Progression of Pseudomyxoma Peritonei after Combined Modality Treatment: Management and Outcome

Abstract

Background: Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment strategy for pseudomyxoma peritonei (PMP) with curative intent. The aim of this study was to determine the patterns of failure in patients who underwent such a procedure and to evaluate management and outcome of progressive disease.Methods: After exclusion of patients with overt malignancy, progression was studied in 96 PMP patients treated primarily by CRS with HIPEC. Location, pathology, management and outcome were recorded.Results: Median follow-up was 51.5 months (0.1-99.5). Median progression free survival (PFS) was 28.2 months (95% CI 18.3->). Progressive disease was mainly located sub hepatic (38%) or in multiple regions (36%). Pathological dedifferentiation was observed in 8 patients (20%). The choice of treatment depended on pathology, extent of disease and PFS. Seventeen patients were treated for progression by second CRS with (n=8) or without HIPEC (n=10). The 3-years overall survival (OS) probability after this treatment was 100% and 53.3% (95% CI 28.2-100%), respectively. Fifteen patients with (slow) progression were observed. Threeyears OS probability of these patients was 66.0% (95% CI 43.4-100%). All patients treated for progression by systemic chemotherapy only (n=6) had died of disease after a median follow up of 14.8 (9.8-33.6) months. A longer PFS after primary treatment was associated with longer OS after progression (P = 0.04).Conclusions: Progressive PMP after primary CRS with HIPEC is probably the result of technical failure and/or tumor biology. Management of progressive PMP can be valuable for selected patients and should depend primarily on the PFS.

Learning curve of combined modality treatment in peritoneal surface disease

“…Patients with PMP deposits in the subhepatic and gastric region are exceptions, as thorough cytoreduction and exposure to HIPEC is difficult at these sites 31 . Incomplete cytoreduction does not, however, necessarily affect prognosis: progressive PMP after incomplete cytoreduction seems manageable and so the procedure appears worthwhile in a considerable number of patients 32 . These considerations do not hold for PMP of the mucinous carcinomatosis subtype, which is comparable with PC from colorectal cancer in both behaviour (invasive properties) and prognosis 22,30 .…”

FOLFOX-4 Chemotherapy for Patients With Unresectable or Relapsed Peritoneal Pseudomyxoma

“…It has been clearly demonstrated that a combined approach of CRS and HIPEC is the standard treatment of patients with PPM because it is able to provide a survival advantage compared with historical controls. Treatment failure may be predicted by several prognostic factors, which were validated in large series [6,[16][17][18]. Several retrospective analyses documented that previous use of systemic chemotherapy was an independent poor prognostic factor for patients undergoing curative CRS and HIPEC for appendiceal neoplasms associated with PPM [19][20][21].…”

Pathologie tumorale du péritoine : cas no 2 : une tumeur péritonéale récidivante

“…. Dans une étude hollandaise publiée en 2007, une dédif-férenciation est observée dans 20 % des récidives [10]. En 2011, en utilisant les classifications de Ronnett et de Bradley, Terence Chua rapporte 16 % d'évolutivité des PP de bas grade en haut grade [11].…”