Pancreatic and peripancreatic somatostatinomas
| Autor: | C C Thorn, Duncan Spalding, J M L Williamson, Robin C. N. Williamson |
|---|---|
| Rok vydání: | 2011 |
| Předmět: |
Adult
Male medicine.medical_specialty Abdominal pain Ampulla of Vater Neurofibromatosis 1 medicine.medical_treatment Common Bile Duct Neoplasms Gastroenterology Pancreaticoduodenectomy Diagnosis Differential Internal medicine Somatostatinoma medicine Humans Aged Cholangiopancreatography Endoscopic Retrograde Incidental Findings Cholestasis business.industry Bile duct General Medicine Jaundice Middle Aged medicine.disease Abdominal Pain Pancreatic Neoplasms Jaundice Obstructive medicine.anatomical_structure General Surgery Surgery Female medicine.symptom Differential diagnosis Pancreas business Tomography X-Ray Computed |
| Zdroj: | Annals of the Royal College of Surgeons of England. 93(5) |
| ISSN: | 1478-7083 |
| Popis: | BACKGROUND Somatostatinomas are rare neuroendocrine tumours with an annual incidence of 1 in 40 million. They arise in the pancreas or periampullary duodenum. Most are clinically non-secretory and do not cause the somatostatinoma syndrome. Many are metastatic at presentation and their management is typically multimodal. CASE HISTORIES Four cases of somatostatinoma are described. Two patients with periampullary disease presented with biliary obstruction, one with frank jaundice and one with incidental bile duct obstruction on investigation of hepatitis B. Each patient had type 1 neurofibromatosis and resection of the somatostatinoma by means of a pylorus-preserving proximal pancreaticoduodenectomy has resulted in long-term survival. Another two patients with metastatic pancreatic somatostatinomas presented with abdominal pain. Contrasting management illustrates current treatment strategies that are dependent in part on the distribution of the disease. DISCUSSION The pathophysiology, presentation, clinical associations and role of diagnostic imaging are discussed for periampullary and pancreatic neuroendocrine tumours. Operative treatment has an important role in both the curative and palliative settings in conjunction with appropriate medical treatments and these are described. Management options depend on the extent of the disease and the cases are used to illustrate the rationale of such strategies. |
| Databáze: | OpenAIRE |
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