34例非阑尾来源腹膜假黏液瘤诊治分析

  目的  探讨非阑尾来源腹膜假黏液瘤（pseudomyxoma peritonei，PMP）的临床病理特征和诊疗经验。  方法  回顾性分析2011年9月至2019年2月于航天中心医院采用肿瘤细胞减灭术（cytoreduction surgery，CRS）联合腹腔热灌注化疗（hyperthermic inter-peritoneal chemotherapy，HIPEC）治疗的34例非阑尾来源PMP患者的临床资料，总结其临床表现及影像学特征，同时利用Log-rank检验对相关因素进行生存分析。  结果  本研究34例患者临床表现主要为腹胀（58.8%）和腹盆腔包块（52.9%）；CRS+HIPEC治疗后主要并发症发生率为14.7%，在随访中9例患者死亡，1、3年生存率分别为69.6%、53.5%；单因素分析中，腹膜癌指数（peritonealcancer index，PCI）>20、未行灌注化疗以及非根治性手术是预后不良的显著危险因素，而性别、年龄、组织来源、病理类型等未体现出显著相关性。  结论  非阑尾来源PMP无特异性临床表现，术前较难判断原发病灶，确诊需要依靠术后病理及免疫组织化学检测。但无论来源如何，均以腹腔内广泛肿瘤种植和局部浸润为主要临床表现，CRS+HIPEC是安全有效的治疗手段。      

肿瘤细胞减灭术加腹腔热灌注化疗治疗腹膜假黏液瘤182例分析

  目的  腹膜假黏液瘤（pseudomyxoma peritonei,PMP）是一种主要来源于阑尾黏液性肿瘤的恶性肿瘤综合征,肿瘤细胞减灭术（cytoreductive surgery,CRS）加腹腔热灌注化疗（hyperthermic intraperitoneal chemotherapy,HIPEC）是国际推荐的PMP标准治疗。本研究旨在评估CRS+HIPEC治疗PMP的疗效及围手术期安全性。  方法  研究首都医科大学附属北京世纪坛医院2001年1月至2008年5月采用CRS+HIPEC治疗182例PMP临床数据库,进行生存分析,通过单因素和多因素分析筛选独立预后因素,并分析围手术期安全性。  结果  182例PMP患者接受CRS+HIPEC治疗,低级别PMP 73例（40.1%）,部分低级别、部分高级别PMP 50例（27.5%）,高级别PMP 53例（29.1%）,PMP伴印戒细胞6例（3.3%）;中位腹膜癌指数（peritoneal cancer index,PCI）30分,PCI≥20分为134例（74.0%）;肿瘤细胞减灭程度（completeness of cytoreduction,CC）评分0~1分者为79例（44.1%）;死亡48例（26.4%）,生存134例（73.6%）,中位生存时间64.7个月（95%CI:43.1~84.3个月）。Cox多因素回归分析发现4个独立预后因素:年龄（HR=12.079,95%CI:1.605~90.916）、CC（HR=0.211,95%CI:0.069~0.641）、是否有吻合口（0个vs. >1个）（HR=5.519,95%CI:1.176~25.907）、吻合口数量（1个vs. >1个）（HR=7.543,95%CI:1.592~35.732）。围手术期死亡率、严重不良事件率分别为1.6%、19.8%。  结论  PMP患者在腹膜肿瘤专科单位接受CRS+HIPEC治疗,达到完全肿瘤细胞减灭,可延长生存,围手术期安全性可接受。      

细胞减灭术加腹腔热灌注化疗治疗结直肠癌腹膜转移癌病例对照研究

  目的  分析细胞减灭术（CRS）加术中腹腔热灌注化疗术（HIPEC）对结直肠癌腹膜转移癌的疗效及安全性。  方法  课题设计为回顾性病例对照研究, 收集结直肠癌腹膜转移癌（CRC PC）患者资料, 按临床病理参数匹配原则, 分为CRS组（CRS+术后全身化疗）29例, HIPEC组（CRS+HIPEC+术后全身化疗）33例。分析两组的总体生存期（OS）及严重不良事件（SAE）。  结果  两组患者临床病理学特征均衡可比, 术中PCI评分及器官/腹膜切除情况相似。两组中位随访时间分别为41.9个月（6.5~110.0个月）和32.0个月（10.5~95.9个月）, OS分别为8.5个月（95% CI: 4.9~12.1个月）和14.5个月（95% CI: 11.9~17.1月）（P=0.007）。术后30天内CRS组3例发生SAE, HIPEC组9例（P=0.126）。多因素分析显示, HIPEC、CC 0~1分、术后化疗周期≥6个周期为改善生存的独立预后因素。  结论  CRS+HIPEC可改善CRCPC患者生存期, SAE无显著增加, 安全性可接受。      

阑尾源性腹膜假黏液瘤单中心诊疗经验

  目的   总结细胞减灭术加腹腔热灌注化疗（cytoreductive surgery & hyperthermic intraperitoneal chemotherapy，CRS+HIPEC）治疗阑尾源性腹膜假黏液瘤（pseudomyxoma peritonei，PMP）的单中心诊疗经验。   方法   回顾性分析2012年1月至2018年12月于航天中心医院收治，病理证实为阑尾源性PMP并经CRS+HIPEC治疗604例患者的临床数据，进行统计学分析。   结果   604例患者经历621次CRS+HIPEC治疗，平均年龄56.7岁，其中女性364例（60.3%），男性240例（39.7%），平均腹膜癌指数（peritoneal cancer index，PCI）为25.7。28.5%（172/604）的患者完全减瘤（CCR 0/1）。3~4级不良事件发生率为21.7%（131/604），围手术期死亡率为0.7%（4/604），术后5年生存率为53.6%。高级别病理类型、不完全减瘤（CCR 2/3）、PCI>20、3~4级不良事件是PMP患者预后不良的独立危险因素。   结论   阑尾源性PMP临床罕见，治疗方法特殊，对于怀疑或确诊PMP的患者，尽早行规范CRS+HIPEC治疗，有望取得良好的预后。      

完全肿瘤细胞减灭术加全盆腔切除加腹腔热灌注化疗在腹膜癌合并盆腔肿瘤中的应用

  目的  肿瘤细胞减灭术（cytoreductive surgery,CRS）+全盆腔切除术（total pelvic exenteraction,TPE）+腹腔热灌注化疗（hyperthermic intraperitoneal chemotherapy,HIPEC）整合治疗策略,是目前唯一可能治愈腹膜癌合并晚期盆腔肿瘤的疗法。本研究探讨CRS+TPE+HIPEC适应证、手术方式和技巧,尤其是避免功能性造瘘的重建方法。  方法  数据来源于本中心前瞻性数据库,选取2006年9月至2021年1月,共1 172例腹膜癌患者,累计接受CRS+HIPEC治疗1 314例次,其中14例接受TPE且无造瘘功能重建,纳入研究对象。  结果  14例患者均通过腹膜切除术实现了腹部肿瘤细胞减灭程度（completeness of cytoreduction,CC）评分0（CC 0）切除,通过TPE实现了盆腔R0切除,且无任何形式造瘘。无消化道吻合口病发症,术后尿漏5例,其中3例无需有创修补,1例行肾造瘘术,1例二次手术修补。无术后90天内死亡。  结论  CRS+HIPEC手术中,TPE非绝对禁忌。在高度专业化的腹膜癌中心,肿瘤病理学家和专业手术团队联合,可提高CRS+TPE+HIPEC整合治疗策略的安全性和有效性,促进术后恢复,提高患者生活质量。CRS+TPE+HIPEC整合治疗策略的应用仍有待进一步深入研究。      

细胞减灭术联合腹腔热灌注化疗治疗恶性腹膜间皮瘤20例分析

  目的  探讨肿瘤细胞减灭术（cytoreductive surgery,CRS）联合腹腔热灌注化疗（hyperthermic intraperitoneal chemotherapy,HIPEC）治疗恶性腹膜间皮瘤（malignant peritoneal mesothelioma,MPM）的围手术期安全性及其疗效。  方法  回顾性分析2015年1月至2020年6月于航天中心医院接受治疗的20例MPM患者的临床病理资料,评价CRS+HIPEC治疗模式安全性,通过单因素及多因素统计分析研究影响患者生存的预后因素。  结果  20例患者中男性8例,女性12例,男女比例为:1:1.5。病理结果均为MPM,病理分型为上皮型。接受CRS+HIPEC治疗后总体1、2、3年生存率分别为73.9%、58.2%和43.6%,其中肿瘤细胞减灭程度（completeness of cytoreduction,CC）达到0/1的患者3年生存率为75%。单因素分析结果显示,性别（P=0.295）,CA125异常（P=0.256）,既往手术（P=0.460）以及静脉化疗（P=0.283）未见对总生存有显著影响。而年龄 > 60岁（P=0.037）,CC-2/3（P=0.027）,腹膜癌指数（peritoneal cancer index,PCI）≥20分（P=0.014）为预后不良的危险因素。  结论  MPM是一种罕见疾病,早期诊断困难,传统治疗预后差,静脉化疗无法有效改善预后。尽早行CRS+HIPEC综合治疗安全可靠,可以明显延长患者生存。      

60例阑尾源性高级别黏液腺癌生存预后分析

  目的  探讨阑尾源性高级别黏液腺癌的临床特点并分析影响预后的因素。  方法  回顾性分析航天中心医院2013年1月至2017年12月期间收治的60例阑尾源性高级别黏液腺癌患者的临床及随访资料。  结果  60例阑尾源性高级别黏液腺癌患者中男性占48%（29例），女性占52%（31例）；发病的中位年龄为57（38~74）岁；术前化验血CEA升高者占65%（39例），术后行腹腔热灌注化疗者占80%（48例）。术后1、2、3年生存率分别为80%、58%、30%。中位生存时间为29个月。单因素分析腹膜癌指数（peritoneal cancer index, PCI）评分低及细胞减灭程度（completeness of cytoreduction，CC）愈小者对延长生存期有统计学意义（P=0.039，P=0.002）。多因素分析显示CC可作为影响总生存的独立预后因素（P=0.037）。  结论  阑尾源性高级别黏液腺癌具有高度侵袭性，预后较差，彻底减瘤对延长患者的生存有积极作用。早期发现、早期干预对患者预后有重要意义。      

基于真实世界数据的腹膜假黏液瘤诊治现状分析

  目的  基于真实世界数据分析腹膜假黏液瘤患者的诊治现状、自然病程及预后因素。  方法  回顾性分析2009年2月至2020年7月在首都医科大学附属北京世纪坛医院就诊的具有完整自然病程的腹膜假黏液瘤患者的相关资料,包括临床病理特征、非规范化治疗情况（误诊时间、误治时间、既往抗肿瘤治疗情况）、肿瘤细胞减灭术+腹腔热灌注化疗（cytoreductive surgery+hyperthermic intraperitoneal chemotherapy,CRS+HIPEC）治疗情况[手术时间、术中输血情况、腹膜癌指数（peritoneal cancer index,PCI）评分、细胞减灭程度（completeness of cytoreduction,CC）评分、脏器切除数量、腹膜切除区域数量、严重不良事件（serious adverse event,SAE）等]、随访生存时间,随访终点为患者死亡。采用Kaplan -Meier法绘制生存曲线,组间比较采用Log-rank检验。影响5年生存的预后因素采用Cox比例风险回归模型进行单因素和多因素分析。  结果  共纳入94例患者,其中男性57例（60.6%）,女性37例（39.4%）, 中位年龄54（24～76）岁,既往抗肿瘤治疗者59例（62.8%）,中位误诊时间0.8（0～62.5）个月,中位误治时间15.3（0～214.8）个月。所有患者均行CRS+HIPEC治疗,中位手术时间10.1（4.8～16.5）h,中位脏器切除数2（0～8）个,中位腹膜切除区域数5（0～9）个,中位PCI评分32（3～39）分,CC评分2～3分者达80.9%（76/94）,SAE发生率35.1%（33/94）。94例患者中位总生存期30.8（2.4～218.4）个月,1、2、3、5年生存率分别为96.8%、63.8%、44.7%和23.4%。分层分析显示,既往腹腔化疗（46.5 个月vs. 26.3个月）、PSS 1～3分（39.0个月 vs. 21.9个月）、低/高级别病理类型（41.5/40.9 个月vs. 20.1个月）、KPS≥80分（41.5 个月vs. 23.9个月）、无淋巴结转移（35.5 个月vs. 17.1个月）、Ki-67<50%（46.4 个月vs. 20.8个月）的患者中位生存时间延长（P<0.05）。5年生存预后分析中,单因素分析显示以下5个因素与5年生存率有关:PSS评分（P=0.021）、既往腹腔化疗（P=0.008）、病理类型（P=0.004）、淋巴结转移（P=0.008）和Ki-67表达程度（P=0.003）。多因素分析显示出以下3个影响5年生存的独立预后因素:既往腹腔化疗（HR=0.458,95%CI:0.253～0.827,P=0.010）、淋巴结转移（HR=2.879,95%CI:1.345～6.163,P=0.006）、Ki-67≥50%（HR=2.502,95%CI:1.418～4.417,P=0.002）。  结论  PMP非规范化治疗现象较普遍,误治时间长,淋巴结转移及Ki-67高表达是独立不良预后因素,CRS+HIPEC术前腹腔灌注化疗可能为PMP的治疗提供新的方向。      

肿瘤细胞减灭加腹腔热灌注化疗术后静脉血栓栓塞症的预防

  目的  评估行肿瘤细胞减灭术（cytoreductive surgery，CRS）加腹腔热灌注化疗（hyperthermic intraperitoneal chemotherapy，HIPEC）治疗的腹膜癌患者静脉血栓栓塞症（venous thromboembolism，VTE）发生风险，研究术后早期主/被动活动联合间歇充气加压按摩对VTE的预防效果。  方法  对2015年5月至2016年8月武汉大学中南医院肿瘤科收治的120例胃肠道及妇科肿瘤等来源的腹膜癌患者行CRS+HIPEC治疗，使用Caprini血栓风险评估模型评价VTE风险，所有患者采取早期肢体主/被动活动及间歇充气加压按摩治疗，记录分析VTE相关事件。  结果  患者中位Carprini评分为12（10~16）分，均为VTE极高危组，在3个月的随访中仅1例患者发生深静脉血栓，经药物治疗后痊愈。  结论  腹膜癌患者VTE风险极高，术后早期足背曲/跖曲及扩胸等主/被动运动联合间歇充气加压按摩，可有效预防VTE。      

CRS对比CRS+IP与CRS+HIPEC治疗上皮性卵巢癌辅助化疗时间及预后的前瞻性观察研究

  目的  完全肿瘤细胞减灭术（cytoreductive surgery,CRS）及术后辅助化疗,是晚期原发性上皮性卵巢癌（epithelial ovarian cancer,EOC）的重要预后因素。然而,EOC患者接受辅助化疗开始时机及其与预后的关系尚不明确,值得深入探讨。  方法  本研究选取2010年1月至2017年12月在印度班加罗尔马尼帕尔综合癌症中心接受完全细胞减灭术的185例晚期原发性EOC患者,其中部分患者接受腹腔化疗或辅助化疗,部分患者未接受腹腔化疗和辅助化疗。分别记录并分析术后开始进行辅助化疗的时间及其对预后的影响。  结果  接受单纯CRS、CRS联合经腹腔港的腹腔内化疗（intraperitoneal chemotherapy,IP）或腹腔热灌注化疗（hyperthermic intraperitoneal chemotherapy,HIPEC）的患者分别为50例、60例和75例。CRS组术后开始接受辅助化疗的平均时间为32天,CRS+IP组为34天,CRS+HIPEC组为41天。CRS组中,术后化疗间隔时间>42天对无复发生存（relapse-free survival,RFS）期有显著影响（36个月 vs. 17个月:P=0.02）;在CRS+IP组中,患者RFS差异无统计学意义（35个月vs. 28个月;P=0.78）;CRS+HIPEC组,RFS差异无统计学意义（35个月 vs. 32个月;P=0.17）。如期行辅助化疗患者生存期较延迟化疗更好（88个月 vs. 71个月,P=0.49）。  结论  化疗时间延迟是单纯接受完全CRS患者RFS的不良预后因素。与非HIPEC组相比,化疗时间延迟对HIPEC组患者并未产生显著影响,其原因可能在于术中单次HIPEC弥补了化疗时间延迟带来的不利影响。      

Misdiagnosis of appendiceal neoplasms as ovarian tumors: Impact of prior gynecologic surgery on definitive cytoreduction and HIPEC

BackgroundFemale patients with pelvic/adnexal masses often undergo gynecologic operations due to presumed ovarian origin. The diagnosis of an appendiceal tumor is often only made postoperatively after suboptimal cytoreduction has been performed. We hypothesized that an index gynecological procedure increases the morbidity of definitive cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) in patients with appendiceal mucinous tumors.MethodsA single-center retrospective review was performed to identify female patients undergoing CRS/HIPEC for appendiceal tumors from 2012 to 2020.ResultsDuring the 8-year period, CRS/HIPEC was performed in 36 female patients with appendiceal mucinous tumors. Eighteen patients (50.0%) had received a prior pelvic operation by gynecologists (PPO Group) for presumed ovarian origin before referral for definitive CRS/HIPEC. The median peritoneal cancer index (PCI) was higher in the PPO group (21 vs. 9, p = 0.04). The median number of days from gynecologic procedure to definitive CRS/HIPEC was 169 days. Compared to patients who did not undergo a prior gynecologic operation, those in the PPO group had higher intraoperative blood loss (650 vs 100 mL, p < 0.01) during CRS/HIPEC as well as longer length of stay (12 vs 8 days, p = 0.02) and higher overall morbidity (72.3% vs 33.3%, p = 0.02). After controlling for PCI, prior gynecologic operation increased risk of 30-day morbidity after definitive CRS/HIPEC (OR 11.6, p < 0.01).ConclusionA multi-disciplinary approach is needed for the primary evaluation of patients with pelvic masses of undetermined origin. A gynecological resection is associated with increased morbidity during definitive cytoreduction and HIPEC for appendiceal mucinous tumors.     

201 consecutive cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) procedures in a single Asian tertiary centre

Introduction: Peritoneal carcinomatosis (PC) is increasingly being treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). We provide a review of a high-volume Asian institute's experience and survival outcomes with this procedure.

Methods: Data were prospectively collected from 201 consecutive CRS and HIPEC procedures performed in a single institution between April 2001 and November 2015. Our primary endpoints were overall survival (OS) and disease-free survival (DFS), and secondary endpoints were morbidity and mortality.

Results: 77% of patients were Chinese, 9% were Malay, 6% were Indian and 8% were other ethnicities. Primary tumours were colorectal (30%), ovarian (32%), appendiceal (20%), primary peritoneal (6.5%), mesothelioma (4.5%) and others (5%). The median peritoneal cancer index (PCI) was 12, and 92% of patients achieved a completeness of cytoreduction score (CC) of 0. High-grade morbidity occurred in 25.8% of cases, and there were no 30-day mortalities. At 5-years, the OS was 55.1% and DFS was 20.3%. Factors associated with improved OS on multivariate analysis were PCI <15 (p?p?=?0.016).

Conclusions: The combined treatment of CRS and HIPEC is beneficial and is associated with reasonable morbidity and mortality in Asian patients with PC from colorectal, ovarian, appendiceal, primary peritoneal and mesothelioma primaries. Complete cytoreduction and extent of disease are the most important prognostic factors for survival.     

完全肿瘤细胞减灭术加腹腔热灌注化疗治疗胃癌腹膜转移的病例筛选策略

目的:构建肿瘤细胞减灭程度(completeness of cytoreduction,CC)预测模型,为肿瘤细胞减灭术(cytoreductive surgery,CRS)加腹腔热灌注化疗(hyperthermic intraperitoneal chemotherapy,HIPEC)治疗胃癌腹膜转移(gastric cancer with peritoneal metastasis,GCPM)提供病例筛选方法。方法:比较完全CRS(complete CRS,CCRS)组和不完全CRS(incomplete CRS,ICRS)组患者基本临床病理特征和治疗参数,通过逻辑回归模型筛选CC独立预测因子,精准预测CCRS可能性。结果:125例患者纳入本研究,其中CC0组52例(41.6%),中位总生存期为30.0(95%CI:16.8~43.3)个月;CC1-3组73例,中位总生存期7.3(95%CI:5.7~8.8)个月,差异有统计学意义(P<0.001),而CC1、CC2和CC3组间中位总生存期差异无统计学意义(P>0.05)。因此,CC0定义为CCRS组,CC1-3定义为ICRS组,构建并优化了以腹膜转移时相(OR=14,95%CI:2.0~97.9,P=0.008)、术前肿瘤标志物(OR=6.5,95%CI:2.1~37.8,P=0.037)和腹膜癌指数(OR=1.5,95%CI:1.3~1.8,P<0.001)预测ICRS的多因素回归模型和预测列线图,内部验证显示,ROC曲线下面积为0.985,列线图显示预测准确度、一致性良好。根据列线图结果将患者分为4个亚组,设定CCRS预测概率≥50%,同时性且术前肿瘤标志物正常组、同时性且术前肿瘤标志物异常组、异时性且术前肿瘤标志物正常组、异时性且术前肿瘤标志物异常组腹膜癌指数界值点分别为:≤16、≤12、≤10和≤5。结论:CCRS+HIPEC可延长部分经选择的GCPM患者生存期,以腹膜癌指数为核心,联合腹膜转移时相和术前肿瘤标志物的病例筛选策略,可有效选择高概率实现CCRS的患者接受CCRS+HIPEC治疗。     

Psoas/iliacus muscle invasion from mucinous appendiceal neoplasm. Radiologic appearance and outcome of treatment in 3 patients

Background

Mucinous appendiceal neoplasms have a pattern of metastases that is different from the other gastrointestinal cancers. The first site for cancer dissemination is the peritoneal space surrounding the primary tumor and this is followed by increasingly extensive peritoneal spread. Invasion of the psoas and iliacus muscle is an unusual phenomenon.

Repeated cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in peritoneal carcinomatosis from appendiceal cancer: Analysis of survival outcomes

Background

Cytoreductive surgery (CRS)/hyperthermic intraperitoneal chemotherapy (HIPEC) is the procedure of choice in patients with peritoneal dissemination from appendiceal cancer. Although recurrence rates are 26%–44% after first CRS/HIPEC, the role of repeated CRS/HIPEC has not been well defined. We hypothesize that patients undergoing multiple CRS/HIPEC's have meaningful long term survival.

Methods

A retrospective study of a prospective database of 294 patients with peritoneal carcinomatosis (PC) was conducted, of these 162 had PC of appendiceal origin. Twenty-six of these patients underwent 56 CRS/HIPEC. Survival and outcomes was analyzed.

Results

The percentage of patients with pre-surgical PCI scores ≥20 for the first, second, and third CRS/HIPEC was 65, 65, and 25%, respectively. Complete cytoreduction (CC 0-1) at first, second, and, third surgeries was 96, 65 and 75%, respectively.The mean operating time was 10.1 h. There was no 30-day peri-operative mortality. Following the first, second, and third CRS/HIPEC 27, 42, and 50% experienced grade III complications, respectively.Mean follow up was 51, 28, and 16 months from the first, second, and third CRS/HIPEC, respectively. Overall survival rate for the first CRS/HIPEC was 100, 83, 54, and 46% at years 1, 3, 5 and 10, respectively; from the second CRS/HIPEC 91, 53, and 34% at 1, 3, and 5 years, respectively; and from the third CRS/HIPEC was 75% at one year.

Conclusion

Repeat CRS/HIPEC can lead to meaningful long term survival rates in patients with appendiceal peritoneal carcinomatosis with morbidity and mortality similar to those of the initial CRS/HIPEC.     

Clinical utility of elevated tumor markers in patients with disseminated appendiceal malignancies treated by cytoreductive surgery and HIPEC

Background

Appendiceal malignancies with peritoneal spread have been successfully treated with Cytoreductive Surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). The aim of this study is to clarify the utility of common tumor markers in selecting patients for the combined treatment.

Methods

Data on 56 patients with appendiceal neoplasms treated with CRS and HIPEC were prospectively collected. Chi square test was used to analyze a link between common tumor markers and completeness of cytoreduction score (CC score) and preoperative peritoneal cancer index score (PCI score). Cox proportional hazard model was used to perform survival analysis.

Results

Forty-two patients were alive after 3 years of follow-up. Hazard ratio of disease related death was 5.6 (95% CI, 1.8–17.2) among patients with high CC score as compared to those with low CC score. Number of abnormal tumor markers (0 vs 1/2/3) correlated with PCI score 16.2 vs 32.5 (p < 0.001) but not with completeness of cytoreduction or survival. The 3-year survival rates in patients with normal vs abnormal CA 125 levels were 83% vs 52%(p = 0.003).

Conclusions

Multiple abnormal tumor markers were not useful as an exclusion criterion for patients undergoing CRS. Elevation in CA 125 was an important indicator of survival in these patients. Complete cytoreduction was crucial for long-term survival.     

Pathogenesis of histologic variations of appendiceal mucinous neoplasms

BackgroundIn order for peritoneal metastases from a primary appendiceal mucinous neoplasm to occur, the wall of the appendix must perforate to allow mucus with tumor cells access to the peritoneal spaces. With progression the peritoneal metastases show a broad spectrum of tumor biology varying from indolent to aggressive activity.MethodsThe histopathology of peritoneal tumor masses was determined from the clinical material resected at the time of cytoreductive surgery (CRS). All groups of patients were treated by a uniform strategy that involved complete CRS and perioperative intraperitoneal chemotherapy. Overall survival was determined.ResultsFrom a database of 685 patients, four histologic subtypes were identified and long-term survival determined. Four hundred and fifty patients (66.0%) had low-grade appendiceal mucinous neoplasm (LAMN), 37 patients (5.4%) had mucinous appendiceal adenocarcinoma of intermediate subtype (MACA-Int), 159 patients (23.2%) had mucinous appendiceal adenocarcinoma (MACA), and 39 patients (5.4%) had a mucinous appendiceal adenocarcinoma with positive lymph nodes (MACA-LN). The mean survival of the four groups was 24.5, 14.8, 11.2 and 7.4 years, respectively (p < 0.0001). These four subtypes of mucinous appendiceal neoplasms were shown to have distinct survival estimates.ConclusionsThe estimated survival of these four histologic subtypes in patients having a complete CRS plus HIPEC is of value to the oncologist managing these patients. A mutations and perforations hypothesis was offered in an attempt to explain the broad spectrum of mucinous appendiceal neoplasms that exist. Inclusion of MACA-Int and MACA-LN as standalone subtypes was thought to be necessary.