Smeenk R M, Verwaal V J, Zoetmulder F A N
Nederlands Kanker Instituut-Antoni van Leeuwenhoek Ziekenhuis, afd. Chirurgie, Amsterdam.
Ned Tijdschr Geneeskd. 2007 Feb 17;151(7):418-23.
Three patients, men aged 47 and 30 and a woman aged 48 years, presented with appendicitis-like symptoms, progressive abdominal distension, and an ovarian pelvic mass, respectively. All three were diagnosed with pseudomyxoma peritonei. Treatment consisted of cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC). After a mean follow-up of 64 months (range: 19-89) after initial treatment, the second patient had died of disease progression but the other two were alive and free of disease. Pseudomyxoma peritonei is a rare disease, characterised by progressive intraperitoneal accumulation of mucinous ascites, produced by a mucinous tumour mass on the peritoneal surfaces. The primary tumour is usually a mucinous adenoma of the appendix. Treatment by cytoreductive surgery and HIPEC can result in a 5-year survival rate of more than 80%, depending on the histopathology and the completeness of the cytoreduction. Recognising pseudomyxoma peritonei in an early stage, when complete cytoreduction is still achievable, may result in a considerable improvement in survival.
三名患者,分别为47岁男性、30岁男性和48岁女性,分别表现为阑尾炎样症状、进行性腹胀和卵巢盆腔肿块。三人均被诊断为腹膜假黏液瘤。治疗包括细胞减灭术,随后进行腹腔热灌注化疗(HIPEC)。在初始治疗后平均随访64个月(范围:19 - 89个月),第二名患者死于疾病进展,但另外两名患者存活且无疾病。腹膜假黏液瘤是一种罕见疾病,其特征是腹膜表面的黏液性肿瘤团块产生的黏液性腹水在腹腔内进行性积聚。原发性肿瘤通常是阑尾黏液性腺瘤。根据组织病理学和细胞减灭的完整性,通过细胞减灭术和HIPEC治疗可使5年生存率超过80%。在仍可实现完全细胞减灭的早期阶段识别腹膜假黏液瘤,可能会使生存率得到显著提高。