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Managing Recurrent Pseudomyxoma Peritonei in 430 Patients After Complete Cytoreduction and HIPEC: A Dilemma for Patients and Surgeons

  • Peritoneal Surface Malignancy
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Epithelial appendiceal neoplasms are uncommon peritoneal malignancies causing a spectrum of disease including pseudomyxoma peritonei (PMP). The optimal management is cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). Despite complete CRS (CCRS), recurrence develops in almost 45% of patients. No consensus exists for the optimal treatment of recurrent disease, with treatment strategies including repeat CRS, watch-and-wait, and palliative chemotherapy. This report aims to describe evolving management strategies for a large cohort with recurrence after CCRS.

Methods

This retrospective study analyzed a prospective database of patients with recurrence after CCRS for appendiceal neoplasms from 1994 to 2017 who had long-term follow-up evaluation with tumor markers and computed tomography (CT).

Results

Overall, 430 (37.6%) of 1145 PMP patients experienced recurrence at a median of 19 months. Of these 430 patients 145 (33.7%) underwent repeat CRS, 119 (27.7%) had a watch-and-wait approach, and 119 (27.7%) had palliative chemotherapy. The patients with recurrence had a median overall survival (OS) of 39 months, a 3-year survival of 74.6%, a 5-year survival of 57.4%, and a 10-year survival of 36.5%. In the multivariate analysis, the patients who had recurrence within 1 year after primary CRS (hazard ratio [HR], 3.55), symptoms at recurrence (HR, 3.08), a high grade of disease or adenocarcinoma pathology (HR, 2.94), signet ring cells (HR, 1.91), extraperitoneal metastatic disease (HR, 1.71), or male gender (HR, 1.61) had worse OS. The OS was longer for the patients who had repeat CRS (HR, 0.41). The patients who underwent repeat CCRS had a 3-year OS of 87.5%, a 5-year OS of 78.1%, and a 10-year OS of 67.9%.

Conclusions

Dilemmas persist around the optimal management of patients with recurrence after CRS and HIPEC for appendiceal tumors. Selected patients benefit from repeat CRS, particularly those with favorable tumor biology and focal disease.

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Authors and Affiliations

  1. Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, UK

    Nima Ahmadi MBBS, BSc(Med) Hons, MS, FRACS, Nima Ahmadi MBBS, BSc(Med) Hons, MS, FRACS, Shinichiro Sakata MBBS, FRACS, William Robert Ball BSc Med Sci (Hons), MbChB, FRCS, Jamish Gandhi MBChB, FRACS, Norman John Carr FRCPath, Alexios Tzivanakis MD, FRCS, Sanjeev Paul Dayal MBBS, MS, FRCS, Faheez Mohamed MBChB, MD, FRCS, Thomas Desmond Cecil FRCS, DM & Brendan John Moran MB, BCh, FRCSI, M.Ch, Doc Med

Authors
  1. Nima Ahmadi MBBS, BSc(Med) Hons, MS, FRACS
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  2. Danko Kostadinov MD, MHA
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  3. Shinichiro Sakata MBBS, FRACS
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  4. William Robert Ball BSc Med Sci (Hons), MbChB, FRCS
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  5. Jamish Gandhi MBChB, FRACS
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  6. Norman John Carr FRCPath
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  7. Alexios Tzivanakis MD, FRCS
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  8. Sanjeev Paul Dayal MBBS, MS, FRCS
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  9. Faheez Mohamed MBChB, MD, FRCS
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  10. Thomas Desmond Cecil FRCS, DM
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  11. Brendan John Moran MB, BCh, FRCSI, M.Ch, Doc Med
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Contributions

Conception and design: NA, JG, BM; Acquisition of data: NA, DK, SS, WB; Analysis and interpretation of data: NA, BM, TC, FM, SD, AT; Drafting of article: NA, DK, SS, WB, BM; Critical revision of article: NA, JG, BM, TC, FM, SD, AT

Corresponding author

Correspondence to Brendan John Moran MB, BCh, FRCSI, M.Ch, Doc Med.

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Ahmadi, N., Kostadinov, D., Sakata, S. et al. Managing Recurrent Pseudomyxoma Peritonei in 430 Patients After Complete Cytoreduction and HIPEC: A Dilemma for Patients and Surgeons. Ann Surg Oncol 28, 7809–7820 (2021). https://doi.org/10.1245/s10434-021-10093-z

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  • DOI: https://doi.org/10.1245/s10434-021-10093-z

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