Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors

Abstract

Pancreatic endocrine tumors (PETs) are uncommon tumors with an annual incidence !1 per 100 000 person-years in the general population. The PETs that produce hormones resulting in symptoms are designated as functional. The majority of PETs are non-functional. Of the functional tumors, insulinomas are the most common, followed by gastrinomas. The clinical course of patients with PETs is variable and depends on the extent of the disease and the treatment rendered. Patients with completely resected tumors generally have a good prognosis, and aggressive surgical therapy in patients with advanced disease may also prolong survival. The epidemiology, prognosis, and established and novel prognostic markers of PETs are reviewed.

Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters

“…The annual incidence of PNETs was reviewed recently and was described as !0.4 cases per 100 000 (Halfdanarson et al 2008), accounting for only 1-3% of all pancreatic neoplasms. population, 14 (70%) of 20 colonic NETs were classified as neuroendocrine carcinoma (well differentiated 10, 71%; poorly differentiated 4, 29%; 0.13 per 100 000).…”

The genetic classification of pancreatic neoplasia

“…Compared with PDA, however, the prognosis is usually much better, with approximately 40 % of affected patients surviving 10 years after diagnosis [126][127][128]. PanNETs are heterogeneous and clinically can be divided into functioning (syndromic) or nonfunctioning (nonsyndromic) tumors.…”

“…PanNETs are heterogeneous and clinically can be divided into functioning (syndromic) or nonfunctioning (nonsyndromic) tumors. Among all functioning PanNETs, insulinomas are the commonest subgroup (30-45 %) [127], followed by gastrinomas (16-30 %), VIPomas, glucagonomas (less than 10 % each), and somatostatinomas (less than 5 %) [129]. Most PanNETs occur sporadically, although up to 10 % arise in individuals with a cancerpredisposition syndrome, such as von Hippel-Lindau syndrome (caused by inherited mutations in VHL), tuberous sclerosis (caused by inherited mutations in TSC1 or TSC2), or multiple endocrine neoplasia 1 (caused by inherited mutations in MEN1) [130].…”

Pancreatic Neuroendocrine Tumors: an Update

“…Glucagonomas (less than 10 %) often present with a typical dermatitis known as migratory necrolytic erythema characterized by necrotic erythematous lesions that eventually resolve in pigmented scaring [8,9]. In addition, glucose intolerance, weight loss, diarrhea, and deep vein thrombosis can be observed.…”

“…Typical symptoms of non-functional pNETs include abdominal pain, back pain, weight loss, jaundice, possibly pancreatitis, and are likely due to the mass effect exerted by the pNETs lesions on the surrounding structures [11]. At the time of diagnosis, 20 % of patients present with locally advanced disease, and approximately 60 % of patients present with metastatic disease [9].…”