恶性腹膜间皮瘤的诊治体会

目的：提高对恶性腹膜间皮瘤的诊断和治疗水平方法：对我院40年来收治的22倒恶性腹膜间皮瘤患者的临床资料结合相关文献进行回顾性分析。结果：22例收治经病理证实的患者，男性9例，女性13倒，年龄18～72岁．13例行减瘤手术＋腹腔或／和全身化疗，1例行手术减瘤＋腹部放疗；4例行剖腹探查＋腹腔及全身化疗；1例未手术．仅行腹腔十全身化疗；3例未手术，仅行全身化疗，经治疗后随访中位生存期18．5月。3例存活超过5年、，结论：恶性腹膜间皮瘤诊断困难．易误诊，预后差，主要依靠剖腹探查、疆水细咆学、B超引导穿刺活检、腹腔镜活检等方法，治疗主要以手术切除减瘤术结合全身化疗的综合治疗为主要手段、     

恶性腹膜间皮瘤13例临床特点及诊治体会

目的：探讨恶性腹膜间皮瘤的临床特点、诊断方法和治疗方法及预后。方法：对13例经病理证实的恶性腹膜间皮瘤临床病例进行回顾性分析。结果：13例恶性腹膜间皮瘤中,主要临床表现为腹胀9例（69.2%）,腹痛6例（46.2%）;主要体征为腹水11例（84.6%）,腹部包块3例（27.3%）;血、腹水肿瘤标记物升高、渗出性腹水及影像学提示腹膜及网膜增厚是常见的客观特征,误诊率高（8例,61.5%）,确诊有赖于各种有创性方法获取病理证实。手术切除或行腹腔镜下减瘤术4例（30.8%）,腹腔化疗8例（61.5%）,全身化疗7例（53.8%）,其中4例腹腔化疗联合全身化疗。本组中位生存期仅2.8个月。结论：该病临床表现缺乏特异性,诊断应尽早并尽可能取腹膜病理活检,治疗应采用以手术、全身及或局部化疗为主的综合治疗方法。     

女性恶性腹膜间皮瘤17例临床分析

目的提高对恶性腹膜间皮瘤的临床特征、诊断及治疗手段的认识。方法回顾性分析17例女性恶性腹膜间皮瘤的临床资料。结果17例中主要表现为腹胀12例,腹痛10例,体征为腹水征阳性,腹部及盆腔肿块;10例血小板计数升高;13例有石棉接触史;10例行腹腔镜活检,4例剖腹探查,3例术后诊断。病理分型：上皮型8例,肉瘤型5例,混合型4例。9例行肿瘤细胞歼灭术,9例腹腔静脉联合化疗,1例腔内静脉联合化疗加放疗,1例腔内热化疗联合静脉化疗,3例腔内化疗。确诊到死亡时间平均为9月。结论石棉是导致恶性间皮瘤的主要诱因之一。该病临床表现缺乏特异性,诊断应尽早并可能取腹膜病理活检,必要时联合免疫组化鉴别,治疗采取以手术、局部及全身化疗为主。     

恶性腹膜间皮瘤——附10例报告

 本文报告经病理组织学证实的恶性腹膜间皮瘤10例，并以临床特点、诊断和治疗等方面进行分析讨论．由于腹膜间皮瘤较少见，误诊率高，作者认为对腹部肿块、腹痛、腹胀患者在排除消化道和泌尿生殖系统肿瘤后应考虑此病的可能．10例经剖腹探查均不能切除，其中2例行腹壁下动脉插管化疗，1例行腹腔和全身化疗，取得一定疗效，提示此瘤采用综合治疗仍有一定价值．     

恶性腹膜间皮瘤五例临床分析

目的 提高对恶性腹膜间皮瘤的认识。方法 结合文献复习。对5例恶性腹膜间皮瘤的诊断及治疗进行讨论。结果 恶性腹膜间皮瘤术前诊断极为困难，常误诊为卵巢癌，最终确诊依据病理检查并结合免疫组化，治疗方案为肿瘤细胞减灭术，术后辅以化疗和放疗。结论 本病恶性程度高，预后差，综合治疗是提高恶性腹膜间皮瘤生存率的有效方法。     

恶性腹膜间皮瘤五例临床分析

目的　提高对恶性腹膜间皮瘤的认识。方法　结合文献复习 ,对 5例恶性腹膜间皮瘤的诊断及治疗进行讨论。结果　恶性腹膜间皮瘤术前诊断极为困难 ,常误诊为卵巢癌 ,最终确诊依据病理检查并结合免疫组化。治疗方案为肿瘤细胞减灭术 ,术后辅以化疗和放疗。结论　本病恶性程度高 ,预后差。综合治疗是提高恶性腹膜间皮瘤生存率的有效方法     

胃肠道间质瘤的临床病理分析

目的：探讨胃肠道间质瘤的临床病理学特征及合理诊治措施。方法：回顾分析1989年5月—2000年6月经手术治疗病理证实的胃肠道、腹膜后间质瘤23例，就其诊断方法、检查手段、手术方式进行分析。结果：本组23例患者中，发生于胃肠道16例(69．5％)，腹膜后7例(30．5％)；良性间质瘤9例(39．2％)，恶性间质瘤14例(60．8％)。23例均行手术切除，临床表现以腹部肿块、隐痛为主。结论：胃肠道间质瘤的临床表现和影像学检查无特异性，明确诊断需依靠病理学检查。手术切除是有效的治疗方法。     

38例腹膜恶性间皮瘤的临床分析

自1965年至1988年,我院收治了38例腹膜恶性问皮瘤患者,其1年、2年和3年生存率分另为63.2％、28.9％和21.1％。全组病例与预后有关的因素包括:体力状况、病程、有否腹水和消瘦、WBC的计数、肿块的数目、病理和治疗方法。建议对腹膜间皮瘤的患者的首次治疗采用多学科的综合治疗。     

恶性胸膜间皮瘤29例分析

目的：探讨恶性胸膜间皮瘤诊断和治疗的临床特点。方法：回顾性分析手术治疗29例恶性胸膜间皮瘤患者的临床资料。结果：术后2年生存率为51．7％(15／29)，5年生存率为31．0％(9/29)。手术并发症发生率为24．1％(7／29)，无手术死亡者。接受根治术、姑息手术、探查术的患者5年生存率存在明显差异；局限型患者5年生存率高于弥漫型患者。患者生存率与TNM分期明显相关。结论：手术治疗恶性胸膜间皮瘤有效。国际间皮瘤研究组提出的新的恶性胸膜间皮瘤TNM分期标准客观且可行。Ⅰ、Ⅱ期患者适行手术治疗；Ⅳ期患者不适行手术治疗；对局限型、上皮型、估计手术能达到肉眼根治的Ⅲ期患者应考虑手术治疗。     

手术切除联合间质内缓释化疗治疗复发性脑胶质瘤

目的 探讨外科手术切除与瘤床问质内缓释化疗治疗复发性脑胶质瘤的疗效。方法 对30例复发性恶性脑胶质瘤患者行开颅手术全切除,术中在瘤床植入Vp-16多聚缓释体,用量为100～150mg。随访3～24个月,观察肿瘤再次复发率和患者死亡率,并与以前随访的46例再次手术的恶性脑胶质瘤结果相对比。结果 30例患者均获随访,3个月复发5例(16．7％),死亡2例(6．7％);6个月复发9例(30．0％),死亡6例(20．0％);12个月复发15例(50．0％),死亡12例(40．0％);24个月复发25例(83．3％),死亡21例(70．0％);全部患者均无化疗不良反应。结论 力争手术全切除联合瘤床间质内缓释化疗,可提高药物在瘤灶处的浓度和作用时间,从而提高复发性脑胶质瘤的疗效。     

11例正常大小卵巢癌综合征的临床及病理分析

目的探讨正常大小卵巢癌综合征的临床特点、诊断标准、有效的治疗方法及其预后。方法回顾性分析１９８４～１９９３年在我院治疗及病理复核证实的１１例正常大小卵巢癌综合征,其中７例为性腺外苗勒氏管肿瘤,也称卵巢外腹膜浆液性乳头状腺癌（ＥＰＳＰＣ）,２例卵巢浆液性腺癌,１例恶性间皮瘤,１例原发灶不明的转移性腺癌。上述无论何种类型,均宜最大限度切除肿瘤,术后残存肿瘤＜２ｃｍ,并辅以有效多程综合化疗。结果１例恶性间皮瘤存活９０个月,随访至１９９７年６月仍健在。１例ＥＰＳＰＣ已存活８７个月,随访至１９９７年１１月仍健在。９例已死亡患者平均生存１７．８个月,比文献报道存活时间长。结论对此综合征患者必须高度重视鉴别诊断,首选手术治疗,尽量切除癌灶（残存肿瘤＜２ｃｍ）,术后辅以有效化疗或放疗,以获得最佳生存和预后。     

恶性胸膜间皮瘤19例临床分析

目的 经病理证实的19例恶性胸膜间皮瘤的诊断经验。方法 分析19例恶性胸膜间皮瘤的临床资料,包括石棉接触史、临床表现、影像学检查、特殊检查、胸水检测和转移情况,并对比15例手术结果。结果 7例工种与石棉有关,占恶性胸膜间皮瘤36.8%(7/19),18例（94.7%)有胸痛,其中16例（84.2%)伴胸腔积液,1例出现Homer‘s综合症并影响臂丛神经,3例（15.8%)CT下穿刺找到间皮瘤细胞,2例（10.8%)胸水中找到间皮瘤细胞,1例锁髓上淋巴结穿刺找到间皮瘤细胞,15例CT发现胸膜增厚或胸膜上结节样病灶,占恶性弥漫性胸膜间皮瘤78.9%,15例手术病例中11例胸膜广泛增厚或胸壁上广泛不规则大小不等结节融合成片,侵犯肺组织或纵隔胸膜、横膈胸膜。结论 石棉接触史是恶生胸间皮瘤主要病因,胸痛、胸腔积液是胸膜间瘤的主要临床表现,CT对诊断胸膜间皮瘤有重要参考价值。CT下活检能提高诊断率。     

胸膜间皮瘤19例临床分析

目的总结１９例胸膜间皮瘤的诊断经验。方法分析１９例胸膜间皮瘤的临床资料,包括年龄、石棉接触史、临床表现、影像学检查、实验室检查和转移情况。结果１９例患者均无明确石棉接触史;８例（４２．１％）无明显胸痛;９例（４７．４％）单纯右侧胸腔积液;２例（１０．５％）臂丛神经受影响;１例（５．２６％）胸水中查到恶性间皮细胞;９例（４７．４％）Ｂ超检查发现胸膜肥厚或结节;１３例（６８．４％）ＣＴ检查发现胸膜结节状病灶。结论石棉接触史并非胸膜间皮瘤的唯一病因;胸痛并非胸膜间皮瘤诊断的必要条件;Ｂ超和ＣＴ检查对临床发现胸膜间皮瘤有提示作用。     

Anaplastic Lymphoma Kinase Gene Rearrangement in Children and Young Adults With Mesothelioma

IntroductionChildren and young adults diagnosed with malignant mesothelioma may have unique genetic characteristics. In this study, we evaluated for the presence of the anaplastic lymphoma kinase (ALK) translocations in these patients.MethodsIn a prospective study of mesothelioma natural history (ClinicalTrials.gov number NCT01950572), we assessed for the presence of the ALK translocation in patients younger than 40 years, irrespective of the site of disease. The presence of this translocation was assessed by means of fluorescence in situ hybridization (FISH). If the patients tested positive for the ALK translocation, both immunohistochemistry and RNA sequencing were performed on the tumor specimen.ResultsBetween September 2013 and December 2018, 373 patients were enrolled in the mesothelioma natural history study, of which 32 patients were 40 years old or younger at the time of their mesothelioma diagnosis. There were 25 patients with peritoneal mesothelioma, five with pleural mesothelioma, one with pericardial mesothelioma, and one with bicompartmental mesothelioma. Presence of an ALK translocation by FISH was seen in two of the 32 patients (6%) with mesothelioma. Both patients, a 14-year-old female and a 27-year-old male, had peritoneal mesothelioma and had no history of asbestos exposure, prior radiation therapy, or predisposing germline mutations. Neither had detectable ALK expression by immunohistochemistry. RNA sequencing revealed the presence of an STRN fusion partner in the female patient but failed to identify any fusion protein in the male patient.ConclusionsYoung patients with peritoneal mesothelioma should be evaluated for the presence of ALK translocations. Presence of this translocation should be assessed by FISH and these patients could potentially benefit from tyrosine kinase inhibitors targeting ALK.     

Radiation therapy in the management of patients with mesothelioma

The results of radiation therapy in the management of 27 patients with malignant mesothelioma were reviewed. Eight patients were treated with a curative intent combining attempted surgical excision of tumor (thoracic in 6 and peritoneal in 2), aggressive radiation therapy, and combination chemotherapy using an adriamycin-containing regimen. One patient achieved a 2-year disease-free interval followed by recurrence of tumor above the thoracic irradiation field. This patient was retreated with localized irradiation and is disease-free after 5 years of initial diagnosis. One patient has persistent abdominal disease at 18 months; the other 6 patients suffered local recurrence within 8-13 months of initiation of treatment. Radiation therapy was used in 19 other patients who received 29 courses for palliation of dyspnea, superior vena cava syndrome, dysphagia, or neurological symptoms of brain metastasis. A palliation index was used to determine the effectiveness of irradiation and revealed that relief of symptoms was complete or substantial in 5 treatment courses, moderately effective in 6 courses and inadequate in 18 treatment courses. Adequate palliation strongly correlated with a dose at or above 4,000 rad in 4 weeks. The management of patients with mesothelioma requires new and innovative approaches to increase the effectiveness of radiation therapy and minimize the significant potential combined toxicity of pulmonary irradiation and adriamycin.     

Malignant peritoneal mesothelioma

BACKGROUND: The clinical characteristics of malignant peritoneal mesothelioma are not fully known, and it appears as a variable entity with different types of clinical presentation and with a difficult diagnosis. PATIENTS: Fifteen patients with malignant peritoneal mesothelioma were analyzed for asbestos exposure, clinical presentation, thrombocytosis, X-rays and echotomographic findings, peritoneal fluid cytology, surgical investigations, diagnosis in vita, therapy, cause of death, diagnosis time, and survival time. RESULTS: Asbestos exposure was present in 12 men. Abdominal pain, ascites, abdominal mass, weight loss and fever were the most common presentation symptoms. In 5 patients, the disease presented as a surgical emergency. Assembling the presenting symptoms, malignant peritoneal mesothelioma was subdivided in 3 types: classical (6 cases), surgical (5 cases) and medical (4 cases). Thrombocytosis was present in 11 cases. Peritoneal fluid cytology was positive for neoplastic mesothelial cells in 8 of 10 cases. Laparotomy (5 patients) and laparoscopy (7 cases) were diagnostic in all cases. Diagnosis in vita was malignant peritoneal mesothelioma for 13 patients, peritoneal carcinomatosis for 1, with only 1 autopsy diagnosis. Seven patients were treated with chemotherapy, showing a progression of the disease. Mean symptoms-to-diagnosis time was 122 days (4-410), and mean symptoms-to-survival time was 345 days (45-1510). CONCLUSIONS: Malignant peritoneal mesothelioma is a very unusual disease characterized by a difficult diagnosis, a rapid evolution, a poor response to therapy, and a very high prevalence of thrombocytosis. A new clinical classification into three types (classical, surgical and medical) may be useful for a correct diagnosis. The early diagnosis of malignant peritoneal mesothelioma remains an important open question.     

BAP1 Is Altered by Copy Number Loss,Mutation, and/or Loss of Protein Expression in More Than 70% of Malignant Peritoneal Mesotheliomas

IntroductionMalignant mesothelioma is a deadly disease that is strongly associated with asbestos exposure. Peritoneal mesotheliomas account for 10% of all the cases. BRCA1 associated protein 1 (BAP1) is a deubiquitinating hydrolase that plays a key role in various cellular processes. Germline and somatic inactivation of BRCA1 associated protein 1 gene (BAP1) is frequent in pleural mesothelioma; however, little is known about its status in peritoneal mesothelioma.MethodsTaking advantage of the extensive French National Network for the Diagnosis of Malignant Pleural Mesothelioma and Rare Peritoneal Tumors and the French National Network for the Treatment of Rare Peritoneal Surface Malignancies, we collected biological material and clinical and epidemiological data for 46 patients with peritoneal mesothelioma. The status of BAP1 was evaluated at the mutational and protein expression levels and combined with our previous data on copy number alterations assessed in the same samples.ResultsWe detected mutations in 32% of the malignant peritoneal mesotheliomas analyzed. In addition, we have previously reported that copy number losses occurred in 42% of the samples included in this series. Overall, 73% of the malignant peritoneal mesotheliomas analyzed carried at least one inactivated BAP1 allele, but only 57% had a complete loss of its protein nuclear expression. Better overall survival was observed for patients with BAP1 mutations (p = 0.04), protein expression loss (p = 0.016), or at least one of these alterations (p = 0.007) independently of tumor histological subtype, age, and sex.ConclusionsAs in pleural mesothelioma, inactivation of BAP1 is frequent in peritoneal mesotheliomas. We found that BAP1 protein nuclear expression is a good prognostic factor and a more reliable marker for the complete loss of BAP1 activity than mutation or copy number loss.     

原发性胃恶性淋巴瘤的临床特点和外科治疗

目的 :探讨原发性胃恶性淋巴瘤的临床特点和治疗。方法 :回顾性分析 2 1例原发性胃恶性淋巴瘤的诊治情况。结果 :2 1例中术前经胃镜活检确诊 7例 ,确诊率仅为 33 3% ,手术切除 2 0例 ,行全胃切除 12例 ,胃大部切除 8例 ,剖腹探查 1例 ,5年生存率为 5 7 1%。结论 :原发性胃恶性淋巴瘤术前确诊以胃镜活检为主要手段 ,但确诊率仍待提高。治疗以手术切除为主 ,术前辅助化疗有助于晚期肿瘤的手术切除     

今又生联合热疗治疗浆膜腔恶性积液近期疗效

将p53抑癌基因(今又生)应用于恶性间皮瘤、消化道肿瘤所致的恶性浆膜腔积液的控制,观察其对恶性积液的治疗效果。选择经病理组织学或细胞学确诊或影像学结合肿瘤标记物血清学检查临床诊断的恶性间皮瘤、食管癌、胃癌、胰腺癌和结肠癌等消化道肿瘤伴发恶性浆膜腔积液的患者32例,治疗前后客观测量积液量,用100mL生理盐水溶解p53抑癌基因(今又生)1支(1×1012VP),注入浆膜腔,72h后体外射频热疗,每周1次,治疗2周后评价积液控制效果。结果8例恶性间皮瘤CR2例,PR3例,NE3例,临床有效率为62%;24例恶性消化道肿瘤CR5例(其中食管癌1例,胃癌3例,结肠癌1例),PR7例(其中食管癌1例,胃癌4例,结肠癌2例),NE12例,临床有效率为50%。初步研究结果提示,p53抑癌基因——今又生可用于治疗恶性间皮瘤、消化道肿瘤(食管癌、胃癌、结肠癌)伴发的恶性浆膜腔积液,积液控制效果明显。