Treatment of metastatic pancreatic neuroendocrine tumors: relevance of ENETS 2016 guidelines

Abstract

The choice of first-line treatment for metastatic pancreatic neuroendocrine tumors (mP-NET) is mainly based on prognostic factors. ENETS-2016 guidelines stratified treatment according to 3 groups: Group 1, patients in whom all lesions could be removed; Group 2, patients with Ki67 <10%, low tumor burden, no symptoms and stable disease, for whom a watch-and-wait strategy or somatostatin analogs are proposed; Group 3, symptomatic patients or with Ki67 >10% or significant tumor burden or progressive disease, for whom a systemic chemotherapy is proposed. This retrospective study aimed to determine patient distribution, characteristics and outcome among these 3 groups. Patients with mP-NET diagnosis from 2004 to 2016 were categorized into the three groups. Prognosis was calculated using the Kaplan-Meier method. All treatments were recorded, and consistency with ENETS guidelines was explored. 104 patients were analyzed: 64% synchronous mP-NET, 80% grade 2 tumors and median overall survival (OS) of 104 (95% CI: 65-143) months. There were 15 patients in ENETS Group 1, 16 in Group 2 and 73 in Group 3. Median OS was not reached in Groups 1 and 2 and was 64 months (35-93) in Group 3. High liver tumor volume, high-grade tumor and progressive disease were associated with worse OS in multivariate analysis. The first-line treatment was in accordance with guidelines in 82%. 77% percent of deceased patients received less than 4 lines of treatment. Most patients are in Group 3 and do not receive all available treatments. Thus, trials are warranted to improve first-line chemotherapy. Alternative treatments may be considered for less aggressive disease.

Characterization, Prognosis, and Treatment of Patients With Metastatic Lung Carcinoid Tumors

“…Regarding prognostic factors, similar to that found for digestive NETs, poor ECOG PS, higher tumor grade (atypical subtype according to the WHO classification), high tumor burden (high CgA levels which is reported to be correlated with tumor burden), and absence of uptake on SRS were significantly associated with poor survival. 29,31,32 WHO classification is currently based on primary tumor analysis and its relevance at the metastatic stage has yet to be established; herein, however, we found that WHO classification remained a strong prognostic factor at the metastatic stage. In contrast with the digestive WHO classification, Ki67 is not required in the lung WHO classification.…”

“…3 Regarding the modality of tumor spread, both herein and in the LUNA study, the main metastatic site was the liver, as has been reported for digestive NETs; however, the frequency of bone and lung sites was much higher in MLC patients. 5,28 Uptake on FDG-PET was more frequent herein than in digestive NETs, 3,29 yet bias cannot be excluded because only half the patients underwent this examination which may be prescribed for more aggressive disease (this was not reported in the LUNA study 5 ).…”

Pancreatic serous cystoadenoma (CSA) showing increased tracer uptake at 68-GaDOTA-peptide Positron Emission Tomography (68Ga-DOTA-peptide PET-CT): a case report

“…2). A diagnosis of pancreatic neuroendocrine tumour was established, with consequently indication to surgery [6]. A distal splenopancreatectomy and cholecystectomy with laparoscopic approach was performed: intraoperatively, we confirmed the presence of a large solid mass, hyper-vascularized, strictly adherent to mesocolon.…”

Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment

“…Regimens included dacarbazine, streptozotocin, oxaliplatin, cisplatin–etoposide, and everolimus. Radiofrequency ablation and chemoembolization have also been used in pediatric pNETs . Furthermore, neuroendocrine tumors have been found to respond to octreotide and peptide receptor radionuclide therapy due to their high expression of somatostatin receptors .…”