Treatment of the pancreatic disease of multiple endocrine neoplasia type 1 (MEN 1).
This review comprises an analysis of the treatment alternatives for pancreatic tumours of the MEN 1 syndrome presently in use at the Surgical Endocrine Unit of Sahlgrenska Hospital, Göteborg, Sweden. Patients with pancreatic tumours localized by imaging studies are offered surgical excision. Otherwise the therapy is individualized. Some patients with Zollinger-Ellison syndromes are managed with medical antiulcer treatment, others are treated with total gastrectomy. MEN 1 patients with other syndromes, e.g. insulinomas, glucagonomas and VIPomas, are offered surgery with the aim of tumour excision. Even noncurative excision might benefit the management of these cases and possibly prolong life. Asymptomatic patients with raised hormonal tumour markers in whom imaging studies have failed to reveal any tumours, are continuously followed without treatment. The malignant behaviour of the pancreatic tumours varies between different MEN 1 families. MEN 1 patients are considered for total pancreatectomy if they have multiple malignant pancreatic tumours and if the pancreatic disease has caused high morbidity and mortality in the family.
Tisell
L E
LE
Department of Surgery, University of Göteborg, Sweden.
Ahlman
H
H
D007516:Adenoma, Islet Cell / Q000235:genetics / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000628:therapy*