Jean-Baptiste Delhorme1, Laurent Villeneuve2, Olivier Bouché3, Gerlinde Averous4, Anthony Dohan5, Jean-Marc Gornet6, Benoit You7, Frédéric Bibeau8, Peggy Dartigues9, Clarisse Eveno10, Juliette Fontaine11, Vahan Kepenekian12, Marc Pocard13, Pascal Rousset14, Francois Quenet15, Pascale Mariani16, Olivier Glehen12, Diane Goéré17. 1. Department of general and digestive, Hautepierre Hospital, Strasbourg University Hospital, 1 avenue Molière, 67200 Strasbourg, France. Electronic address: jean-baptiste.delhorme@chru-strasbourg.fr. 2. Department of Public Health, Clinical Research and Epidemiology, Hospices Civils de Lyon, Lyon, France. 3. Department of Digestive Oncology, Reims University Hospital, Reims, France. 4. Department of pathology, Hautepierre Hospital, Strasbourg University Hospital, Strasbourg, France. 5. Department of Abdominal Imaging, Hôpital Lariboisière-APHP, Paris, France. 6. Department of hepato-gastroenteology, Hospital Saint-Louis, Paris, France. 7. Department of Medical Oncology, Institut de Cancérologie des Hospices Civils de Lyon (IC-HCL), Pierre-Bénite, France. 8. Department of pathology, Caen University Hospital, Caen, France. 9. Pathology Department, Gustave Roussy, Villejuif, France. 10. Department of general and digestive, Claude Huriez University Hospital, Lille, France. 11. Depatement of pathology, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite, France. 12. Department of General Surgery and Surgical Oncology, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite, France. 13. Department of general and digestive, Hôpital Lariboisière-APHP, Paris, France. 14. Department of Radiology, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Pierre Bénite, France. 15. Department of Surgery, Institut du Cancer de Montpellier, Montpellier, France. 16. Department of Surgery, Institut Curie, Paris, France. 17. Departement of general and digestive surgery, St Louis University Hospital, Paris, France.
Abstract
INTRODUCTION: This document is a summary of the French Intergroup guidelines regarding the management of appendicular epithelial tumors (AT) and pseudomyxoma peritonei (PMP) published in March 2020, available on the website of the French Society of Gastroenterology (SNFGE) (www.tncd.org). METHODS: All French medical societies specialized in the management of AT and PMP collaboratively established these recommendations based on literature until December 2019 and the results of a Delphi vote carried out by the Peritoneal Surface Oncology Group International experts, and graded into 4 categories (A, B, C, Expert Agreement) according to their level of evidence. RESULTS: AT and PMP are rare but represent a wide range of clinico-pathological entities with several pathological classification systems and different biological behaviors. Their treatment modalities may vary accordingly and range from simple surveillance or laparoscopic appendectomy to complete cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) and / or systemic chemotherapy. The prognosis of these neoplasms may also largely vary according to their pathological grade and spreading at diagnosis or during the follow-up. Given the rarity of certain situations, the therapeutic strategy adapted to each patient, must be discussed in a specialized multidisciplinary meeting after a specialized pathological and radiological pre-therapeutic assessment and a clinical examination by a surgeon specializing in the management of rare peritoneal malignancies. CONCLUSION: These recommendations are proposed to achieve the most beneficial strategy in a daily practice as the wide range and the rareness of these entities renders their management challenging. These guidelines are permanently being reviewed.
INTRODUCTION: This document is a summary of the French Intergroup guidelines regarding the management of appendicular epithelial tumors (AT) and pseudomyxoma peritonei (PMP) published in March 2020, available on the website of the French Society of Gastroenterology (SNFGE) (www.tncd.org). METHODS: All French medical societies specialized in the management of AT and PMP collaboratively established these recommendations based on literature until December 2019 and the results of a Delphi vote carried out by the Peritoneal Surface Oncology Group International experts, and graded into 4 categories (A, B, C, Expert Agreement) according to their level of evidence. RESULTS: AT and PMP are rare but represent a wide range of clinico-pathological entities with several pathological classification systems and different biological behaviors. Their treatment modalities may vary accordingly and range from simple surveillance or laparoscopic appendectomy to complete cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) and / or systemic chemotherapy. The prognosis of these neoplasms may also largely vary according to their pathological grade and spreading at diagnosis or during the follow-up. Given the rarity of certain situations, the therapeutic strategy adapted to each patient, must be discussed in a specialized multidisciplinary meeting after a specialized pathological and radiological pre-therapeutic assessment and a clinical examination by a surgeon specializing in the management of rare peritoneal malignancies. CONCLUSION: These recommendations are proposed to achieve the most beneficial strategy in a daily practice as the wide range and the rareness of these entities renders their management challenging. These guidelines are permanently being reviewed.