Division of Hematology/Oncology, Karmanos Cancer Institute, Wayne State University, 4100 John R, Detroit, MI 48201, USA.
Med Oncol. 2010 Dec;27(4):1073-8. doi: 10.1007/s12032-009-9337-2. Epub 2009 Oct 23.
Appendiceal carcinomas are classified into three distinct histopathological disease entities: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), or peritoneal mucinous carcinomatosis with intermediate or discorant features (PMCA I/D). The treatment of appendiceal tumors should be based on accurate histopathological classification, per previously reported case series. The objective of this study was to evaluate the clinicopathologic presentation and outcome of patients with appendiceal tumors treated at our institution over a 15-year period. We identified patients with appendiceal tumors diagnosed or treated at our institution from January 1989 through May 2004. Chart review for age, gender, signs and symptoms at diagnosis, and treatment was performed. Review of the pathologic specimens was performed and tumors were classified as DPAM, PMCA I/D, or PMCA. Forty patients were identified (median age 52.5 years; males 38%). The number of patients with DPAM, PMCA I/D, and PMCA was 15 (38%), 6 (15%), and 18 (46%), respectively. Peritoneal involvement was seen in 11 (73%) of patients with DPAM, 5 (83%) of PMCA I/D, and 11 (61%) of PMCA. The median survival for patients with DPAM, PMCA I/D, and PMCA was 7.7 years (90% CI: 2.9--upper limit not estimable), 1.2 years (90% CI: 0.9-1.6), and 0.7 years (90% CI: 0.4-1.5), respectively. The difference in survival across the three groups was statistically significant. Three distinct histopathological disease entities exist in appendiceal tumors. The prognosis and management of these tumors should be based on the extent of disease and pathologic diagnosis.
弥漫性腹膜黏液腺癌(DPAM)、腹膜黏液性癌(PMCA)或具有中间或不相符特征的腹膜黏液性癌(PMCA I/D)。阑尾肿瘤的治疗应基于准确的组织病理学分类,这是根据之前的病例系列报告得出的。本研究的目的是评估在我们机构治疗的阑尾肿瘤患者的临床病理表现和结局。我们确定了 1989 年 1 月至 2004 年 5 月在我们机构诊断或治疗的阑尾肿瘤患者。对年龄、性别、诊断时的体征和症状以及治疗进行了图表回顾。对病理标本进行了检查,并将肿瘤分为 DPAM、PMCA I/D 或 PMCA。共确定了 40 例患者(中位年龄 52.5 岁;男性 38%)。DPAM、PMCA I/D 和 PMCA 的患者数量分别为 15 例(38%)、6 例(15%)和 18 例(46%)。DPAM 患者中有 11 例(73%)存在腹膜受累,PMCA I/D 患者中有 5 例(83%),PMCA 患者中有 11 例(61%)。DPAM、PMCA I/D 和 PMCA 患者的中位生存时间分别为 7.7 年(90%CI:2.9-上限不可估计)、1.2 年(90%CI:0.9-1.6)和 0.7 年(90%CI:0.4-1.5)。三组间的生存差异具有统计学意义。阑尾肿瘤存在三种不同的组织病理学疾病实体。这些肿瘤的预后和管理应基于疾病的范围和病理诊断。