Varona J F, Guerra J M, Salamanca J, Colina F, Lopez G, Morales M
Department of Internal Medicine, 12 de Octubre University Hospital of Madrid, Spain.
Hepatogastroenterology. 2005 May-Jun;52(63):812-6.
BACKGROUND/AIMS: Pseudomyxoma peritonei is an uncommon disease characterized by the presence of mucinous peritoneal implants associated with an abdominal neoplasm. Our objective is to consider the characteristics of this entity in our western Mediterranean urban population.
All cases diagnosed with pseudomyxoma peritonei by our hospital during a period of 16 years were reviewed. Data from their clinical records and the biopsy samples were analyzed.
We found 21 cases of pseudomyxoma peritonei with a male/female ratio of 10/11 and a mean age of 59 years. The predominant presentation symptom was abdominal pain (17 cases, 6 of them with acute abdomen). The most frequent primary site of origin of the pseudomyxoma was the appendix (10 cases). The histologic diagnosis was malignant (associated with carcinoma) in 17 cases and indeterminate behavior in 4. The follow-up was available for 15 patients (mean follow-up of 41 months), while six patients have been lost. Nine patients have died during the follow-up and the other 6 patients are still alive after follow-up.
Laparotomy is the main tool for diagnosing pseudomyxoma peritonei. The appendix is the most frequent primary site of origin of pseudomyxoma peritonei, followed by bowel; the latter being more important than previously described. In most cases the histology is malignant. The prognosis is bad with a mortality greater than 60% at 5 years.
背景/目的：腹膜假黏液瘤是一种罕见疾病，其特征为存在与腹部肿瘤相关的黏液性腹膜种植。我们的目的是研究在地中海西部城市人群中该疾病的特征。
回顾我院在16年期间诊断为腹膜假黏液瘤的所有病例。分析其临床记录和活检样本数据。
我们发现21例腹膜假黏液瘤，男女比例为10/11，平均年龄59岁。主要表现症状为腹痛（17例，其中6例为急腹症）。腹膜假黏液瘤最常见的原发部位是阑尾（10例）。组织学诊断为恶性（与癌相关）17例，行为不确定4例。15例患者有随访资料（平均随访41个月），6例失访。随访期间9例患者死亡，另外6例随访后仍存活。
剖腹手术是诊断腹膜假黏液瘤的主要手段。阑尾是腹膜假黏液瘤最常见的原发部位，其次是肠道；肠道的重要性比之前描述的更高。大多数病例组织学为恶性。预后较差，5年死亡率超过60%。
