Chromogranin A Levels in Diagnosis, Treatment and Follow-Up of 42 Patients with Non-Functioning Pancreatic Endocrine Tumours

Background/Aims: Non-functioning pancreatic endocrine tumours (NFPET) constitutethe largest component (35-50%) of pancreatic endocrine tumours. They are characterized by the absence of symptoms of hormone hypersecretion and frequently have clinical manifestations similar to the more common exocrine pancreatic adenocarcinoma. The present study aims to evaluate the clinical features, diagnostic approach and, in particular, the significance of serum chromogranin A levels (CgA) in the management and outcome of 42 patients with NFPET (from a series of 121 patients with pancreatic endocrine tumours). Methods: Twenty-five males and 17 females were included, and the mean age at diagnosis was 52.3 years (range: 26-68 years). The diagnosis for each patient was established by histopathological examination and immunohistochemistry.Afterthehistopathological confirmation of diagnosis and during the follow-up period, patients were evaluated clinically and radiologically (including OctreoScan), whilst fasting gut hormones (including CgA) were also estimated. At diagnosis, all patients were checked for the presence of multiple endocrine neoplasia type I syndrome. The follow-up was complete and ranged from 12 to 86 months (mean: 49 months). Results: Dyspepsia (66.5%) and weight loss (47.6%) were the most common symptoms at diagnosis, while in 21.4% of patients tumour lesions were revealed incidentally. Plasma CgA levels were significantly or moderately elevated in all patients with liver metastases at diagnosis (64.3%). The levels also reflected tumour progression or response to treatment during the follow-up period. OctreoScan showed avid uptake in 77.8% of patients with hepatic metastases. Moreover, in 2 patients OctreoScan revealed unexpected metastatic mesenteric deposits, which had not been found by the other studies. However, it was negative in 6 patients with liver metastases, in whom tumours were proved to be poorly differentiated (high-grade). Conclusions: (1) NFPET may present with clinical manifestations similar to those of an exocrine pancreatic tumour; (2) plasma CgA levels reflect tumour load, and also seem to correlate with tumour progression or response to treatment; (3) surgery in patients with localized disease at presentation can be curative, while it can also reduce tumour burden in patients with metastases; (4) long-acting somatostatin analogues provide good quality of life and temporary disease stabilization in patients with low-grade tumours; (5) systemic chemotherapy or chemoembolization seem to be beneficial in high-grade and progressive tumours.