Marcello Deraco1, Eran Nizri2, Olivier Glehen3, Dario Baratti4, Jean-Jacques Tuech5, Jean-Marc Bereder6, Vahan Kepenekian3, Shigeki Kusamura4, Diane Goere7. 1. Peritoneal Surface Malignancies Unit, Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: marcello.deraco@istitutotumori.mi.it. 2. Peritoneal Surface Malignancies Unit, Istituto Nazionale dei Tumori, Milan, Italy; Department of Surgery A, Tel- Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel. 3. Department of Surgical Oncology, Centre Hospitalier Lyon Sud, Pierre-Benite, France. 4. Peritoneal Surface Malignancies Unit, Istituto Nazionale dei Tumori, Milan, Italy. 5. Charles Nicolle University Hospital, Rouen, France. 6. L'Archet II, University Hospital, Nice, France. 7. Gustave Roussy Institute, Villejuif, France.
Abstract
BACKGROUND: Well differentiated papillary peritoneal mesothelioma (WDPPM) is a rare variant of mesothelioma which affects mainly women in the reproductive age. The disease may present multifocally and recur after primary resection. Our aim was to describe the outcomes of cytoreduction (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in this disease. METHODS: Patients with histological diagnosis of WDPPM were retrieved from the PSOGI registry. Demographical and clinical data were extracted as well as outcomes data (overall survival (OS) and recurrence free survival (RFS)). RESULTS: We analyzed 45 patients for whom complete data was available. The majority of patients were women (n = 33, 73%) with a median age of 44 years. Preoperative chemotherapy (CT) was administered in 8 patients (18%). Median peritoneal carcinomatosis index was 9 (1-30), and complete cytoreduction was achieved in 69% of patients. There was one case (2%) of postoperative mortality, and 24% rate of severe morbidity. Overall, there were 4 deaths and 5 years OS was 80%. 8 patients (18%) had disease recurrence, all within 5 years from operation. On univariate analysis preoperative CT, high PCI and severe morbidity were associated with reduced RFS. On multivariate analysis, only preoperative CT (HR = 32.6, 95% CI: 2.39-446.2, p = 0.009) and high PCI (HR = 21.7, 95% CI: 1.11-425.7, p = 0.04) remained significant risk factors. CONCLUSIONS: WDPPM can be a lethal disease with substantial recurrence even after aggressive treatment. Patients presenting with extensive disease or disease recurrence after surgical excision are at increased risk for relapse. CRS + HIPEC can be safely applied to WDPPM in specialized centers.
BACKGROUND: Well differentiated papillary peritoneal mesothelioma (WDPPM) is a rare variant of mesothelioma which affects mainly women in the reproductive age. The disease may present multifocally and recur after primary resection. Our aim was to describe the outcomes of cytoreduction (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in this disease. METHODS:Patients with histological diagnosis of WDPPM were retrieved from the PSOGI registry. Demographical and clinical data were extracted as well as outcomes data (overall survival (OS) and recurrence free survival (RFS)). RESULTS: We analyzed 45 patients for whom complete data was available. The majority of patients were women (n = 33, 73%) with a median age of 44 years. Preoperative chemotherapy (CT) was administered in 8 patients (18%). Median peritoneal carcinomatosis index was 9 (1-30), and complete cytoreduction was achieved in 69% of patients. There was one case (2%) of postoperative mortality, and 24% rate of severe morbidity. Overall, there were 4 deaths and 5 years OS was 80%. 8 patients (18%) had disease recurrence, all within 5 years from operation. On univariate analysis preoperative CT, high PCI and severe morbidity were associated with reduced RFS. On multivariate analysis, only preoperative CT (HR = 32.6, 95% CI: 2.39-446.2, p = 0.009) and high PCI (HR = 21.7, 95% CI: 1.11-425.7, p = 0.04) remained significant risk factors. CONCLUSIONS: WDPPM can be a lethal disease with substantial recurrence even after aggressive treatment. Patients presenting with extensive disease or disease recurrence after surgical excision are at increased risk for relapse. CRS + HIPEC can be safely applied to WDPPM in specialized centers.
Authors: Alberto M Marchevsky; Andras Khoor; Ann E Walts; Andrew G Nicholson; Yu Zhi Zhang; Victor Roggli; John Carney; Anja C Roden; Henry D Tazelaar; Brandon T Larsen; Nolwenn LeStang; Lucian R Chirieac; Sonja Klebe; Ming-Sound Tsao; Marc De Perrot; Andrew Pierre; David M Hwang; Yin P Hung; Mari Mino-Kenudson; William Travis; Jennifer Sauter; Mary Beth Beasley; Françoise Galateau-Sallé Journal: Mod Pathol Date: 2019-09-04 Impact factor: 7.842