Pancreatic endocrine neoplasms: a current update on genetics and imaging

Abstract

Pancreatic endocrine neoplasms are rare pancreatic tumours that may occur sporadically or as part of inherited syndromes such as multiple endocrine neoplasia-1 syndrome, von Recklinghausen disease, von Hippel-Lindau syndrome and tuberous sclerosis complex. Recent advances in the genetics and pathology of hereditary syndromes have provided valuable insights into the pathophysiology and biology of sporadic pancreatic endocrine neoplasms. Evolving molecular data on the biology of these neoplasms have the potential for diagnostic, therapeutic and prognostic use.

Aggressive Resection in Patients with Advanced Pancreatic Neuroendocrine Tumors: Case Report and Review of the Literature

“…PNETs are rare neoplasms accounting for less than 3% of all pancreatic neoplasms [1][2][3][4][5]. They may be categorised as functional or non functional tumors based on the characteristic clinical syndrome they cause owing to specific hormone synthesis and secretion.…”

“…Except for insulinoma; PNETs are malignant in more than 50-60% of cases. In various studies, 50 to 70% of patients with advanced disease have died within 5 years of tumor progression [1,3,[4][5][6].…”

“…To enhance clinical decision making utility, these systems have recently been rationalised by ENETs in the form of clinical guidelines for diagnosis and management [7]. It is obvious that PNETs were more favourable than exocrine cancer in terms of outcomes and should therefore be addressed differently [1,5,8,9]. Surgery is the only potentially curative treatment for these tumors because chemotherapy and radiation therapy have not demonstrated any significant antitumor effects [1,5,9].…”

“…It is obvious that PNETs were more favourable than exocrine cancer in terms of outcomes and should therefore be addressed differently [1,5,8,9]. Surgery is the only potentially curative treatment for these tumors because chemotherapy and radiation therapy have not demonstrated any significant antitumor effects [1,5,9]. Furthermore, if all of the tumor can be removed with extensive surgery; the duration and quality of life may be greatly enhanced [9].…”

“…With regard to liver metastases, a number of studies have shown that combined or staged resection and small volume metastatic liver disease improves survival outcomes [5,11,[15][16][17][18][19]. ENETS guidelines [7] suggest that resection should only be reserved if at least 90% of the volume's tumor can be removed.…”