PANCREAS ALERTS
N Engl J Med 2000; 343:551-4
Department of Internal Medicine and Gastroenterology, University of Bologna. Bologna, Italy.
The authors report three cases of gastric carcinoid tumors that regressed in patients who had Zollinger-Ellison syndrome and a family history of multiple endocrine neoplasia type 1 after long-term treatment with somatostatin analogues. One of these patients had more than 30 tumors spread throughout the corpus and the fundus of the stomach, all of which disappeared after one year of treatment. Before treatment, serum gastrin levels were elevated in all of the patients, and the patient with diffuse gastric carcinoid tumors had the highest levels. During treatment, serum gastrin levels decreased markedly. Since hypergastrinemia is an important factor in the pathogenesis of gastric carcinoid tumors in patients with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome, it is likely that the disappearance of the tumors is related to the decrease in serum gastrin levels. In patients with type I gastric carcinoid tumors, the tumors regress rapidly after antrectomy and subsequent normalization of serum gastrin levels.
These findings do not exclude the possibility that somatostatin analogues have a direct effect on the proliferation of enterochromaffin-like cells. Such an effect is also suggested by the finding that a type III gastric carcinoid tumor regressed after long-term administration of octreotide in a patient with normal gastrin levels. Enterochromaffin-like cells have a specific somatostatin receptor that belongs to subtype 2.
Gastroenterology 2000; 119:1087-95
Federation Medico-Chirurgicale d'Hepato-Gastroenterologie, France.
Pancreatic involvement in von Hippel-Lindau (VHL) disease, a genetic disorder with a dominant mode of inheritance affecting various organs, has rarely been studied. In this multicenter prospective study, the authors assessed the prevalence, type of lesions, natural history, and impact of pancreatic involvement in patients with VHL.
A total of 158 consecutive patients with VHL disease from 94 families were studied. All patients underwent systematic screening for VHL lesions, including computerized tomography (CT) scanning of the pancreas reviewed by an experienced radiologist. Clinical data, investigations and all treatments undergone were also reviewed. Pancreatic involvement was observed in 122 patients (77.2%) and included true cysts (91.1%), serous cystadenomas (12.3%), neuroendocrine tumors (12.3%), or combined lesions (11.5%). The pancreas was the only organ affected in 7.6% of patients. Patients with pancreatic lesions had significantly fewer pheochromocytomas than those without (P<0.0001) and patients with neuroendocrine pancreatic tumors had less frequent renal involvement than those without (P=0.013). None of the patients with neuroendocrine tumors had symptoms of hormonal hypersecretion. Pancreatic lesions evolved in half of patients but required specific treatment in only 8.2% when they were symptomatic or when resection of large neuroendocrine tumors was necessary. >
In conclusion, pancreatic involvement is seen in most patients with VHL disease and, although the symptoms are rare, specific treatment of pancreatic lesions is required in selected patients, mainly those with neuroendocrine tumors.