Elsevier

Pancreatology

Volume 4, Issue 5, 2004, Pages 417-435
Pancreatology

Hereditary pancreatic endocrine tumours

https://doi.org/10.1159/000079616Get rights and content

Abstract

The two main types of hereditary pancreatic neuroendocrine tumours are found in multiple endocrine neoplasia type 1 (MEN-1) and von Hippel-Lindau disease (VHL), but also in the rarer disorders of neurofibromatosis type 1 and tuberous sclerosis. This review considers the major advances that have been made in genetic diagnosis, tumour localization, medical and surgical treatment and palliation with systemic chemotherapy and radionuclides. With the exception of the insulinoma syndrome, all of the various hormone excess syndromes of MEN-1 can be treated medically. The role of surgery however remains controversial ranging from no intervention (except enucleation for insulinoma), intervening for tumours diagnosed only by biochemical criteria, intervening in those tumours only detected radiologically (1–2 cm in diameter) or intervening only if the tumour diameter is > 3 cm in diameter. The extent of surgery is also controversial, although radical lymphadenectomy is generally recommended. Pancreatic tumours associated with VHL are usually non-functioning and tumours of at least 2 cm in diameter should be resected. Practice guidelines recommend that screening in patients with MEN-1 should commence at the age of 5 years for insulinoma and at the age of 20 years for other pancreatic neuroendocrine tumours and variously at 10–20 years of age for pancreatic tumours in patients with VHL. The evidence is increasing that the life span of patients may be significantly improved with surgical intervention, mandating the widespread use of tumour surveillance and multidisciplinary team management.

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      Citation Excerpt :

      PNTs can be associated to inherited disorders as multiple endocrine neoplasia (MEN-1), von Hippel Lindau syndrome, neurofibromatosis and tuberous sclerosis. About 80–100% of MEN-1 patients develop PNTs; of which 50–60% are gastrinomas, 20% are insulinomas and 3–5% are vasoactive intestinal peptide (VIP)omas or glucagonomas [1,33,37,38]. PNTs exhibit variable imaging characteristics that are not exclusive to a specific category of lesion.

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    J.P. Neoptolemos, MB, BChir, MD, FRCS, Professor of Surgery, Head, Department of Surgery, University of Liverpool, UCD Building 5th floor, Royal Liverpool University Hospital, Daulby Street, Liverpool L69 3GA (UK), Fax +44 151 7065826

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