Department of Surgery, Saifee Hospital, 15/17, Maharshi Karve Rd, Charni Road East, Opera House, Girgaon, Mumbai, Maharashtra, 400004, India.
Peritoneal Malignancy Program, Department of Surgery, Fondazione IRCCS Istituto Nazionale Dei Tumori, Via Venezian, 1, 20133, Milan, Italy.
Eur J Surg Oncol. 2021 May;47(5):1132-1139. doi: 10.1016/j.ejso.2020.08.017. Epub 2020 Aug 25.
Appendiceal non-mucinous neoplasms (AnMN) are rare and poorly understood malignancies with no standard treatment. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is largely used to treat peritoneal disseminations from appendiceal mucinous neoplasms (AMN), but its role with AnMN is unclear.
A prospective database of 315 patients with advanced appendiceal primaries undergoing CRS/HIPEC during 1996-2020 was reviewed. Baseline characteristics, operative and long-term outcomes of AnMN were compared with those of AMN. AMN were categorized according to PSOGI classification into high-grade, low-grade, and acellular mucin (AC), based on peritoneal disease histology.
Twenty-three patients (7.3%) with goblet cell carcinoma (GCC; n = 9), intestinal-type adenocarcinoma (ITAC; n = 12), and mixed adeno-neuroendocrine carcinoma (MANEC; n = 2) were identified. AnMN patients were more likely to be males (P = 0.006), have preoperative systemic chemotherapy (P = 0.001), grossly incomplete CRS (P = 0.001), and nodal metastases (P = 0.001), but not systemic relapse after CRS/HIPEC (P = 0.133). Median follow-up was 25.1 months (range 0.8-77.3) for AnMN, and 80.9 months (range 0.1-279.2) for AMN. Median overall survival was 24.0 months for AnMN, 66.2 months for high-grade AMN (P = 0.015), 160.0 months for low-grade ANM (P = 0.001), and not reached for AC (P = 0.001). Among AnMN patients, median survival was 23.4 months for GCC, 38.7 months for ITAC, 20.3 months for MANEC (P = 0.855). In the overall series, histological subtype (P = 0.001), incomplete cytoreduction (P = 0.001), and positive lymph-nodes (P = 0.003) correlated with poorer survival at multivariate analysis.
AnMN share with AMN a predominant local-regional dissemination pattern, but prognosis after CRS/HIPEC is worse. This strategy needs to be carefully considered for AnMN.
阑尾非黏液性肿瘤（AnMN）是一种罕见且了解甚少的恶性肿瘤，目前尚无标准治疗方法。细胞减灭术和腹腔热灌注化疗（CRS/HIPEC）主要用于治疗阑尾黏液性肿瘤（AMN）的腹膜播散，但对于 AnMN 的作用尚不清楚。
回顾了 1996 年至 2020 年期间接受 CRS/HIPEC 治疗的 315 例晚期阑尾原发性肿瘤患者的前瞻性数据库。比较了 AnMN 与 AMN 的基线特征、手术和长期结果。根据腹膜疾病的组织学，根据 PSOGI 分类将 AMN 分为高级别、低级别和无细胞黏液（AC）。
确定了 23 例（7.3%）患者患有杯状细胞癌（GCC；n=9）、肠型腺癌（ITAC；n=12）和混合腺-神经内分泌癌（MANEC；n=2）。AnMN 患者更可能为男性（P=0.006）、术前全身化疗（P=0.001）、大体上不完全 CRS（P=0.001）和淋巴结转移（P=0.001），但 CRS/HIPEC 后无全身性复发（P=0.133）。AnMN 的中位随访时间为 25.1 个月（范围 0.8-77.3），AMN 的中位随访时间为 80.9 个月（范围 0.1-279.2）。AnMN 的中位总生存期为 24.0 个月，高级别 AMN 为 66.2 个月（P=0.015），低级别 AMN 为 160.0 个月（P=0.001），AC 未达到（P=0.001）。在 AnMN 患者中，GCC 的中位生存期为 23.4 个月，ITAC 为 38.7 个月，MANEC 为 20.3 个月（P=0.855）。在整个系列中，组织学亚型（P=0.001）、不完全细胞减灭术（P=0.001）和阳性淋巴结（P=0.003）与多变量分析中的较差生存相关。
AnMN 与 AMN 一样，主要表现为局部-区域播散模式，但 CRS/HIPEC 后的预后较差。对于 AnMN，需要仔细考虑这种策略。
