661 - Tumors of the Appendix: the Experience of Ipo-Porto

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ABSTRACT Introduction Malignant neoplasms of ileocecal appendix are rare, an clinical features are unspecific, often mimicking acute appendicitis. They are diagnosed in about 1% of appendicectomy specimens. The most common histologic types are mucinous adenocarcinoma, intestinal-type adenocarcinoma and carcinoid tumor. Methods The authors reviewed the primary appendix carcinomas treated in Instituto Portugues de Oncologia Francisco Gentil-Porto (IPOFG-Porto) between January 2000 and June 2011. Results Fourty two patients were treated during this period: 30 women (71%) and 12 men, aged between 20 and 90 years old (median age 58). Mucinous adenocarcinoma was the most common histologic subtype, with 27 cases (64%), followed by adenocarcinoma NOS (10 cases - 24%); carcinoids were 5 cases (12%). Fifteen percent presented as stage IV, 7% stage III, 24% stage II and 19% stage I. The primary treatment was surgery: 16 patients were submitted to appendicectomy, 6 patients to appendicectomy, ooforectomy and hysterectomy, 12 to hemicolectomy. Of patients submitted to appendicectomy, 11 (50%) had a second laparotomy in order to perform hemicolectomy. Five percent of the patients received adjuvant chemotherapy (mFOLFOX6). Hyperthermic Intraoperative Peritoneal Chemotherapy (HIPEC) was performed in 18 patients with stage IV disease (peritoneal carcinomatosis). The median follow-up was 35 months. Thirty three percent of the patients (n = 14) died because of disease progression and 62% (n = 26) were alive with no evidence of disease. Conclusion Appendiceal carcinomas are rare tumours and there is limited evidence on the indications for right hemicolectomy. The role of adjuvant chemotherapy for adenocarcinoma of the appendix is unknown. Optimal treatment of patients with intraperitoneal dissemination of appendiceal adenocarcinoma (mucinous peritoneal carcinomatosis) is unclear. Selected patients treated with aggressive surgical cytoreduction and HIPEC may have a favorable outcome in long term, but patient selection and the experience of the treating team are critical. The benefit of systemic chemotherapy for advanced disease is unknown. Disclosure All authors have declared no conflicts of interest.