Spiliotis John, Efstathiou Elias, Halkia Evgenia, Vaxevanidou Archodoula, Datsis Anastasios, Sugarbaker Paul
Department of Surgery, Metaxa Cancer Hospital, Piraeus, Greece.
Hepatogastroenterology. 2012 May;59(115):705-8. doi: 10.5754/hge10220.
BACKGROUND/AIMS: Pseudomyxoma peritonei syndrome (PMP) may be associated with slow "benign" or malignant process. However, the natural history of this disease is slow progression to death. Its treatment is variable and controversial. In this current study we have compared the patients with Pseudomyxoma peritonei syndrome with recurrences due to the cell entrapment hypothesis.
Data were derived from a database of patients with PMP treated at our hospitals from 2004 to 2009. All patients had undergone various surgical operations prior to referral to our institutions for definitive treatment. All patients had recurrences in special sites due to entrapment of malignant cells.
There are 6 patients, four men and two women. The initial clinical presentation of the disease was hernia in one, appendicitis in three and ovarian mass in one. The mean time from the initial operation to be definitive management was 23.5 months with an average of 1.83 operations per patient. After cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) the mean survival was 31 months with minimal recurrences and only 0.3 operations per patient.
Our data suggest that the patients should be referred to a center with a peritoneal surface malignancy program after the PMP diagnosis as soon as possible. Incomplete debulking procedures and minimal invasive operations promote uncontrollable intraabdominal tumor growth due to tumor cell entrapment and the tendency of PMP to grow at wound sites.
背景/目的：腹膜假黏液瘤综合征（PMP）可能与缓慢的“良性”或恶性进程相关。然而，这种疾病的自然病程是缓慢进展直至死亡。其治疗方法多样且存在争议。在本研究中，我们根据细胞种植假说对腹膜假黏液瘤综合征复发患者进行了比较。
数据来源于2004年至2009年在我院接受治疗的PMP患者数据库。所有患者在转诊至我院进行确定性治疗之前均接受了各种外科手术。所有患者均因恶性细胞种植在特殊部位出现复发。
共有6例患者，4例男性，2例女性。疾病的初始临床表现为1例疝气、3例阑尾炎和1例卵巢肿块。从初次手术到确定性治疗的平均时间为23.5个月，每位患者平均接受1.83次手术。细胞减灭术和腹腔内热灌注化疗（HIPEC）后，平均生存期为31个月，复发极少，每位患者仅接受0.3次手术。
我们的数据表明，PMP诊断后患者应尽快转诊至有腹膜表面恶性肿瘤治疗项目的中心。由于肿瘤细胞种植以及PMP在伤口部位生长的倾向，不完全减瘤手术和微创手术会促使腹腔内肿瘤生长失控。
