661 - Tumors of the appendix: the experience of IPO-PORTO

Date 28 September 2012
Event ESMO Congress 2012
Session Publication Only
Topics Gastrointestinal Cancers
Presenter Teresa Malheiro Sarmento
Authors T. Malheiro Sarmento1, M.H. Abreu1, R. Fragoso2, M. Machado3, N. Sousa4, C. Faustino4, P. Ferreira1
  • 1Medical Oncology, Portuguese Oncology Institute of Porto, 4200-072 - Porto/PT
  • 2Medical Oncology, Instituto Portugues de Oncologia Centro do Porto, 4200-072 - Porto/PT
  • 3Oncologia Ii, Portuguese Oncology Institute of Porto, 4200-072 - Porto/PT
  • 4Medical Oncology/ Hematology, Portuguese Oncology Institute of Porto, 4200-072 - Porto/PT



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.


The authors reviewed the primary appendix carcinomas treated in Instituto Português de Oncologia Francisco Gentil-Porto (IPOFG-Porto) between January 2000 and June 2011.


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.


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.


All authors have declared no conflicts of interest.