腹膜间皮瘤(peritoneal mesothelioma)

腹膜间皮瘤(peritoneal mesothelioma) 为原发于腹膜上皮和间皮组织的肿瘤，临床很少见。病
理上可将之分为腺瘤样间皮瘤(adenomatoid mesothelioma)、囊性间皮瘤(cystic mesothelioma)和恶
性间皮瘤(peritoneal malignant mesothelioma,PMM)。前两者属良性肿瘤。囊性间皮瘤多见于女性，
病因不明，好发于盆腔或附件周围，呈单个或多个囊性肿块；病人常因扪及腹块而就诊。PMM约占恶性
间皮瘤之30%；其发生也与接触石棉有密切关系，约5%病人有接触史[1]；石棉纤维经口摄入后，通过
肠壁易位到腹膜而致病。从接触石棉到确诊，本病潜伏期可长达25～40年之久。但国内1951～1993年
20篇文献报告161例PMM中仅1例有石棉接触史。在无石棉接触史的人群中，其发病率约1人/1百万人·年，
可能与某些病毒感染及遗传因素有关[2、3、4]。国外曾报告1例PMM病人40余年前曾接触过胶质二氧化
钍(Thorotrast)[5]。PMM常发生于40岁以上男性。脏层或壁层腹膜均可罹及；肿瘤可直接侵犯腹、盆腔
脏器；50%～70%病人伴淋巴和(或)血行转移远处如肝、肾、肾上腺、肺、骨和淋巴结等。本病临床表现
缺乏特异性，可有腹痛、便秘、腹胀、体重减轻及其他肠梗阻表现[6]。体检可发现腹水或腹块等。腹水
为渗出液，部分为血性。本病易误诊为结核性腹膜炎、复发性自发性腹膜炎、肠系膜炎症或腹膜转移癌
等。腹水透明质酸明显增高，＞0.8g/L者仅见于PMM。腹水脱落细胞检查亦有一定价值，但结果常难以
判断。血清糖类抗原—125(CA125)升高，有助诊断本病[6、7]。B超和CT表现多样，典型者腹膜不规则
增厚，大网膜粘连呈饼状，肠系膜呈薄纸样；CT也可显示增强的胰周大肿块，或腹腔内弥漫性实质性大
肿块，并侵犯肠管和肠系膜；或腹膜结节，或呈囊性包块；多伴有不同程度腹水[8]。超声或CT引导下穿
刺活检有一定价值。腹腔镜检查是诊断PMM的简单而有效的方法，镜下见腹膜、网膜弥漫性结节和斑块，
并可直视下活检病理学检查。我们曾给1例83岁男性病人腹腔镜检查，腹膜活检病理报告间皮细胞增生，
后经组织免疫生化检查方确诊PMM。Butchart等将PMM分为4期:I期，肿瘤局限于腹膜；Ⅱ期，肿瘤侵犯
腹腔内淋巴结；Ⅲ期，肿瘤向腹腔以外淋巴结转移；Ⅳ期，远处血行转移。上述分类有助选择治疗方法。
PMM迄今无有效的规范的治疗方案。预后很差，诊断后生存期中位数为1年，存活逾2年者不足20%。主要
死于恶液质或肠梗阻，死因很少与肿瘤远处转移有关。

 Peritoneal mesothelioma (peritoneal mesothelioma) in the primary peritoneal mesothelial epithelium and the tumor, clinical rarely see. Pathology can be categorized as adenomatous mesothelioma (adenomatoid mesothelioma), cystic mesothelioma (cystic mesothelioma), and malignant mesothelioma (peritone al malignant mesothelioma, PMM). The first two are benign. Cystic Mesothelioma more common in women, cause unknown, occurs in the pelvic or around the annex, was single or multiple cystic masses; Patients often palpable abdominal mass and treatment. SILVER malignant mesothelioma about 30%; Its incidence is also closely linked with exposure to asbestos. About 5% of patients with history of exposure [1]; Asbestos fibers oral intake, translocation through the intestinal wall into the peritoneal and pathogenicity. From exposure to asbestos that were diagnosed, the disease incubation period could be as long as 25 ~ 40 years. But domestic 1951 ~ 1993 20 literature analyzing 161 cases only one cases with asbestos exposure history. In the absence of a history of exposure to asbestos population, the incidence rate of about 1 / 1 million a year, may be some infection and genetic factors [2,3,4]. Abroad have reported a case of analyzing patients 40 years ago, I contacted glial thorium dioxide (Thorotrast) [ 5]. SILVER often occurred in men over the age of 40. Visceral or parietal peritoneum and may happen; Tumors can be a direct violation of abdominal, pelvic organ; 50% ~ 70% of patients with lymphatic and (or) hematogenous metastasis distant as liver, kidney, adrenal gland, lung, bone and lymph node, and so on. Clinical manifestations of this lack of specificity, and may have abdominal pain, constipation, swelling, weight loss and other obstruction performance [6]. Physical examination can be found ascites or abdominal mass. Ascites exudate part of gutsy. The disease often misdiagnosed as tuberculosis peritonitis and recurrent spontaneous bacterial peritonitis, mesenteric inflammation or peritoneal metastasis of cancer, and so on. Ascites hyaluronic acid increased significantly, "0.8 g / L were only found in analyzing. Ascites cytology has a certain value, but often difficult to judge. Serum carbohydrate antigen 125 (CA125) increased help diagnosis of the disease [6,7]. Ultrasound and CT performance varied, the typical person irregularly thickened peritoneum, omentum was Caky adhesions, tissue samples were mesenteric; CT may also show increased Peripancreatic large mass, or intraperitoneal substantive diffuse large mass, and bowel and mesenteric violations; or peritoneal nodules or showed a cystic mass; more with different degree of ascites [8]. Ultrasound or CT-guided biopsy is a definite value. Laparoscopy is analyzing the diagnosis of simple and effective method of endoscopic see peritoneum, omentum and diffuse nodular plaque, may look biopsy pathology examination. We had given one 83-year-old male patient laparoscopy, peritoneal biopsy report mesothelial cell hyperplasia, After immunohistochemical tests confirmed analyzing side. Butchart and others will be divided into four SILVER : I, confined to the peritoneal tumor; Phase II, intraperitoneal tumor invasion lymph nodes; Phase III, to the peritoneal tumor metastasis outside; Ⅳ, distant metastasis. These classifications will help choose the method of treatment. SILVER so far no effective standard treatment programs. The prognosis is poor, survival after diagnosis for a period, the median survival of more than two years of less than 20%. Mainly died for cachexia, or obstruction, and the cause of death rarely distant metastasis of the tumor.

 

PMM为少见的腹膜恶性肿瘤，一向认为其预后很差， 迄今尚无规范化治疗方案。但这种状况有望改观。
近年，以手术为主，化疗、放疗、免疫治疗为辅的联合疗法已初见端倪。

SILVER rare peritoneal malignant and that the prognosis has been poor, so far there is no standardized treatment program. However, this situation is expected to change. In recent years, with surgery, chemotherapy, radiotherapy, the treatment of immune therapy supplemented by the joint has have appeared.

　　对主诉慢性腹痛、腹胀的中、老年病人，特别是有腹水和(或)腹块者，经超声或CT检查证实腹腔内肿块
或腹膜上有结节；腹水呈渗出性，腹水透明质酸显著增高；血清CA125上升， 应高度怀疑PMM。对这些患者
应予以超声或CT引导下穿刺活检， 腹腔镜检查，甚至剖腹探查；在估计肿瘤扩散范围的同时，于脏层和壁层
腹膜多处活检，取得足够组织，供病理检查和组织免疫或化学检查。多数PMM患者在确诊时，已难以手术完全
切除肿瘤。 如无肠梗阻表现，应先给予2～3个疗程诱导性腹腔内化疗，以最大限度地减少肿瘤在肠管表面种
植，为外科手术创造条件，并有助临床医生掌握肿瘤对化疗反应的资料。在诱导性化疗后2个月， 施行减瘤
手术，切除病变腹膜，力求清除所有肿瘤组织。术中或术后早期应给辅助性腹腔内化疗，并配合放疗及BRM辅
助治疗。 对术后肿瘤复发者，如条件许可应考虑再手术。

 

The chief complaint of abdominal pain, abdominal distension, elderly patients, especially those with ascites and (or) abdominal mass, by ultrasound or CT scan confirmed the diagnosis of abdominal peritoneum or a nodule; Ascites was exudative, ascites hyaluronic acid increased significantly; Serum CA125 rising, the highly suspected analyzing. These patients should be ultrasound or CT-guided biopsy, laparoscopic examination and even exploratory laparotomy; estimate the extent of the spread of the tumor, in the visceral and parietal peritoneum multiple biopsies adequate, for pathological examination and immunohistochemical or chemical examination. Most analyzing patients at the time of diagnosis, has been difficult to complete surgical excision of tumor. If no obstruction performance should give 2 - 3 treatment-induced intraperitoneal chemotherapy to minimize the surface of intestinal tumor in cultivation, to create conditions for the surgery. and help clinicians master tumor response to chemotherapy information. In the induction chemotherapy after two months, the purposes of tumor reduction surgery, peritoneal lesions removed, so as to remove all the tumor tissue. Intraoperative or postoperative adjuvant to be intraperitoneal chemotherapy, and radiotherapy and BRM with adjuvant therapy. Right after tumor recurrence, such as conditions permit should consider further surgery.