Pathology of Mucinous Appendiceal Tumors and Pseudomyxoma Peritonei

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• 作者：Veena Ramaswamy
• 关键词：Psuedomyxoma peritonei ; Cytoreductive surgery ; Hyperthermic intraperitoneal chemotherapy ; Immunohistochemistry ; Cytokeratin
• 刊名：Indian Journal of Surgical Oncology
• 出版年：2016
• 出版时间：June 2016
• 年：2016
• 卷：7
• 期：2
• 页码：258-267
• 全文大小：544 KB
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• 作者单位：Veena Ramaswamy (1)
  
  1. SRL LAB, Fortis Hospital, No 154/9, Opposite IIM-B, Bannerghatta Road, Bangalore, 560076, India
  
• 刊物主题：Surgical Oncology; Oncology; Surgery;
• 出版者：Springer India
• ISSN：0976-6952

文摘

Neoplasms of the appendix are rare, but because of their unusual presentation and unpredictable biologic behavior, it is important to diagnose them correctly. Mucinous tumors account for 58 % of malignant tumors of appendix in SEER database and the remaining are carcinoids. The mucinous appendiceal tumors have a potential to spread to the peritoneum and viscera in the form of gelatinous material with or without neoplastic cells resulting in Pseudomyxoma peritonei. (PMP) PMP is a clinical entity that has a unique biological behavior and can arise from seemingly benign tumors to frankly malignant ones. Several classifications exist for PMP of which Ronnet’s classification has been the most popular. In 2010, the WHO proposed a 2 tier classification that classified PMP as either low grade or high grade based on the presence of mucin, cytological and architectural features. According to this classification when the underlying cause for PMP is an appendiceal tumor it is always a mucinous adenocarcinoma rather than a mucocoele or adenoma and these terms should no longer be used. This system of classification helps in predicting the behavior of the tumor and proper treatment strategies. The understanding of the pathogenesis of the disease has also improved with identification of newer biomarkers and molecular genetic alterations. IHC markers CK 20, CDX2 and MUC2 are found to be positive in these tumors in addition to KRAS mutation and loss of heterozygosity in some gene loci. Proper histopathologic classification and predicting the tumor behavior requires a close interaction between the pathologist and the surgeon. The use of the combined modality treatment of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) has led to a 5-year survival ranging from 62.5 % to 100 % for low grade, and 0 %-65 % for high grade disease. This article focuses on the etiopathogenesis, clinical behavior, diagnosis and classification of mucinous tumors of the appendix and pseudomyxoma peritonei.