Abstract

BackgroundPseudomyxoma peritonei (PMP) is a rare malignancy, and there is insufficient evidence about systemic chemotherapy for this disease.MethodsWe retrospectively evaluated the efficacy and safety of a chemotherapeutic regimen with 5-fluorouracil and oxaliplatin (modified FOLFOX6, mFOLFOX6) for patients with unresectable pseudomyxoma peritonei. Patients who received the therapy between April 2000 and February 2019 at the Department of Medical Oncology, Tohoku University Hospital, were enrolled in this study.ResultsEight patients were treated with mFOLFOX6. The sites of primary tumor were appendix in six patients, ovary in a patient, and urachus in a patient. Six patients received surgery. Seven patients had histologically high-grade PMP, and one patient had low-grade PMP. The median follow-up duration was 27.2 months. All the patients had non-measurable regions as the targets of tumor response. Non-complete response or non-progressive disease was observed in seven patients, with a disease control rate of 87.5%. The median progression-free survival and overall survival were 13.0 months and 27.9 months, respectively. An obvious reduction in the symptoms was observed in two patients. Five patients experienced decline in the serum tumor markers, CEA or CA19-9. The grade 3/4 toxicity that was observed was grade 4 neutropenia in one patient and grade 3 neutropenia in two patients.ConclusionsmFOLFOX6 might be an effective and tolerable treatment option for patients with unresectable PMP. To our knowledge, this is the first case series of mFOLFOX6 in patients with unresectable PMP and the first case series of systemic chemotherapy for Asian patients with unresectable PMP.

Highlights

  • Pseudomyxoma peritonei (PMP) is a rare clinical manifestation of malignancy, characterized by intraperitoneal dissemination of the tumor and progressive accumulation of Electronic supplementary material The online version of this article contains supplementary material, which is available to authorized users.The histological features of PMP are heterogeneous; several classification systems have been proposed [1,2,3]

  • The median pretreatment CA19-9 of 127 U/mL decreased to 70 U/ml during mFOLFOX6; this change was not. This retrospective analysis demonstrated that some patients with unresectable PMP benefited from mFOLFOX6 treatment

  • Since 2000, three studies that analyzed systemic chemotherapy except adjuvant setting in more than five patients with PMP have reported the efficacy of capecitabine plus mitomycin C, FOLFOX4, capecitabine plus bevacizumab, and mainly fluoropyrimidine alone or combination therapy [9,10,11,12,13]

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Summary

Introduction

In the fourth edition of the WHO Classification of Tumors of the Digestive System, PMP is classified as low or high grade based on the histological criteria previously proposed by Bradley et al [2, 4]. More effective treatment options are required for patients with resectable or unresectable/recurrent PMP. Pseudomyxoma peritonei (PMP) is a rare malignancy, and there is insufficient evidence about systemic chemotherapy for this disease. Conclusions mFOLFOX6 might be an effective and tolerable treatment option for patients with unresectable PMP. To our knowledge, this is the first case series of mFOLFOX6 in patients with unresectable PMP and the first case series of systemic chemotherapy for Asian patients with unresectable PMP

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