Background - The development of endocrine tumours of the duodenopancre
atic area (ETDP) is thought to be slow, but their natural history is n
ot well known. The aim of this study was to determine the factors that
influence survival of patients with ETDP. Patients/Methods - Eighty t
wo patients with ETDP (44 non-functioning tumours, 23 gastrinomas, sev
en calcitonin-secreting tumours, four glucagonomas, three insulinomas,
one somatostatinoma) followed from October 1991 to June 1997 were inc
luded in the study. The following factors were investigated: primary t
umour size, hormonal clinical syndrome, liver metastases, lymph node m
etastases, 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. Res
ults - Twenty eight patients (34%) died within a median of 17 months (
range 1-110) from diagnosis. Liver metastases (p = 0.001), lymph node
metastases (p = 0.001), progression of liver metastases (p < 0.00001),
lack of complete resection of the primary tumour (p = 0.001), extrano
dular/extrahepatic metastases (p = 0.001), local invasion (p = 0.001),
primary tumour size greater than or equal to 3 cm (p = 0.001), non-fu
nctioning tumours (p = 0.02), and poor tumoral differentiation (p = 0.
006) were associated with an unfavourable outcome by univariate analys
is. Multivariate analysis identified only liver metastases (risk ratio
(RR) = 8.3; p < 0.0001), poor tumoral cell differentiation (RR = 8.1;
p = 0.0001), and lack of complete resection of the primary tumour (RR
= 4.8; p = 0.0007) as independent risk factors. Five year survival ra
tes were 40 and 100% in patients with and without liver metastases, 85
and 42% in patients with and without complete resection of primary tu
mour, and 17 and 71% in patients with poor and good tumour cell differ
entiation respectively. Conclusion - Liver metastases are a major prog
nostic factor in patients with ETDP. Progression of liver metastases i
s also an important factor which must be taken into account when decid
ing on the therapeutic approach. The only other independent prognostic
factors are tumoral cell differentiation and complete resection of th
e primary tumour.