hr: not reported; 95% ci: 1.26 to 9.8; p = 0.016).…”

“…To date, no randomized studies have compared the use of crs-hipec with other methods of oncologic management in patients with disseminated mucinous neoplasms. The evidence comes from one comparative study 31 that assessed the differences between patients treated during the debulking era (n = 33) and during the crs plus hipec era (n = 87), and four retrospective cohort studies [32][33][34][35] that conducted multivariable analyses including the use of crs plus hipec as a variable. The certainty of this evidence is very low.…”

The Prognostic Impact of Pathology on Patients With Pseudomyxoma Peritonei Undergoing Debulking Surgery: A Systematic Review and Meta-Analysis of Retrospective Studies

“…Sugarbaker et al ( 8 ) first introduced complete cytoreductive surgery (CRS) plus heated intraoperative intraperitoneal chemotherapy (HIPEC), which provided new ideas for PMP treatment and has been suggested as the standard therapy ( 9 ). However, a portion of patients (16.5–25.8%) ( 10 , 11 ) were no longer suitable for radical surgery at the time of diagnosis due to the huge burden of PMP; for example, the massive disease invades the first porta hepatis or the caudate lobe, which is difficult to be cleaned, and the proximal third of the stomach and small bowel are involved, which would lead the length of the remnant intestines to be shorter than 2 m. For these patients, who represent a group with highly advanced PMP, the next best choice is debulking surgery ( 12 ). The definition of debulking surgery is a surgical technique that is done when the macroscopic tumor in the abdominal cavity cannot be removed.…”

Ultrasound for Preoperatively Predicting Pathology Grade, Complete Cytoreduction Possibility, and Survival Outcomes of Pseudomyxoma Peritonei

“…Recent studies ( 24 , 25 ) have indicated that preoperative imaging techniques, such as CT and magnetic resonance imaging (MRI), could predict the resectability of PMP. Additionally, some US manifestations were significant prognostic factors for the resectability in PMP cases.…”

“…It was difficult to clear all the hepatoduodenal and hepatogastric ligament tumors which severely affected the outcome of surgery. It demonstrated that the presence of residual unresectable disease in porta hepatis may influence the prognosis ( 25 ) and might explain why the involvement of the porta hepatis predicted PMP outcomes. The evaluation of porta hepatis using two-dimensional US has previously been reported to be associated with the unresectability of PMP; however, to the best of our knowledge, no study has reported its predictive role for CC score and survival rates.…”