Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy to Treat Pseudomyxoma Peritonei of Ovarian Origin: A Retrospective French RENAPE Group Study
Authors: Trecourt Alexis , Bakrin Naoual , Glehen Olivier , Gertych Witold , Villeneuve Laurent , Isaac Sylvie , Benzerdjeb Nazim , Fontaine Juliette , Genestie Catherine , Dartigues Peggy , Leroux Agnès , Quenet François , Marchal Frederic , Odin Cecile , Khellaf Lakhdar , Svrcek Magali , Thierry Sixte , Augros Marilyn , Omar Alhadeedi , Devouassoux-Shisheboran Mojgan , Kepenekian Vahan ,   the RENAPE Group, Abba Julio , Abboud Karine , Aimé Adeline , Amroun Koceila , André Thierry , Arvieux Catherine , Averous-Lang Gerlinde , Bardier Armelle , Rejeb Houda Ben , Bereder Jean-Marc , Bertheau Philippe , Bibeau Frédéric , Boige Valérie , Bonnot Pierre-Emmanuel , Bouché Olivier , Bouhidel Fatiha , Bouzard Marie-Dominique , Brigand Cécile , Broudin Chloé , Celerier Bertrand , Ceribelli Cécilia , Charissoux Aurélie , Chevallier Anne , Clément Elise , Coget Julien , Courvoisier-Clément Thomas , Dazza Marie , de Chaisemartin Cécile , Di Fiore Frédéric , Dumont Frédéric , Durand-Fontanier Sylvaine , Eveno Clarisse , Ezanno Anne-Cécile , Facy Olivier , Ferron Gwenaël , Gagnière Johann , Galan Alexandre , Gelli Maximiliano , Ghouti Laurent , Gladieff Laurence , Goere Diane , Guilloit Jean-Marc , Guyon Frédéric , Heyd Bruno , Heymann Marie-Françoise , Hübner Martin , Illac-Vauquelin Claire , Kaci Rachid , Kefleysus Amaniel , Kepenekian Vahan , Kianmanesh Reza , Laverrière Marie-Hélène , Lebrun-Ly Valérie , Lefevre Jérémie H. , Lelong Bernard , Lemaistre Anne-Isabelle , Malgras Brice , Mariani Pascale , Mariani Antoine , Meeus Pierre , Mery Eliane , Narducci Fabrice , Nougaret Stéphanie , Orry David , Ortega-Deballon Pablo , Paquette Brice , Péron Julien , Peyrat Patrice , Pezet Denis , Pirro Nicolas , Pocard Marc , Poizat Flora , Raimbourg Judith , Rat Patrick , Ries Pauline , Rousset Pascal , Sage Pierre-Yves , Senellart Hélène , Sgarbura Olivia , Smolenschi Cristina , Sourrouille Isabelle , Taibi Abdelkader , Tessier Williams , Thibaudeau Emilie , Touchefeu Yann , Trilling Bertrand , Tuech Jean-Jacques , Valmary-Degano Séverine , Varatharajah Sharmini , Verriele-Beurrier Véronique , Vogin Guillaume , Wernert Romuald , You Benoit 
Published: February 2024

Abstract
Background
Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) to treat OPMP.

Methods
Patients from the French National Network for Rare Peritoneal Tumors (RENAPE) database with proven OPMP treated by CRS/HIPEC and with histologically normal appendix and digestive endoscopy were retrospectively included. Clinical and follow-up data were collected. Histopathological and immunohistochemical features were reviewed.

Results
Fifteen patients with a median age of 56 years were included. The median Peritoneal Cancer Index was 16. Following CRS, the completeness of cytoreduction (CC) score was CC-0 for 9/15 (60%) patients, CC-1 for 5/15 (33.3%) patients, and CC-2 for 1/15 (6.7%) patients. The median tumor size was 22.5 cm. After pathological review and immunohistochemical studies, tumors were classified as Group 1 (mucinous ovarian epithelial neoplasms) in 3/15 (20%) patients; Group 2 (mucinous neoplasm in ovarian teratoma) in 4/15 (26.7%) patients; Group 3 (mucinous neoplasm probably arising in ovarian teratoma) in 5/15 (33.3%) patients; and Group 4 (non-specific group) in 3/15 (20%) patients. Peritoneal lesions were OPMP pM1a/acellular, pM1b/grade 1 (hypocellular) and pM1b/grade 3 (signet-ring cells) in 13/15 (86.7%), 1/15 (6.7%) and 1/15 (6.7%) patients, respectively. Disease-free survival analysis showed a difference (p = 0.0463) between OPMP with teratoma/likely-teratoma origin (groups 2 and 3; 100% at 1, 5, and 10 years), and other groups (groups 1 and 4; 100%, 66.6%, and 50% at 1, 5, and 10 years, respectively).

Conclusion
These results suggested that a primary therapeutic strategy using complete CRS/HIPEC for patients with OPMP led to favorable long-term outcomes.