Pathological prognostic factors of pseudomyxoma peritonei: comprehensive clinicopathological analysis of 155 cases

Hum Pathol. 2020 Mar;97:9-18. doi: 10.1016/j.humpath.2019.12.008. Epub 2020 Jan 9.

Abstract

Background: Pseudomyxoma peritonei (PMP) is an extremely rare malignancy, characterized by extensive peritoneal implantation and colloidal ascites. This study was to explore the pathological prognostic factors of PMP.

Methods: Specimens from 155 PMP patients were analyzed by H&E and immunohistochemistry. Parameters included primary tumor location, histological grade, lymph node metastasis, tumor emboli in the blood and lymph vessels, perineural invasion, Ki67 labeling index, p53, mismatch repair (MMR) gene mutations, MUC1, MUC2, MUC5AC, and MUC6. Clinicopathological and follow-up data were subjected to univariate and multivariate analyses.

Results: The patients included 63.2% (n = 98) low-grade mucinous carcinoma peritonei, 31.6% (n = 49) high-grade mucinous carcinoma peritonei and 5.2% (n = 8) high-grade mucinous carcinoma peritonei with signet ring cells. There were 9.7% (n = 15) with lymph node metastasis; 11.6% (n = 18) with angiolymphatic invasion; 6.3% (n = 8) with defective MMR (dMMR); 35.5% (n = 55) with Ki67 labeling index ≥ 50%; 36.1% (n = 56) with p53 mutation. For PMP from appendiceal origin (n = 140), univariate analysis identified 10 potential prognostic factors. But Multivariate analysis identified only histologic grade was the independent prognostic factor for OS. Mortality risk of high-grade peritoneal mucinous carcinoma or high-grade peritoneal mucinous carcinoma with signet ring cells was 7.056 times (P < .0001, 95% CI: 2.701-18.435) or 27.224 times (P < .0001, 95% CI: 6.207-119.408), respectively, higher than low-grade.

Conclusions: For PMP from the appendiceal origin, histological grade could be the only independent prognostic factor.

Keywords: Appendiceal neoplasm; Mismatch repair genes; Pathological grading; Prognosis; Pseudomyxoma peritonei.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics
  • DNA Mismatch Repair
  • Female
  • Humans
  • Lymphatic Metastasis
  • Male
  • Middle Aged
  • Mutation
  • Neoplasm Grading
  • Neoplastic Cells, Circulating / pathology
  • Peritoneal Neoplasms / chemistry
  • Peritoneal Neoplasms / genetics
  • Peritoneal Neoplasms / mortality
  • Peritoneal Neoplasms / pathology*
  • Pseudomyxoma Peritonei / genetics
  • Pseudomyxoma Peritonei / metabolism
  • Pseudomyxoma Peritonei / mortality
  • Pseudomyxoma Peritonei / pathology*
  • Risk Assessment
  • Risk Factors

Substances

  • Biomarkers, Tumor