The prognostic relevance of histologic subtype in appendiceal adenocarcinoma

BackgroundThe aim of this population-based study was to determine the prognostic value of the histologic subtypes mucinous (MAC), non-mucinous (AC) and signet ring cell (SRCC) adenocarcinoma among patients with appendiceal cancer.Methods and materialsData from the Netherlands Cancer Registry (NCR) of patients with primary appendiceal adenocarcinomas with MAC, AC and SRCC histologic subtype, diagnosed between 2001 and 2015 were used (n = 675). To categorize patients according to the recent histopathological classification, the NCR was linked with the Dutch Pathology Registry (PALGA). Log-rank tests and Kaplan-Meier analyses were performed to estimate overall survival (OS), and the cox proportional hazards model was run to identify prognostic factors.ResultsAC was the most frequently encountered histologic subtype (50.9%), followed by MAC (35.8%) and SRCC (13.3%). In locoregional disease, histologic subtype was not a prognostic factor for OS with 5-year survival rates for patients with AC, MAC and SRCC of 60.0%, 60.5% and 69.6% respectively (p = 0.68). Metastatic disease was more common in SRCC (53.8%) than in MAC (38.8%) and AC (23.4%) (p < 0.0001). Median OS for patients with metastatic disease was 12.6, 27.7 and 18.2 months in AC, MAC and SRCC respectively (p < 0.005). MAC was associated with higher survival compared to AC (HR 0.48, 95%CI 0.34–0.69). In subanalyses, MAC was only a positive prognostic factor compared to AC in patients with peritoneal metastases (HR 0.42, 95%CI 0.28–0.62).ConclusionHistologic subtype had no prognostic relevance in locoregional or systemic metastatic disease in appendiceal adenocarcinoma. In peritoneal metastases, mucinous histologic subtype was a favorable prognostic factor, compared to non-mucinous and signet ring cell subtype.     

Peritoneal MRI in patients undergoing cytoreductive surgery and HIPEC: History,clinical applications,and implementation

MR imaging provides considerable advantages in the evaluation of patients with peritoneal metastases. A standardized peritoneal MRI protocol, including diffusion-weighted and gadolinium-enhanced sequences, allows an efficient exploration of small peritoneal tumors that are often missed on other imaging tests. In experienced hands, a dedicated reading allows producing a quantitative and qualitative evaluation of lesional localization to better assist surgeons in the selection of candidates for curative surgery by evaluating the possibility of complete resection, and to plan the surgical procedure. Based on a close collaboration between oncologic surgeon and radiologist, MRI provides a powerful tool for accurate preoperative imaging in patients being considered for curative surgery but also in their surveillance to detect an early recurrence.     

Sclerosing encapsulating peritonitis as a potential complication of cytoreductive surgery and HIPEC: Clinical features and results of treatment in 4 patients

Sclerosing encapsulating peritonitis (SEP) is a rare entity characterized by encapsulation of the small bowel and/or the colon by a fibrous tissue that forms a shell. Intraperitoneal chemotherapy (IPC) has been reported to be a potential causative factor of secondary SEP. However, few studies have reported on secondary SEP related to cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Here, we review results from four clinical cases of SEP associated with CRS and HIPEC. In all four patients, additional surgery was necessary to alleviate recurrent episodes of small bowel obstruction. These obstructions can occur as early as several weeks after CRS plus HIPEC or as late as 3 years after treatment. Of utmost importance is the prevention of fistulization which can result in enteric contamination of the peritoneal space. To date, no solution to SEP has been identified except additional surgery but it is evident that these reoperative experiences are difficult for both surgeon and patient. The etiopathogenesis of SEP in this setting remains unknown but it is clear that it is related to chronic inflammation of the peritoneum. Large studies are needed to identify the incidence and potential common causes of SEP after CRS and HIPEC.     

Pseudomyxoma peritonei: a case report and current concepts in the literature

This report evaluates the natural history and clinicopathologic variables of patients with pseudomyxoma peritonei (PMP), and reviews the authors' experience with different treatments in the current literature. PMP results from implantation of malignant tumors or peritoneal irritation caused from ruptured benign cysts. This entity is traditionally characterized by the accumulation of mucinous ascites and a relatively long survival period. Diffuse peritoneal spread occurs in most patients with PMP, and distant metastasis is infrequent. Disease progression is difficult to predict because of the spectrum of underlying pathologic processes with varying malignant potential. Debulking surgery, radiation therapy and chemotherapy have all been advocated for optimal patient management. Received: 26 January 1999 / Accepted in revised form: 1 September 1999     

复发性腹膜假黏液瘤病理分析

目的 探讨复发性腹膜假黏液瘤（PMP）病理级别的变化,分析复发前病理级别同P53表达的相关性。方法 回顾分析2008年1月~2016年4月74例复发手术并行病理检查≥2次腹膜假黏液瘤临床病理资料,按照2010年WHO标准,分别分为低级别腹膜假黏液瘤及高级别腹膜假黏液瘤,观察复发前、后级别变化情况,并分析复发前次手术病理级别同P53表达的关系。结果 74例复发病例中,复发前后级别不变,提高、降低的病例分别为65例,6例,3例。复发前低级别、高级别病变P53阳性率分别为9.58%±15.02%及47.46%±34.18%,相关系数为0.521（P=0.000）;Logistic分析显示P53变量偏回归系数为0.054,差异均具有统计学意义（P=0.000）。高级别腹膜假黏液瘤P53阳性率高,而低级别PMPP53阳性率低。结论 腹膜假黏液瘤自然病程长,复发率高,复发前病理级别可作为复发后选择术中治疗方案的参考;P53指标可作为评价级别的指标之一。     

腹膜假性粘液瘤的诊断及治疗

探讨腹膜假性粘液瘤的诊断及治疗方法,并回顾有关文献。对北京协和医院１９８３～１９９８年收治并确诊 的１７例腹膜假性粘液瘤病例进行回顾性分析研究。结果：１７例患者中,男性１例,女性１６例。年龄２０～７１岁, 平均５２岁。１７例患者中,１４例伴卵巢粘液性囊腺瘤,其中４例合并阑尾粘液囊肿。１７例患者中包括１例卵 巢浆液性囊腺瘤,１例卵巢粘液腺癌,１例男性阑尾粘液囊肿。所有病人均经手术治疗１～６次,平均每人２．５次。 ２次手术间隔７个月～７年４个月,平均３．６年。术后１年内死亡３例。结论：尽管腹膜假性粘液瘤被认为是一 种分化较好的低度恶性或交界性肿瘤,但复发是极常见的现象,远处转移几乎不存在。其来源最多见于卵巢 和阑尾。外科手术切除仍是目前最有效的治疗手段。     

阑尾低级别黏液性肿瘤临床诊治的研究进展

目的 总结阑尾低级别黏液性肿瘤的临床诊治的研究进展。方法 在中国知网、万方数据和PubMed、Web of Science等数据库,以“阑尾低级别黏液性肿瘤” “阑尾黏液性肿瘤” “腹膜假性黏液瘤”和“low-grade appendiceal mucinous neoplasm” “appendiceal mucinous neoplasm” “pseudomyxoma peritonei”为关键词,检索2018年8月以前发表的有关阑尾低级别黏液性肿瘤临床诊治的文献,进行归纳总结。结果 阑尾低级别黏液性肿瘤是阑尾上皮性肿瘤的主要病理类型之一,主要特点是不同原因引起阑尾管腔的慢性炎症导致管腔的狭窄,黏液物质不断聚集,在腔内压力不断增加的基础下,黏液侵及阑尾壁内或突破至壁外。阑尾低级别黏液性肿瘤的病理诊断标准更加形象具体,有助于诊断、病理分期及评估预后;对该肿瘤的临床表现、体征及影像学的认识更加透彻,有助于疾病的早期发现和鉴别诊断;其治疗方式在切除范围及黏液清除程度上有了基本统一的治疗方案。结论 阑尾低级别黏液性肿瘤在临床表现上无特异性,CT及超声的诊断率在逐步提高,该肿瘤具有低度恶性,要做到完整切除肿瘤,即使扩大切除范围也要避免瘤体破裂的风险。     

Mucinous Adenocarcinomas with Intra‐Abdominal Dissemination: A Review of Current Therapy

Peritoneal carcinomatosis has been considered a terminal disease with a median survival time of 5.2–12.6 months. Systemic chemotherapy and cytoreductive surgery (CRS) have long been used to treat macroscopic disease, with limited success. However, a comprehensive treatment approach involving cytroreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) has evolved into a novel approach for peritoneal carcinomatosis. Surgery removes the primary cancer and any dissemination within the peritoneal cavity and adjuvant HIPEC eradicates macroscopic or microscopic tumor residue, thus reducing the risk for recurrence. This approach offers a new potential treatment option for patients with metastatic disease confined to the peritoneum. The present review provides an update of the most recent data on the current therapy for pseudomyxoma peritonei (PMP) and mucinous colorectal adenocarcinoma (MCA) with metastatic disease confined to the peritoneum.     

腹膜假性黏液瘤手术后复发诊治问题

腹膜假性黏液瘤是一种易复发的罕见病。该病最初被用于描述阑尾囊腺瘤的腹膜内黏液播散,大量黏液逐渐堆积填充腹腔,产生特征性的胶冻状腹水,也用于表示产黏液的腹盆腔腺癌破裂导致的腹膜黏液播散。其复发监测及治疗是临床一大难题,故有必要加强多学科协作,以改善患者的预后。