Management of Well-differentiated Gastroenteropancreatic Neuroendocrine Tumors (GEPNETs): A Review

Pasricha, Gurleen; Padhi, Parikshit; Daboul, Nour; Monga, Dulabh

doi:10.1016/j.clinthera.2017.10.010

Cited by 19 publications

(18 citation statements)

References 71 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In addition, we noted that PNENs, irrespective of disease extent, exhibited elevated scores. We interpret this to reflect the previously well-described more malignant nature of pancreatic NEN disease [56]. The elevated scores may therefore be consistent with disease progression but also the fact that molecular measurements of the various omic clusters (that constitute the hallmarks of neoplasia) identify the mechanistic oncogenic drivers that provide the basis for the increased intrinsic malignancy of pancreatic NEN disease.…”

Section: Discussionsupporting

confidence: 74%

Prospective Evaluation of the NETest as a Liquid Biopsy for Gastroenteropancreatic and Bronchopulmonary Neuroendocrine Tumors: An ENETS Center of Excellence Experience

et al. 2020

View full text Add to dashboard Cite

Background: There is a substantial unmet clinical need for an accurate and effective blood biomarker for neuroendocrine neoplasms (NEN). We therefore evaluated, under real-world conditions in an ENETS Center of Excellence (CoE), the clinical utility of the NETest as a liquid biopsy and compared its utility with chromogranin A (CgA) measurement. Methods: The cohorts were: gastroenteropancreatic NEN (GEPNEN; n = 253), bronchopulmonary NEN (BPNEN; n = 64), thymic NEN (n = 1), colon cancer (n = 37), non-small-cell lung cancer (NSCLC; n = 63), benign lung disease (n = 59), and controls (n = 86). In the GEPNEN group, 164 (65%) had image-positive disease (IPD, n = 135) or were image-negative but resection-margin/biopsy-positive (n = 29), and were graded as G1 (n = 106), G2 (n = 49), G3 (n = 7), or no data (n = 2). The remainder (n = 71) had no evidence of disease (NED). In the BPNEN group, 43/64 (67%) had IPD. Histology revealed typical carcinoids (TC, n = 14), atypical carcinoids (AC, n = 14), small-cell lung cancer (SCLC, n = 11), and large-cell neuroendocrine carcinoma (LCNEC, n = 4). Disease status (stable or progressive) was evaluated according to RECIST v1.1. Blood sampling involved NETest (n = 563) and NETest/CgA analysis matched samples (n = 178). NETest was performed by PCR (on a scale of 0–100), with a score ≥20 reflecting a disease-positive status and >40 reflecting progressive disease. CgA positivity was determined by ELISA. Samples were deidentified and measurements blinded. The Kruskal-Wallis, Mann-Whitney U, and McNemar tests, and the area under the curve (AUC) of the receiver-operating characteristics (ROC) were used in the statistical analysis. Results: In the GEPNEN group, NETest was significantly higher (34.4 ± 1.8, p < 0.0001) in disease-positive patients than in patients with NED (10.5 ± 1, p < 0.0001), colon cancer patients (18 ± 4, p < 0.0004), and controls (7 ± 0.5, p < 0.0001). Sensitivity for detecting disease compared to controls was 89% and specificity was 94%. NETest levels were increased in G2 vs. G1 (39 ± 3 vs. 32 ± 2, p = 0.02) and correlated with stage (localized: 26 ± 2 vs. regional/distant: 40 ± 3, p = 0.0002) and progression (55 ± 5 vs. 34 ± 2 in stable disease, p = 0.0005). In the BPNEN group, diagnostic sensitivity was 100% and levels were significantly higher in patients with bronchopulmonary carcinoids (BPC; 30 ± 1.3) who had IPD than in controls (7 ± 0.5, p < 0.0001), patients with NED (24.1 ± 1.3, p < 0.005), and NSCLC patients (17 ± 3, p = 0.0001). NETest levels were higher in patients with poorly differentiated BPNEN (LCNEC + SCLC; 59 ± 7) than in those with BPC (30 ± 1.3, p = 0.0005) or progressive disease (57.8 ± 7), compared to those with stable disease (29.4 ± 1, p < 0.0001). The AUC for differentiating disease from controls was 0.87 in the GEPNEN group and 0.99 in BPC patients (p < 0.0001). Matched CgA analysis was performed in 178 patients. In the GEPNEN group (n = 135), NETest was significantly more accurate for detecting disease (99%) than CgA positivity (53%; McNemar...

show abstract

Section: Discussionsupporting

confidence: 74%

Prospective Evaluation of the NETest as a Liquid Biopsy for Gastroenteropancreatic and Bronchopulmonary Neuroendocrine Tumors: An ENETS Center of Excellence Experience

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Nonsurgical treatment options mainly include somatostatin analogs (SSAs), multikinase inhibitors, targeted therapies, chemotherapy and radiolabeled SSAs. 22 SSAs, including octreotide long-acting repeatable, lanreotide, and pasireotide, are treatments for NETs at the earliest stage, and as antisecretory and antiproliferative agents, they are mainly used to alleviate the symptoms of carcinoid syndrome and to control tumor growth in symptomatic and asymptomatic, unresectable, high-tumor burden patients with well-differentiated NETs. In addition, there is a consensus that SSAs should be used regardless of somatostatin receptor imaging results in patients with carcinoid syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…In patients with inoperable NETs, the goal is to control endocrine‐related symptoms and tumor growth and prolong survival. Nonsurgical treatment options mainly include somatostatin analogs (SSAs), multikinase inhibitors, targeted therapies, chemotherapy and radiolabeled SSAs . SSAs, including octreotide long‐acting repeatable, lanreotide, and pasireotide, are treatments for NETs at the earliest stage, and as antisecretory and antiproliferative agents, they are mainly used to alleviate the symptoms of carcinoid syndrome and to control tumor growth in symptomatic and asymptomatic, unresectable, high‐tumor burden patients with well‐differentiated NETs.…”

Section: Discussionmentioning

confidence: 99%

Clinical analysis of 547 patients with neuroendocrine tumors in a Chinese population: A single‐center study

Liu

Zhai

et al. 2019

Cancer Medicine

View full text Add to dashboard Cite

Background Neuroendocrine tumors (NETs) are rare, which has resulted in a lack of published data on their epidemiology and clinical features. We therefore aimed to investigate the epidemiology, clinical features, treatments, and prognosis of patients with NETs. Methods The clinicopathologic characteristics of 547 patients who were pathologically diagnosed with NETs were retrospectively analyzed, including age, sex, primary and metastatic sites, symptoms, pathology, treatment, and prognosis. Results The 547 patients had a wide age range (9‐87 years), with a male to female ratio of 1:1.1. The primary tumor sites included 413 in the digestive system, 74 in the lung, 15 in the mediastinum, 8 in unknown sites, and 37 in other sites. Of the 413 patients with digestive system NETs, the pancreas, rectum, and stomach were the most common primary sites. Blood metastases were found in 84 patients at initial diagnosis, and the liver, bone, and lung were the most frequent sites of metastasis. Lymph node metastases were found in 82 patients at initial diagnosis. Surgery and chemotherapy were the most widely applied treatments. Statistical analysis showed that age <50 years, female sex, lower‐grade tumor, no distant metastasis, intestinal NET and surgery indicated a favorable prognosis. Conclusions A difference between China and other countries is that small intestinal NETs are quite common in other countries but are rare in China. In China, the most common primary sites are the pancreas, rectum, and stomach. Furthermore, no unified treatments exist, though prognoses could be improved by using methods such as surgery, targeted therapies, and somatostatin analogs. Clinical Trial Registration This study was not a clinical trial.

show abstract

“…There is a significant advantage of imaging studies for the establishment of diagnosis (25,26). CT is essential in determining the size, the location of the tumour origin, the involvement of nearby structures, vessels, lymph nodes and the presence of calcification (6).…”

Section: Diagnosismentioning

confidence: 99%

VIPoma: Mechanisms, clinical presentation, diagnosis and treatment (Review)

Abdullayeva

2019

Wrld Acd Sci

View full text Add to dashboard Cite

Vasoactive intestinal peptide (VIP) secreting tumour (VIPoma) is a rare neuroendocrine tumour that most often originates from pancreatic islet cells and affects one in ten million individuals per year. In adults, it develops most commonly in the fortieth year of life with a sparse female predominance. Excessive VIP secretion induces refractory watery diarrhoea, hypokalemia and achlorhydria. Other symptoms include hyperglycemia (20-50%), hypercalcaemia (25-50%), hypochlorhydria (20-50%) and flushing (15-30%). VIP plasma levels are increased in almost all patients with VIPoma, and, together with profusing secretory diarrhoea, it is sufficient to establish the diagnosis. Moreover, the majority of VIPomas are malignant or have already metastasised at the time of diagnosis. The treatment of the neoplasm comprises medical management and surgery. While surgery remains the gold standard of treatment, peptide receptor radionuclide therapy represents one of the most effective and well-tolerated treatment options. The average survival rate of patients with VIPoma is 96 months. The objective of this review was to summarise all features of pancreatic VIPoma, as well as present novel treatment approaches for this rare neoplasm.

show abstract

Management of Well-differentiated Gastroenteropancreatic Neuroendocrine Tumors (GEPNETs): A Review

Cited by 19 publications

References 71 publications

Prospective Evaluation of the NETest as a Liquid Biopsy for Gastroenteropancreatic and Bronchopulmonary Neuroendocrine Tumors: An ENETS Center of Excellence Experience

Prospective Evaluation of the NETest as a Liquid Biopsy for Gastroenteropancreatic and Bronchopulmonary Neuroendocrine Tumors: An ENETS Center of Excellence Experience

Clinical analysis of 547 patients with neuroendocrine tumors in a Chinese population: A single‐center study

VIPoma: Mechanisms, clinical presentation, diagnosis and treatment (Review)

Contact Info

Product

Resources

About