Guillaume Vogin1,2, Liza Hettal3, Jean-Michel Vignaud4, Peggy Dartigues5, Diane Goere6, Gwenaël Ferron7, Bruno Heyd8, Jean-Marc Bereder9, Jean-Jacques Tuech10, Olivier Glehen11,12,13, Cécile de Chaisemartin14, Yoann Lherm12,15, Laurent Villeneuve12,15,13, Vahan Kepenekian11, Frédéric Marchal16,17. 1. Department of Radiation Oncology, Institut de Cancérologie de Lorraine, Vandoeuvre-les-Nancy, France. g.vogin@nancy.unicancer.fr. 2. UMR 7365 CNRS-Université de Lorraine, IMoPA, Vandoeuvre-les-Nancy, France. g.vogin@nancy.unicancer.fr. 3. UMR 7365 CNRS-Université de Lorraine, IMoPA, Vandoeuvre-les-Nancy, France. 4. Department of Pathology, Centre Hospitalier Universitaire de Nancy, Vandoeuvre-les-Nancy, France. 5. Department of Pathology, Gustave Roussy Institute, Villejuif, France. 6. Department of Surgery, Gustave Roussy Institute, Villejuif, France. 7. Department of Surgical Oncology, Institut Universitaire du Cancer de Toulouse - Oncopole, Toulouse, France. 8. Department of Surgical Oncology, Jean Minjoz University Hospital, Besançon, France. 9. Department of Surgical Oncology, L'Archet II University Hospital, Nice, France. 10. Department of Surgical Oncology, Charles Nicolles University Hospital, Rouen, France. 11. Department of Digestive Surgery, Centre Hospitalier Lyon Sud, Pierre-Bénite, France. 12. RENAPE, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Pierre-Bénite, France. 13. EMR 3738, Lyon 1 University, Lyon, France. 14. Department of Surgical Oncology, Paoli Calmettes Institute, Marseille, France. 15. Unité de Recherche Clinique, Pôle de Santé Publique, Hospices Civils de Lyon, Pierre-Bénite, France. 16. Department of Surgery, Institut de Cancérologie de Lorraine, Vandoeuvre-les-Nancy, France. 17. UMR 7039, CNRS-Université de Lorraine, CRAN, Vandoeuvre-les-Nancy, France.
Abstract
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.
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.
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