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) constitute the 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 studyaims toevaluate 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. After the histopathological 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) surgeryin 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.

Prophylactic and therapeutic use of recombinant activated factor VII in patients with cirrhosis and coagulation impairment.

Patients with cirrhosis and impaired coagulation often pose major therapeutic problems during bleeding episodes or invasive procedures. Recombinant activated factor VII (rFVIIa), which has been licensed for the treatment of haemophilia patients with factor VIII or IX inhibitors, has been occasionally used in cirrhotic patients. We present five patients with cirrhosis and coagulopathy who received 1-4 recombinant activated factor VII infusions either prophylactically in order to safely undergo an invasive procedure or therapeutically in order to control a severe bleeding episode which did not respond to standard supportive care. In particular, recombinant activated factor VII infusions were given in two patients before a percutaneous liver biopsy, in one patient before teeth extraction and in two patients with haemoperitoneum after an invasive procedure. Infusions of recombinant activated factor VII achieved rapid correction of prothrombin time in all cases allowing the safe performance of invasive procedures or resulting in efficient control of the bleeding episode. In conclusion, recombinant activated factor VII seems to be a rather promising agent for the prevention or treatment of complications of haemostasis impairment in cirrhotic patients. However, its exact role in this setting needs to be evaluated within well-designed, controlled clinical trials.

Autoimmune pancreatitis.

Autoimmune pancreatitis is a type of idiopathic pancreatitis. It is also referred to as sclerosing pancreatitis, lymphoplasmatocytic sclerosing pancreatitis, chronic pancreatitis with irregular stenosis of the main pancreatic duct and as sclerosing pancreatocholangitis. Clinical characteristics of autoimmune pancreatitis are jaundice, abdominal pain, weight loss and diabetes mellitus. Radiologically, there is diffuse enlargement of pancreas with stenosis of pancreatic duct without calcifications in the pancreatic parenchyma. In autoimmune pancreatitis, antibodies against lactoferrin and carbonic anhydrase have been detected, but they are not specific because they are present in some other autoimmune diseases too. Also in autoimmune pancreatitis, there are increased levels of gammaglobulins and characteristically high titres of IgG4, which are a subtype of IgG. Autoimmune pancreatitis is usually treated successfully by prednisolone.