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Annals of Surgical Oncology

, Volume 25, Issue 4, pp 1094–1100 | Cite as

Peritoneal Carcinomatosis of Urachus Origin Treated by Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC): An International Registry of 36 Patients

  • Frederic Mercier
  • Guillaume Passot
  • Laurent Villeneuve
  • Edward A. Levine
  • Yutaka Yonemura
  • Diane Goéré
  • Paul H. Sugarbaker
  • Christelle Marolho
  • David L. Bartlett
  • Olivier GlehenEmail author
  • BIG-RENAPE Working Group
Urologic Oncology

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.

Notes

Acknowledgement

The authors thank Peggy Jourdan-Enfer and Anaïs Poulet for their expert help with data collection. The authors thank Lorna Saint Ange for help with editing the manuscript, and Evelyne Decullier for help with data analysis.

The collaborators of the BIG-RENAPE Working Group include the following: J. Abba (Department of Surgical Oncology, CHU Grenoble University, Grenoble, France); K. Abboud (Department of Surgical Oncology, CHU St Etienne, St Etienne, France); M. Alyami (Department of Surgical Oncology, Centre Hospitalier Lyon Sud - EMR 3738, Lyon 1 University, Lyon, France); C. Arvieux (Department of Surgical Oncology, CHU Grenoble University, Grenoble, France); N. Bakrin (Department of Surgical Oncology, Centre Hospitalier Lyon Sud - EMR 3738, Lyon 1 University, Lyon, France); J.-M. Bereder (Department of Surgical Oncology, CHU L’Archet 2, Nice, France); D. Bouzard (Department of Surgical Oncology, CHU Louis Mourier, Colombes, France); C. Brigand (Department of Surgical Oncology, CHRU Hautepierre, Strasbourg, France); S. Carrère (Department of Surgical Oncology, Institut du Cancer de Montpellier, Montpellier, France); D. Delroeux (Department of Surgical Oncology, CHU Jean Minjoz, Besançon, France); F. Dumont (Department of Surgical Oncology, ICO - René Gauducheau, St Herblain, France); C. Eveno (Department of Surgical Oncology, CHU Lariboisière, Paris, France); O. Facy (Department of Surgical Oncology, CHU Dijon, Dijon, France); F. Guyon (Department of Surgical Oncology, Institut Bergonié, Bordeaux, France); G. Ferron (Department of Surgical Oncology, IUCT Oncopole, Toulouse, France); R. Kianmanesh (Department of Surgical Oncology, CHU Robert Debré, Reims, France); R. Lo Dico (Department of Surgical Oncology, CHU Lariboisière, Paris, France); G. Lorimier (Department of Surgical Oncology, CHU Angers, Angers, France); F. Marchal (Department of Surgical Oncology, Institut de Cancérologie de Lorraine, Vandoeuvre-lès-Nancy, France); P. Mariani (Department of Surgical Oncology, Institut Curie, Paris, France); P. Meeus (Department of Surgical Oncology, Centre Léon Bérard, Lyon, France); S. Msika (Department of Surgical Oncology, CHU Louis Mourier, Colombes, France); P. Ortega-Deballon (Department of Surgical Oncology, CHU Dijon, Dijon, France); B. Paquette (Department of Surgical Oncology, CHU Jean Minjoz, Besançon, France); P. Peyrat (Department of Surgical Oncology, Centre Léon Bérard, Lyon, France); N. Pirro (Department of Surgical Oncology, CHU La Timône, Marseille, France); M. Pocard (Department of Surgical Oncology, CHU Lariboisière, Paris, France); J. Porcheron (Department of Surgical Oncology, CHU St Etienne, St Etienne, France); F. Quenet (Department of Surgical Oncology, Institut du Cancer de Montpellier, Montpellier, France); P. Rat (Department of Surgical Oncology, CHU Dijon, Dijon, France); O. Sgarbura (Department of Surgical Oncology, Institut du Cancer de Montpellier, Montpellier, France); E. Thibaudeau (Department of Surgical Oncology, ICO - René Gauducheau, St Herblain, France); J.-J. Tuech (Department of Surgical Oncology, CHU Charles Nicolle, Rouen, France); F. Zinzindohoue (Department of Surgical Oncology, Hôpital Européen Georges Pompidou, Paris, France).

The collaborators of the PSOGI Working Group include the following: S. A. Ahrendt (Department of Surgery, University of Pittsburgh Medical Center Shadyside Hospital, Pittsburgh, USA); E. Akaishi (Department of Surgical Oncology, Centro de Oncologia Hospital Sirio Libanes, Sao Paolo, Brazil); S. H. Baik (Department of Surgery, Gangnam Severance Hospital - Yonsei University College of Medicine, Seoul, Korea); D. Baratti (Department of Gastrointestinal Surgery, San Raffaele Scientific Institute, Milan, Italy); A. Bhatt (Department of Surgical Oncology, Fortis Hospitals Limited, Bangalore, India); P. Cachin (Department of Surgery, Akademiska sjukhuset, Uppsala University Hospital, Uppasala, Sweden); W. Ceelen (Department of Gastrointestinal Surgery, Gent University Hospital, Ghent, Belgium); I. De Hingh (Department of Surgery, Catharina Ziekenhuis, Eindhoven, The Netherlands); M. De Simone (Department of Surgical Oncology, Candiolo Cancer Institute - FPO, IRCCS, Turin, Italy); P. Dubé (Department of Surgery, University of Montreal, Montreal, Canada); R. P. Edwards (Department of Surgery, University of Pittsburgh Medical Center Shadyside Hospital, Pittsburgh, USA); J. Franko (Department of Surgical Oncology, Mercy Medical Center, Baltimore, USA); L. Gonzalez-Bayon (Department of Surgical Oncology, Hospital Gregorio Marañón, Madrid, Spain); V. Gushchin (Department of Surgical Oncology, Mercy Medical Center, Baltimore, USA); M. P. Holtzman (Department of Surgery, University of Pittsburgh Medical Center Shadyside Hospital, Pittsburgh, USA); M.-C. Hsieh (Department of General Surgery, Wan-Fang Hospital, Taipei, Taiwan); D. Kecmanovic (Department of Surgery, First Surgical Clinic, Clinical Center of Serbia, Belgrade, Serbia); K. W. Lee (Department of Surgery, University of Pittsburgh Medical Center Shadyside Hospital, Pittsburgh, USA); K. Lehmann (Department of Surgery and Transplantation, University Hospital of Zurich, Zurich, Switzerland); Y. Liu (NPO to Support Peritoneal Surface Malignancy Treatment, Kyoto, Japan); S. Mehta (Division of Peritoneal Surface Oncology, Saifee Hospital, Mumbai, India); D. L. Morris (Department of Surgery, University of New South Wales, Sydney, Australia); S. O’Dwyer (Department of Colorectal Surgery, Christie Cancer Center, Manchester, UK); E. Orsenigo (Department of Gastrointestinal Surgery, San Raffaele Scientific Institute, Milan, Italy); P. K. Pande (Department of Surgical Oncology, BLK Superspeciality Hospital, New Delhi, India); E. J. Park (Department of Surgery, Gangnam Severance Hospital - Yonsei University College of Medicine, Seoul, Korea); J. F. Pingpank (Department of Surgery, University of Pittsburgh Medical Center Shadyside Hospital, Pittsburgh, USA); P. Piso (Department of Surgery, University of Regensburg, Regensburg, Germany); F. Rajan (Department of Surgical Oncology, Kovai Medical Centre, Coimbatore, India); B. Rau (Department of Surgical Oncology, Charite Campus Mitte University of Berlin, Berlin, Germany); A. Sardi (Department of Surgical Oncology, Mercy Medical Center Baltimore, USA); L. Sideris (Department of Surgery, University of Montreal, Montreal, Canada); A. Sommariva (Melanoma and Sarcoma Unit, Istituto Oncologico Veneto, Padua, Italy); J. Spiliotis (First Department of Surgical Oncology, Metaxa Cancer Memorial Hospital, Piraeus, Greece); A. A. K. Tentes (Department of Surgery, Metropolitan Hospital, Athens, Greece); M. Teo (Department of Surgical Oncology, National Cancer Centre Singapore, Singapore, Singapore); R. Yarema (Department of Oncology and Medical Radiology Danylo Halytsky Lviv National Medical University, Lviv, Ukraine); R. Younan (Department of Surgery, Centre Hospitalier de l’Université de Montréal, Montreal, Canada); S. S. Zaveri (Department of Surgical Oncology, Manipal Hospital, Bangalore, India); H. J. Zeh (Department of Surgery, University of Pittsburgh Medical Center, Shadyside Hospital, Pittsburgh, USA).

Disclosure

The authors report no conflicts of interest relevant to this article.

References

  1. 1.
    Agrawal AK, Bobinski P, Grzebieniak Z, et al. Pseudomyxoma peritonei originating from urachus–case report and review of the literature. Curr Oncol. 2014; 21:155–65.CrossRefGoogle Scholar
  2. 2.
    Liu Y, Ishabashi H, Hiranu M, et al. Cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei originating from urachus. Ann Surg Oncol. 2015; 22:2799–805.CrossRefPubMedGoogle Scholar
  3. 3.
    Mendeloff J, McSwain NE Jr. Pseudomyxoma peritonei due to mucinous adenocarcinoma of the urachus. Southern Med J. 1971;64:497–98.CrossRefPubMedGoogle Scholar
  4. 4.
    Loggie BW, Fleming RA, Hosseinian AA. Peritoneal carcinomatosis with urachal signet-cell adenocarcinoma. Urology. 1997;50:446–8.CrossRefPubMedGoogle Scholar
  5. 5.
    Sugarbaker PH, Verghese M, Yan TD, Brun E. Management of mucinous urachal neoplasm presenting as pseudomyxoma peritonei. Tumori. 2008; 94:732–736.CrossRefPubMedGoogle Scholar
  6. 6.
    Stenhouse G, McRae D, Pollock AM. Urachal adenocarcinoma in situ with pseudomyxoma peritonei: a case report. J Clin Pathol. 2003; 56:152–3.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Yanagisawa S, Fujinaga Y, Kadoya M. Urachal mucinous cystadenocarcinoma with a cystic ovarian metastasis. AJR. 2003; 180:1183–4.CrossRefPubMedGoogle Scholar
  8. 8.
    Sasano H, Shizawa S, Naguro H, Yamaki T. Mucinous adenocarcinoma arising in a giant urachal cyst associated with pseudomyxoma peritonei and stromal osseus metaplasia. Pathol Int. 1997; 47:502–5.CrossRefPubMedGoogle Scholar
  9. 9.
    de Bree E, Witkamp A, Van De Vijver M, et al. Unusual origins of pseudomyxoma peritonei. J Surg Oncol. 2000; 75:270–4.CrossRefPubMedGoogle Scholar
  10. 10.
    Carr NJ, Finch J, Ilesley IC, et al. Pathology and prognosis in pseudomyxoma peritonei:a review of 274 cases. J Clin Pathol. 2012; 65:919–23.CrossRefPubMedGoogle Scholar
  11. 11.
    Takeuchi M, Matsuzaki K, Yoshida S, et al. Imaging findings of urachal mucinous cystadenocarcinoma associated with pseudomyxoma peritonei. Acta Radiol. 2004; 45:348–50.CrossRefPubMedGoogle Scholar
  12. 12.
    Passot G, Vaudoyer D, Villeneuve L, et al. What made hyperthermic intraperitoneal chemotherapy an effective curative treatment for peritoneal surface malignancy: a 25-year experience with 1125 procedures. J Surg Oncol. 2016; 113:796–803.CrossRefPubMedGoogle Scholar
  13. 13.
    Ansari N, Chandrakumaran K, Dayal S, et al. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in 1000 patients with perforated appendiceal epithelial tumours. Eur J Surg Oncol. 2016; 42:1035–41.CrossRefPubMedGoogle Scholar
  14. 14.
    Jacquet P, Sugarbaker PH. Current methodologies for clinical assessment of patients with peritoneal carcinomatosis. J Exp Clin Cancer Res. 1996; 15:49–58.Google Scholar
  15. 15.
    Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res. 1996; 82:359–74.CrossRefPubMedGoogle Scholar
  16. 16.
    Glehen O, Mohammed F and Gilly FN. Peritoneal carcinomatosis from digestive tract cancer: new management by cytoreductive surgery and intraperitoneal chemohyperthermia. Lancet Oncol. 2004; 5:219–28.CrossRefPubMedGoogle Scholar
  17. 17.
    Common Terminology Criteria for Adverse Events (CTCAE) Version 4.0. https://evs.nci.nih.gov/ftp1/CTCAE/CTCAE_4.03_2010-06-14.
  18. 18.
    Chua TC, Moran BJ, Sugarbaker PH, et al. Early and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Clin Oncol. 2012; 30:2449–56.CrossRefPubMedGoogle Scholar
  19. 19.
    Elias D, Mariani A, Cloutier AS et al. Modified selection criteria for complete cytoreductive surgery plus HIPEC based on peritoneal cancer index and small bowel involvement for peritoneal carcinomatosis of colorectal origin. Eur J Surg Oncol. 2014; 40:1467-73.CrossRefPubMedGoogle Scholar
  20. 20.
    Van Sweringen HL, Hanseman DJ, Ahmad SA, et al. Predictors of survival in patients with high-grade peritoneal metastases undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Surgery. 2012; 152:617–24.CrossRefPubMedGoogle Scholar
  21. 21.
    Baumgartner JM, Tobin L, Heavey SF, et al. Predictors of progression in high-grade appendiceal or colorectal peritoneal carcinomatosis after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2015; 22:1716–21.CrossRefPubMedGoogle Scholar
  22. 22.
    El Halabi H, Gushchin V, Francis J, et al. The role of cytoreductive surgery and heated intraperitoneal chemotherapy (CRS/HIPEC) in patients with high-grade appendiceal carcinoma and extensive peritoneal carcinomatosis. Ann Surg Oncol. 2012; 19:110–4.CrossRefPubMedGoogle Scholar
  23. 23.
    Miner TJ, Shia J, Jacques DP, et al. Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy. Ann Surg. 2005; 241:300–8.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Szarvas T, Modos O, Niedworok C, et al. Clinical, prognostic, and therapeutic aspect of urachal carcinoma-a comprehensive review with meta-analysis of 1010 cases. Urol Oncol. 2016; 34:388–98.CrossRefPubMedGoogle Scholar
  25. 25.
    Bruins HM, Visser O, Ploeg M, et al. The clinical epidemiology of urachal carcinoma: results of a large population based study. J Urol. 2012; 188:1102–7.CrossRefPubMedGoogle Scholar
  26. 26.
    Siefer-Radtke AO, Gee J, Shen Y, et al. Multimodality management of urachal carcinoma: the M.D. Anderson cancer center experience. J Urol. 2003; 169:1295–8.CrossRefGoogle Scholar
  27. 27.
    Niedworok C, Panitz M, Szarvas T, et al. Urachal carcinoma of the bladder –impact of clinical and immunological parameters on patients’ prognosis. J Urol. 2016; 195:1690–6.CrossRefPubMedGoogle Scholar
  28. 28.
    Ashley RA, Inman Ba, Sebo TJ, et al. Urachal carcinoma: clinicopathologic features and log-term outcomes of an aggressive malignancy. Cancer. 2006; 15:712–20.CrossRefGoogle Scholar
  29. 29.
    Kim IK, Lee JY, Kwon JK, et al. Prognostic factors for urachal cancer: a Bayesian model-averaging approach. Korean J Urol. 2014; 55:574–80.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Society of Surgical Oncology 2018

Authors and Affiliations

  • Frederic Mercier
    • 1
  • Guillaume Passot
    • 1
    • 2
  • Laurent Villeneuve
    • 2
    • 3
  • Edward A. Levine
    • 4
  • Yutaka Yonemura
    • 5
    • 6
    • 7
    • 8
    • 9
  • Diane Goéré
    • 10
  • Paul H. Sugarbaker
    • 11
  • Christelle Marolho
    • 3
  • David L. Bartlett
    • 12
  • Olivier Glehen
    • 1
    • 2
    Email author
  • BIG-RENAPE Working Group
  1. 1.Department of Surgical OncologyCHU Lyon Sud, Hospices Civils de LyonPierre BéniteFrance
  2. 2.EMR 37-38Lyon 1 UniversityLyonFrance
  3. 3.Pôle Information Médicale Evaluation RechercheHospices Civils de Lyon, Unité de Recherche CliniqueLyonFrance
  4. 4.Section of Surgical Oncology, Department of General SurgeryWake Forest School of MedicineWinston-SalemUSA
  5. 5.Asian School of Peritoneal Surface OncologyKyotoJapan
  6. 6.NPO to support Peritoneal Surface Malignancy TreatmentKyotoJapan
  7. 7.Regional Cancer TherapiesPeritoneal Surface Malignancy Center, Kishiwada Tokushukai HospitalKishiwadaJapan
  8. 8.Kutatsu General HospitalKusatsuJapan
  9. 9.Ikeda HospitalNagaizumi-choJapan
  10. 10.Department of Surgery, Gustave RoussyUniversité Paris-SaclayVillejuifFrance
  11. 11.Center for Gastrointestinal Malignancies, Program in Peritoneal Surface OncologyMedStar Washington Hospital CenterWashingtonUSA
  12. 12.Division of Surgical OncologyUniversity of Pittsburgh Medical CenterPittsburghUSA

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