Long-term survival following treatment of pseudomyxoma peritonei - An analysis of surgical therapy

Long-term survival following treatment of pseudomyxoma peritonei - An analysis of surgical therapy
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DOI:
10.1097/01.sla.0000152015.76731.1f
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发表时间:
2005-02-01
期刊:
影响因子:
9
通讯作者:
Coit, DG
Coit, DG
中科院分区:
医学1区
文献类型:
--
作者:
Miner, TJ;Shia, JR;Coit, DG

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背景资料:腹膜假性粘液瘤(PMP)是一种自然病史不明确的临床综合征。肿瘤生物学、患者选择和治疗范围对最终结果的相对贡献没有得到很好的描述。方法:对1980-2002年间在纪念斯隆-凯特琳癌症中心治疗的被诊断为PMP的患者进行鉴定。结果:97例患者接受了平均2.2+/-0.1次手术(范围1-6次)。虽然55%(53/97)的患者细胞完全减少,但91%(48/53)的患者病情复发。完全细胞减少术后中位无病间隔时间为24个月。中位总生存期为9.8年,与低级别病理亚型(P<0.001)和实现细胞完全减少的能力(P<0.001)独立相关。10年生存率为21%(20/97),其中90%(18/20)为低度病理特征。在死亡或完成随访时,只有12%(12/97)的患者无病。结论:PMP患者的预后与肿瘤生物学密切相关。虽然生存率的提高与低级别的病理和易于完全细胞减少的肿瘤有关,但PMP的复发是常见的。治疗可能是有益的,特别是在控制症状方面,但定义为长期无病状态的绝对治愈并不常见。
Background Data: Pseudomyxoma peritonei (PMP) is a clinical syndrome with a poorly defined natural history. Relative contributions of tumor biology, patient selection, and the extent of treatment on ultimate outcome are not well characterized.Methods: Patients treated at the Memorial Sloan-Kettering Cancer Center between 1980 and 2002 with a diagnosis of PMP were identified. Patient characteristics, pathologic features, and details of treatment were analyzed retrospectively.Results: The 97 patients included in this study underwent a mean 2.2 +/- 0.1 operations (range, 1-6). Although complete cytoreduction was achieved in 55% (53/97), disease recurred in 91% (48/53) of patients. The median disease-free interval after complete cytoreduction was 24 months. The median overall survival was 9.8 years and was independently associated with low-grade pathologic subtype (P < 0.001) and the ability to achieve complete cytoreduction (P < 0.001). Ten-year survival was attained in 21% (20/97) of the patients, of which 90% (18/20) had low-grade pathologic features. At the time of death or completion of follow-up, only 12% (12/97) of the patients were disease free.Conclusions: Outcome in patients with PMP is strongly associated with tumor biology. Although improved survival is associated with low-grade pathology and tumors amenable to complete cytoreduction, recurrence of PMP is common. Treatment may be beneficial, particularly in controlling symptoms, but absolute cure, defined as a prolonged disease-free state, is uncommon.