Well-Differentiated Papillary Mesothelioma of the Peritoneum: A Retrospective Study from the RENAPE Observational Registry

Ann Surg Oncol. 2019 Mar;26(3):852-860. doi: 10.1245/s10434-018-07153-2. Epub 2019 Jan 11.


Background: Well-differentiated papillary mesothelioma of the peritoneum (WDPMP) is a rare entity. Questions regarding management are still being debated as no more than 50 cases have been reported in the literature.

Objective: We aimed to analyze the clinical, therapeutic, and prognostic data of patients with WDPMP from the RENAPE observational registry.

Patients and methods: All patients diagnosed with WDPMP and prospectively included in the RENAPE national registry between 2010 and 2018 were also included in our study. Expert pathologists from the RENA-PATH group confirmed all cases. All clinical, therapeutic, postoperative, and prognostic data were extracted and analyzed.

Results: We report on 56 patients with a mean age of 52 years (range 21-74). WDPMP was incidentally diagnosed during imaging or surgery in 16% and 36% of patients, respectively, and an association with synchronous malignancy was found in 18% of patients. Nine lesions showed discrete signs of fatty invasion. The median Peritoneal Cancer Index was 11 (range 0-33). Eleven patients were treated with definitive excision, 4 were treated with cytoreductive surgery (CRS) only, 37 were treated with CRS and hyperthermic intraperitoneal chemotherapy (HIPEC), and 2 were treated with CRS plus HIPEC plus early postoperative intraperitoneal chemotherapy. CRS was considered to be complete in 90% of cases. One patient died postoperatively and 16 patients (31%) faced postoperative complications. The median disease-free survival was 144 months; Four patients relapsed, with a median period of 27 months. No prognostic factors could be identified.

Conclusions: Our analysis confirms the favorable prognosis of WDPMP. CRS and HIPEC could be a therapeutic option for diffuse, symptomatic, and/or recurrent disease.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Aged
  • Carcinoma, Papillary / mortality*
  • Carcinoma, Papillary / pathology
  • Carcinoma, Papillary / therapy
  • Chemotherapy, Cancer, Regional Perfusion / mortality*
  • Combined Modality Therapy
  • Cytoreduction Surgical Procedures / mortality*
  • Female
  • Follow-Up Studies
  • Humans
  • Hyperthermia, Induced / mortality*
  • Male
  • Mesothelioma / mortality*
  • Mesothelioma / pathology
  • Mesothelioma / therapy
  • Middle Aged
  • Peritoneal Neoplasms / mortality*
  • Peritoneal Neoplasms / pathology
  • Peritoneal Neoplasms / therapy
  • Prognosis
  • Registries / statistics & numerical data*
  • Retrospective Studies
  • Survival Rate
  • Young Adult