Pancreatic endocrine tumour: a 22-year clinico-pathological experience with morphological, immunohistochemical observation and a review of the literature

Abstract

The clinico-pathological features of 53 Chinese patients (27 males; 26 females) with pancreatic endocrine tumours were studied. The age range was from 14 to 78 years old (mean: 48 years) with the modal peak in the sixth decade for both sexes. Pancreatic endocrine tumours accounted for 14% of the primary pancreatic tumours operated on in Queen Mary Hospital. The autopsy incidence was 0.11%. Seventy-two per cent (38 cases) of the tumours were clinically functioning, comprising 33 insulinomas, three gastrinomas and two glucagonomas. A rare case of malignant gastrinoma associated with Cushing's syndrome was also documented. The functional tumours were seen in the younger patients. The calculated annual incidence of clinically significant tumours was approximately 0.2 per 100,000 population. There was no correlation between the site, functional status and histological patterns of the tumours. Seventy-two per cent of the tumours showed a trabecular pattern. Calcification was present in 5.7% (three cases); two such cases being gastrinomas. Amyloid was found in 25% of tumours, chiefly (92%) in the insulinomas. The main difficulty encountered in diagnosis was distinguishing between solid and cystic tumours of the pancreas. The incidence of malignancy was 15% and the histological features were poor predicative indicators of malignant potential. The metastatic pancreatic endocrine tumours were often detected in the liver and lymph nodes. Immunohistochemical stains showed evidence of multi-hormone production in 18% of cases and all tumours showed a positive reaction to at least one of the six markers, namely, neuron-specific enolase (NSE), chromogrannin (CG), synaptophysin (SYN), insulin (INS), glucagon (GLU) or somatostatin (SOM). The three panendocrine markers (NSE, SYN, CG) were satisfactory for initial screening of the endocrine nature of the tumours if used in combination, as 92% of tumours were positive for at least one of these three markers.