Chronic perineal fistula as a late failure manifestation of pseudomyxoma peritonei syndrome

Pseudomyxoma peritonei syndrome (PMP) is a rare but fascinating entity in surgical oncology. It consists of the accumulation of mucus in the peritoneal cavity, sometimes in huge amounts, in most of the cases after rupture of an appendiceal tumor. The diagnosis and management of PMP has changed significantly in the past 15 years, with the institution of an aggressive therapeutic combination: cytoreductive surgery and intraperitoneal perioperative chemotherapy. Few reports deal with the late manifestations and complications at endstage of the disease. The cases presented here are unique in that they illustrate a late 'failure' in the perianal area in three patients with long-term PMP.     

罕见病网络互助信息平台构建及模式探析

以腹膜假性黏液瘤网络交流互助平台的构建升级与发展为例,构建罕见病网络互助信息平台模型,推广罕见疾病网络互助模式,指出存在的问题及相应的解决办法。     

Pre-operative tumour marker status predicts recurrence and survival after complete cytoreduction and hyperthermic intraperitoneal chemotherapy for appendiceal Pseudomyxoma Peritonei: Analysis of 519 patients

Background

Cytoreductive surgery (CRS) combined with Hyperthermic Intraperitoneal Chemotherapy (HIPEC) is the optimal treatment for Pseudomyxoma Peritonei (PMP). Despite treatment, disease often recurs and may not be amenable to further CRS. Clinical experience suggests a spectrum of disease which may correlate with tumour marker levels. The aim of this study was to analyse the influence of markers on recurrence and survival.

Methods

The details of all patients undergoing surgery for PMP of appendiceal origin at a national centre for peritoneal malignancy were recorded in a dedicated prospective database. The data on all patients who had CRS and HIPEC between March 1994 and January 2012 was analysed and recurrence and survival correlated with pre-operative levels of CEA, CA-125 and CA19-9.

Results

Overall, 519 (69%) of 752 consecutive patients, underwent complete CRS and HIPEC. The median (range) age was 56 (20–82) years with 342/519 (66%) females. The mean overall (OS) and disease free survival (DFS) in the 131/519 patients who had normal preoperative tumour markers was 168 (128–207) and 125 (114–136) months respectively, significantly higher when compared with the 109/519 (21%) who had all three tumour markers elevated (OS of 65 (42–88) and DFS of 55 (41–70) months respectively) (P = 0.002).

Conclusions

Elevated tumour markers predict an increased risk of recurrence and reduced survival after complete CRS. This may reflect cell biology in low grade tumours and is an independent prognostic feature. Further analysis may help to select patients for post-operative chemotherapy, second look procedures or stratification of follow up.     

Pseudomyoma Peritonei — Preoperative Diagnosis on CT Sean A Report of 2 Cases

The entity of pseudomyxoma peritonei was often diagnosed formerly on laparotomy. However, with the advent of sonography and computerised tomography it is now possible to suggest correct pre-operative diagnosis and thereby proper management. Such two cases diagnosed pre-operatively on CT are reported here.     

Chinese expert consensus on cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignancies

Locoregional spread of abdominopelvic malignant tumors frequently results in peritoneal carcinomatosis(PC). The prognosis of PC patients treated by conventional systemic chemotherapy is poor, with a median survival of 6 mo. However, over the past three decades, an integrated treatment strategy of cytoreductive surgery(CRS) + hyperthermic intraperitoneal chemotherapy(HIPEC) has been developed by the pioneering oncologists, with proved efficacy and safety in selected patients. Supported by several lines of clinical evidence from phases Ⅰ, Ⅱ and Ⅲ clinical trials, CRS + HIPEC has been regarded as the standard treatment for selected patients with PC in many established cancer centers worldwide. In China, an expert consensus on CRS + HIPEC has been reached by the leading surgical and medical oncologists, under the framework of the China Anti-Cancer Association. This expert consensus has summarized the progress in PC clinical studies and systematically evaluated the CRS + HIPEC procedures in China as well as across the world, so as to lay the foundation for formulating PC treatment guidelines specific to the national conditions of China.     

A Primary Peritoneal Pseudomyxoma Peritonei Arising from the Mullerian Tissue: a Case Report

A primary Pseudomyxoma Peritonei is uncommon. For a long time, ovaries, colon and appendix were proposed like a site origin of these enigmatic lesions. Recent publications show that the majority of cases are due to a rupture of appendicle adenoma.

We report a case of a pseudomyxoma peritonei arising in an elderly patient with a previous medical history of appendectomy undergone in infancy. The pseudomyxoma was misdiagnosed as an abdominal abscess related to colitis. In our opinion, metaplasia of the Müllerian system is a possible source of a primary peritoneal pseudomyxoma.     

创伤性肠粘连形成过程中腹膜组织变化的形态学观察

目的:通过研究肠粘连形成过程的组织形态学变化,探索肠粘连的可能发病机理。方法:SD大鼠35只,5只作为正常对照,30只按Harris方法形成腹膜粘连模型。分别于术后3小时至7天处死,动态取粘连形成过程中创面部位盲肠浆膜、壁层腹膜和腹膜粘连部位组织,置10%福尔马林固定,石蜡切片,HE染色,光镜观察以上粘连组织形态学改变。结果:1-3天粘连的腹壁和盲肠正常结构破坏,纤维蛋白渗出渐形成一条红染的边缘清楚的粘附带。4-5天表面炎性渗出,以及新鲜肉芽组织形成,间皮细胞减少或消失。6-7天间皮细胞与纤维细胞之间融合在一起,不易辨认出肠与腹膜组织的明确界限,于粘连缝隙的表面可见一层不连续的扁平的间皮细胞。结论:纤维蛋白渗出增加和间皮细胞在腹膜粘连形成中可能扮演重要作用。肠粘连预防的重点放在尽量减少纤维蛋白渗出和促进腹膜间皮的生长可能是值得临床进一步研究和探索的方向。通过观察腹膜粘连的病理改变,对外科手术以及其它创伤所引起的腹膜病变的去粘连有非常实际的理论价值及形态学依据。     

PET-CT对腹膜假性黏液瘤的诊断价值

[目的]探讨正电子发射计算机断层显像(PET-CT)对腹膜假性黏液瘤(PMP)诊断价值.[方法]回顾性分析本院收治的1例经手术证实的PMP患者的PET-CT影像特点,根据其最大18F-FDG标准吸收值(SUV-max)及其影像特点总结其诊断经验.[结果]PET-CT图像显示腹、盆腔内大量水样密度影,CT值范围约15～19HU,未见明显糖代谢增高.SUVmax为0.86,增强扫描“腹水”未见明显异常强化;改变体位(右侧卧位)检查腹腔内水样密度影形态变化不明显,腹腔肠管未见明显活动;肝脏边缘呈扇贝形受压,肠管受压、内移;网膜、肠系膜未见明显增厚,腹腔内未见明显肿大淋巴结,综上考虑为PMP可能性大,术后病理证实为低级别PMP.[结论]PMP较罕见,PET-CT对该病有较好的诊断价值.