review of literature on clinical pancreatology - The Pancreapedia

achieved in selected cases by tissue sparing surgical techniques. Most patients unfortunatelypresent with a locally advanced or metastatic disease. For patients with an advancedfunctioning NET, control <strong>of</strong> the hormonal syndrome may also represent a surgical indication.Various cytoreductive techniques or, in highly selected cases, liver transplantation can beapplied. For locally advanced non-functioning tumours, there is an indication for surgery inlarge tumours which tend to create local complications because <strong>of</strong> bleeding or bowelobstruction. Especially in ileal NETs aggressive surgical therapy is recommended because <strong>of</strong>prevention <strong>of</strong> long-term complications, which may improve survival [681].Medical treatmentThe incidence <strong>of</strong> pancreatic endocrine tumor (PET) accounts for approximately 2 percent <strong>of</strong>total pancreatic tumors and for approximately 1.5 pecent <strong>of</strong> autopsy cases, reflecting therecently increasing trend. According to WHO criteria (2004), PET is classified by the type <strong>of</strong>hormone produced by the tumor and its biological behavior. Together with the classical clinicalimages and hormone markers, 11 C-5-HTP-Positron emission tomography, OctreoScan ( 111 In-DTPA0-octreotide)scintigram, SACI-test and IOUS are used for diagnosis. Surgery is thetreatment <strong>of</strong> choice, if supposed to be curative and tolerable. In case <strong>of</strong> a well-differentiatedendocrine tumor, with no indication <strong>of</strong> resection or IVR, somatostatin analog is another therapyshowing stable disease status for a long period. Systemic chemotherapy, including 5-FU+streptozotocin, and streptozotocin+doxorubicin, are used in cases <strong>of</strong> well -differentiatedendocrine carcinoma, and cisplatin+etoposide are applied for poorly-differentiated endocrinecarcinoma (or small cell carcinoma). Recent studies focus on molecular target therapyincluding small molecules and monoclonal antibody, such as tyrosine kinase inhibitor, anti-VEGF antibody and moor inhibitor [682].Cytotoxic treatmentGastroenteropancreatic neuroendocrine tumours (GEP NET) are heterogeneous and raremalignancies although their prevalence is increasing. Multiple therapeutic approaches areavailable to date for their management, including surgery, hormonal and immuneradionucleide therapies and chemotherapy. The purpose <strong>of</strong> one <strong>review</strong> was to collect,examine, and analyze data available regarding contemporary chemotherapeuticmanagement <strong>of</strong> GEP NET in order to determine whether or not chemotherapy still takesplace in the therapeutic arsenal <strong>of</strong> GEP NET. Anthracyclins, 5-fluorouracil (5-FU), DTIC andstreptozotocin are amongst the most commonly used chemotherapeutic agents, usuallyprescribed in combination. Their efficiency in reducing tumor burden is not always associatedwith better survival, perhaps due to severe toxicity. Chemotherapy in GEP NET is mainlydevoted to poorly differentiated tumours, but also in well differentiated carcinomas either noteligible or resistant to other therapies. Chemotherapy remains therefore useful in specificcases <strong>of</strong> GEP NET management. However, a new era <strong>of</strong> antitumoral agents, such astargeted therapies, could eventually replace these old recipes in the near future [683].Radiolabeled targeted therapiesGastro-entero-pancreatic neuroendocrine tumours (GEP NET) are a heterogeneous group <strong>of</strong>proliferative disorders whose management dramatically relies on tumour biology. For welldifferentiated,low-proliferative index tumours, locoregional treatment and targetedradioisotopic therapies <strong>of</strong>fer an attractive and seemingly efficient alternative to palliativesurgical resections. Lack <strong>of</strong> well-designed, prospective, randomized multicentric studieshinders a balanced evaluation <strong>of</strong> available locoregional treatment methods: embolization,chemo-embolization, radio-embolization. According to available datas, all techniques achievea 50-60 percent radiological response rate and almost 80 percent <strong>of</strong> symptomatic relieve forthe patients, while their impact on progression-free and overall survival remains not