Frederic Mercier1, Guillaume Passot1,2, Laurent Villeneuve2,3, Edward A Levine4, Yutaka Yonemura5,6,7,8,9, Diane Goéré10, Paul H Sugarbaker11, Christelle Marolho3, David L Bartlett12, Olivier Glehen13,14. 1. Department of Surgical Oncology, CHU Lyon Sud, Hospices Civils de Lyon, 165 Chemin du Grand Revoyet, 69495, Pierre Bénite, France. 2. EMR 37-38, Lyon 1 University, Lyon, France. 3. Pôle Information Médicale Evaluation Recherche, Hospices Civils de Lyon, Unité de Recherche Clinique, Lyon, France. 4. Section of Surgical Oncology, Department of General Surgery, Wake Forest School of Medicine, Winston-Salem, NC, USA. 5. Asian School of Peritoneal Surface Oncology, 510 Fukushima-Cho, Shimogyou-ku, Kyoto, Japan. 6. NPO to support Peritoneal Surface Malignancy Treatment, 510 Fukushima-Cho, Shimogyou-ku, Kyoto, Japan. 7. Regional Cancer Therapies, Peritoneal Surface Malignancy Center, Kishiwada Tokushukai Hospital, 4-27-1 Kamori-Cho, Kishiwada, Osaka, Japan. 8. Kutatsu General Hospital, 1660 Yabase-Cho, Kusatsu, Shiga, Japan. 9. Ikeda Hospital, 411-5 Honnjyuku, Nagaizumi-cho, Shizuoka, Japan. 10. Department of Surgery, Gustave Roussy, Université Paris-Saclay, Villejuif, France. 11. Center for Gastrointestinal Malignancies, Program in Peritoneal Surface Oncology, MedStar Washington Hospital Center, Washington, DC, USA. 12. Division of Surgical Oncology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA. 13. Department of Surgical Oncology, CHU Lyon Sud, Hospices Civils de Lyon, 165 Chemin du Grand Revoyet, 69495, Pierre Bénite, France. olivier.glehen@chu-lyon.fr. 14. EMR 37-38, Lyon 1 University, Lyon, France. olivier.glehen@chu-lyon.fr.
Abstract
PURPOSE: Peritoneal carcinomatosis or pseudomyxoma peritonei from urachus is a rare form of presentation, often diagnosed at an advanced state of tumor burden. Because of its rarity, little is known about its natural history, prognosis, or optimal treatment. We searched a large international multicenter database of peritoneal surface disease to identify cases of peritoneal carcinomatosis of urachus that were treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) at expert centers. The aim is to improve knowledge and understanding of the disease and standardize its treatment. METHODS: A prospective multicenter international database was retrospectively searched to identify all patients with urachus tumor and peritoneal metastases who underwent CRS and HIPEC through the Peritoneal Surface Oncology Group International (PSOGI). Postoperative complications, long-term results, and principal prognostic factors were analyzed. RESULTS: The analysis included 36 patients. After median follow-up of 48 months, median overall survival (OS) was 58.5 months. Three- and 5-year OS was 55.4 and 46.2%, respectively. Patients who underwent complete macroscopic CRS had significantly better survival than those treated with incomplete CRS, with median OS not achieved and of 20.1 months, respectively [95% confidence interval (CI) 4.4-30.5, p < 0.001]. There were no postoperative deaths, and 37.9% of patients had major complications. CONCLUSION: CRS and HIPEC may increase long-term survival in selected patients with peritoneal metastases of urachus origin, especially when complete CRS is achieved.
PURPOSE: Peritoneal carcinomatosis or pseudomyxoma peritonei from urachus is a rare form of presentation, often diagnosed at an advanced state of tumor burden. Because of its rarity, little is known about its natural history, prognosis, or optimal treatment. We searched a large international multicenter database of peritoneal surface disease to identify cases of peritoneal carcinomatosis of urachus that were treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) at expert centers. The aim is to improve knowledge and understanding of the disease and standardize its treatment. METHODS: A prospective multicenter international database was retrospectively searched to identify all patients with urachus tumor and peritoneal metastases who underwent CRS and HIPEC through the Peritoneal Surface Oncology Group International (PSOGI). Postoperative complications, long-term results, and principal prognostic factors were analyzed. RESULTS: The analysis included 36 patients. After median follow-up of 48 months, median overall survival (OS) was 58.5 months. Three- and 5-year OS was 55.4 and 46.2%, respectively. Patients who underwent complete macroscopic CRS had significantly better survival than those treated with incomplete CRS, with median OS not achieved and of 20.1 months, respectively [95% confidence interval (CI) 4.4-30.5, p < 0.001]. There were no postoperative deaths, and 37.9% of patients had major complications. CONCLUSION: CRS and HIPEC may increase long-term survival in selected patients with peritoneal metastases of urachus origin, especially when complete CRS is achieved.