Sugarbaker Paul H
Program in Peritoneal Surface Malignancy, Washington Cancer Institute, 106 Irving Street, NW, Suite 3900, WA, DC 20010, USA.
Cancer J. 2009 May-Jun;15(3):225-35. doi: 10.1097/PPO.0b013e3181a9c781.
The appendiceal malignancies usually arise within a mucocele. The tumor within this structure can be minimally aggressive or of an invasive character. If a low-grade appendiceal malignancy is removed intact, recurrence does not occur. If rupture of the wall of the mucocele occurs with either low-grade or high-grade disease, the epithelial cells within will disseminate to the peritoneal surfaces. Sometimes a second-look surgery must be performed to determine if intraperitoneal spread of mucus and cancer cells has occurred. If the diagnosis of peritoneal dissemination of an appendiceal malignancy has been established, a new treatment with curative intent is indicated. Cytoreductive surgery is combined with perioperative intraperitoneal chemotherapy as a comprehensive management plan. In approximately 900 patients treated at the Washington Cancer Institute, the quantitative prognostic indicators for appendiceal cancer with peritoneal dissemination have been determined. Patients with a complete cytoreduction and low-grade tumor have an 80% survival at 20 years; with high-grade tumors, the survival drops to approximately 45%. The extent of malignancy present within the abdomen by the peritoneal cancer index has a significant impact on survival for both high-grade and low-grade disease. The most important indicator is the completeness of cytoreduction; for both high-grade and low-grade disease, all patients with an incomplete cytoreduction have died by 10 years. The presence versus absence of lymph nodal metastases on survival have minimal significance (P = 0.0495). These results of treatment can be achieved with a 19% incidence of severe complications and a 2% mortality.
阑尾恶性肿瘤通常起源于黏液囊肿。该结构内的肿瘤侵袭性可能很低，也可能具有侵袭性。如果完整切除低度阑尾恶性肿瘤，不会复发。如果黏液囊肿壁破裂，无论疾病是低度还是高度，内部的上皮细胞都会播散到腹膜表面。有时必须进行二次手术以确定黏液和癌细胞是否已发生腹膜播散。如果阑尾恶性肿瘤腹膜播散的诊断已经确立，则需要采取有治愈意图的新治疗方法。减瘤手术与围手术期腹腔内化疗相结合作为综合管理方案。在华盛顿癌症研究所接受治疗的约900名患者中，已确定了腹膜播散性阑尾癌的定量预后指标。实现完全减瘤且肿瘤为低度的患者20年生存率为80%；对于高度肿瘤，生存率降至约45%。腹膜癌指数所反映的腹部恶性肿瘤范围对高度和低度疾病的生存均有显著影响。最重要的指标是减瘤的彻底性；对于高度和低度疾病，所有减瘤不彻底的患者在10年内均死亡。有无淋巴结转移对生存的意义极小（P = 0.0495）。这些治疗结果的严重并发症发生率为19%，死亡率为2%。
