Abstract: | Background—Thedevelopment of endocrine tumours of the duodenopancreatic area (ETDP)is thought to be slow, but their natural history is not well known. Theaim of this study was to determine the factors that influence survivalof patients with ETDP. Patients/Methods—Eightytwo patients with ETDP (44 non-functioning tumours, 23 gastrinomas,seven calcitonin-secreting tumours, four glucagonomas, threeinsulinomas, one somatostatinoma) followed from October 1991 to June1997 were included in the study. The following factors wereinvestigated: primary tumour size, hormonal clinical syndrome, livermetastases, lymph node metastases, extranodular/extrahepatic metastases, progression of liver metastases, local invasion, complete resection of the primary tumour, and degree of tumoral differentiation. The prognostic significance of these factors was investigated by uni-and multi-variate analysis. Results—Twenty eightpatients (34%) died within a median of 17 months (range 1-110) fromdiagnosis. Liver metastases (p =0.001), lymph node metastases (p = 0.001), progression of liver metastases (p<0.00001), lack of completeresection of the primary tumour (p = 0.001), extranodular/extrahepaticmetastases (p =0.001), local invasion (p = 0.001), primary tumour size3 cm (p = 0.001), non-functioning tumours (p = 0.02), and poortumoral differentiation (p = 0.006) were associated with anunfavourable outcome by univariate analysis. Multivariate analysisidentified only liver metastases (risk ratio (RR) = 8.3; p<0.0001),poor tumoral cell differentiation (RR = 8.1; p = 0.0001), and lack ofcomplete resection of the primary tumour (RR = 4.8; p = 0.0007) asindependent risk factors. Five year survival rates were 40and 100% inpatients with and without liver metastases, 85 and 42% in patientswith and without complete resection of primary tumour, and 17 and 71%in patients with poor and good tumour cell differentiation respectively. Conclusion—Livermetastases are a major prognostic factor in patients with ETDP.Progression of liver metastases is also an important factor which mustbe taken into account when deciding on the therapeutic approach. Theonly other independent prognostic factors are tumoral celldifferentiation and complete resection of the primary tumour.
Keywords:prognostic factors; survival; endocrine tumours; gastrinoma; cell differentiation; liver metastases |