Introduction

Pseudomyxoma peritonei (PMP) is a rare, slow growing tumor, traditionally considered chemoresistant. The only curative approach is cytoreductive surgery (CRS) followed by hyperthermic intraperitoneal chemotherapy (HIPEC). At disease relapse, or in patients with inoperable disease at diagnosis, no standard treatment has been defined, though nonrandomized series showed promising results with fluoropyrimidine-based regimens.

Patients and Methods

We conducted a prospective study in patients with relapsed or unresectable PMP and confirmed disease progression at baseline. Patients received MMC (7 mg/m² every 6 weeks, up to a maximum of 4 cycles) plus metronomic capecitabine (625 mg/sqm/day b.i.d.) and bevacizumab (7.5 mg/kg every 3 weeks) until disease progression, unacceptable toxicity, or consent withdrawal. Primary endpoint was progression-free survival (PFS); secondary endpoints were overall survival (OS), overall response rate according to RECIST v1.1 criteria, serum markers response and safety.

Results

Fifteen patients were included. At a median follow-up of 26.1 months (IQR, 17.7-49.6), median PFS was 17.9 months (95% CI, 11.0-NE), with 1-year PFS and OS rates of 73% and 87%. Safety profile was manageable, with only 13% G3/G4 treatment-related adverse events.

Conclusion

Metronomic capecitabine, bevacizumab, and MMC are an active regimen in advanced and progressive PMP and favorably compares with historical series.

中文翻译：

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肌原霉素-C、节拍卡培他滨和贝伐珠单抗治疗不可切除或复发性阑尾源性腹膜假粘液瘤患者

介绍

腹膜假粘液瘤(PMP) 是一种罕见的、生长缓慢的肿瘤，传统上被认为具有化疗耐药性。唯一的治疗方法是细胞减灭手术（CRS），然后进行腹腔热灌注化疗（HIPEC）。在疾病复发或诊断时患有无法手术的疾病的患者中，尚未确定标准治疗方法，尽管非随机系列显示基于氟嘧啶的治疗方案有希望的结果。

患者和方法

我们对复发或不可切除的 PMP 患者进行了一项前瞻性研究，并在基线时证实了疾病进展。患者接受MMC（每 6 周 7 mg/m ² ，最多 4 个周期）加节律卡培他滨（625 mg/m2/天 bid）和贝伐单抗（每 3 周 7.5 mg/kg），直至疾病进展、出现不可接受的毒性，或同意撤回。主要终点是无进展生存期（PFS）；次要终点是总生存期 (OS)、根据 RECIST v1.1 标准的总缓解率、血清标志物反应和安全性。

结果

包括十五名患者。中位随访时间为 26.1 个月（IQR，17.7-49.6），中位 PFS 为 17.9 个月（95% CI，11.0-NE），1 年 PFS 和 OS 率为 73% 和 87%。安全性可控，只有 13% 的 G3/G4 治疗相关不良事件。

结论

节拍卡培他滨、贝伐珠单抗和 MMC 是晚期和进行性 PMP 的有效治疗方案，与历史系列相比具有优势。