Prognosis and predictive factors

Aggressive endocrine tumours include gastrin cell, somatostatin cell, and EC-cell tumours that invade beyond the sub-mucosa or show lymph node or distant (liver) metastases. Aggressive tumours have been reported to be 10% of all gastrin cell duodenal-upper jejunal tumours {233}, 58% of sporadic ZES cases {429} and 45% of ZES-MEN-1 cases {429}. In the case of somatostatin cell tumours, about two-thirds were aggressive in one study {381}.

Gastrin cell tumours associated with an overt ZES are prognostically less favourable than their nonfunctioning counterparts, having a higher incidence of metastases (3 of 14 cases as against 0 of 28), and being deeply infiltrative (7 of 14 as against 3 of 19) {1780}. These findings suggest a different natural history of gastrin cell tumours in the two conditions. Nonfunctioning tumours represent a generally benign condition, while ZES tumours have a low-grade malignancy, especially when arising in sites where gastrin cells are not normally present, such as in the jejunum or pancreas {233}. Metastases in regional lymph nodes have been reported in 4 of 8 cases of duodenal gastrinomas with ZES-MEN-1 syndrome {1521}, in 2 of 3

Fig. 4.14 Somatostatin cell tumour exhibiting characteristic tubuloglandular pattern and a psammo-ma body.

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