Vidarsdottir Halla, Jónasson Jón Gunnlaugur, Möller Pall Helgi
Laeknabladid. 2011 Oct;97(10):537-42. doi: 10.17992/lbl.2011.10.392.
Adenocarcinoma of the appendix is less than 0.5% of all gastrointestinal cancers. The aim of this study was to analyse the incidence, symptoms, pathology and treatment of appendiceal adenocarcinoma in a well defined cohort as well as the prognosis of the patients.
This is a retrospective study on all patients diagnosed with adenocarcinoma of the appendix in Iceland from 1990-2009. Information on epidemiological factors, survival and treatment was collected. All histological material was reviewed. Overall survival was estimated with median follow up of 15 months (range, 0-158).
A total of 22 patients were diagnosed with appendiceal adenocarinoma in the study period (median age 63 yrs, range: 30-88, 50% males). Age-standardized incidence was 0.4/100,000/year. The most common symptom was abdominal pain (n=10). Eight patients had clinical signs of appendicitis. Most patients were diagnosed at operation or at pathological examination but one patient was diagnosed at autopsy. Five patients had an appendectomy and 11 a right hemicolectomy. One patient was not operated on and in three patients only a biopsy was taken. Twelve patients had chemotherapy and seven of them for metastatic disease. Eight patients had adenocarcinoma, seven mucinous adenocarcinoma, three signet ring adenocarcinoma, one mixed goblet cell carcinoid and mucinous adenocarcinoma,one mixed adenocarcinoma and signet ring adenocarcinoma and two a mucinous tumour of unknown malignant potential. In eight cases the tumor originated in adenoma. Most of the patients had a stage IV disease (n=13), three stage III, three stage II and three stage I. Operative mortality was 4.8% (n=1). Disease specific five year survival was 54% but overall five year survival was 44% respectively.
Adenocarcinoma of the appendix is a rare disease. No patients were diagnosed pre-operatively. Over half of the patients presented with stage IV disease.
阑尾腺癌在所有胃肠道癌症中占比不到0.5%。本研究旨在分析一个明确队列中阑尾腺癌的发病率、症状、病理及治疗情况，以及患者的预后。
这是一项对1990年至2009年冰岛所有诊断为阑尾腺癌患者的回顾性研究。收集了流行病学因素、生存情况及治疗方面的信息。对所有组织学材料进行了复查。总体生存情况通过中位随访15个月（范围0 - 158个月）进行评估。
研究期间共22例患者被诊断为阑尾腺癌（中位年龄63岁，范围30 - 88岁，50%为男性）。年龄标准化发病率为每年0.4/10万。最常见症状为腹痛（n = 10）。8例患者有阑尾炎临床体征。大多数患者在手术时或病理检查时确诊，但1例患者在尸检时确诊。5例患者行阑尾切除术，11例患者行右半结肠切除术。1例患者未接受手术，3例患者仅进行了活检。12例患者接受了化疗，其中7例为转移性疾病化疗。8例为腺癌，7例为黏液腺癌，3例为印戒细胞腺癌，1例为混合性杯状细胞类癌和黏液腺癌，1例为混合性腺癌和印戒细胞腺癌，2例为恶性潜能未知的黏液性肿瘤。8例肿瘤起源于腺瘤。大多数患者为IV期疾病（n = 13），3例为III期，3例为II期，3例为I期。手术死亡率为4.8%（n = 1）。疾病特异性5年生存率为54%，总体5年生存率分别为44%。
阑尾腺癌是一种罕见疾病。术前无患者被诊断。超过半数患者表现为IV期疾病。
