7 Pancreatic Neuroendocrine Tumors

Suggested citation:  Endocrine Society. Endocrine Facts and Figures: Cancers and Neoplasias. First Edition. 2017

Neuroendocrine tumors that secrete pancreatic hormones (pNETs), also known as islet cell tumors, are a subgroup of neuroendocrine tumors (NETs) that can arise within the pancreas. These pNETs can be functioning NETs (e.g. insuliomas, gastrinomas, glucagonomas, VIPomas, somatostatinomas) or non-functioning NETs (e.g. PPoma). The primary location of insulomas and glucagonomas is the pancreas, but gastriomas (pancreas, duodenum), VIPoma (pancreas, neural, periganglionic, adrenal), somatostastinoma (pancreas, duodenal/jejuna), GRFoma (pancreas, lung, jejunal, adrenal, foregut, retroperitoneal) are also or primarily found extra-pancreatically.309


In the general population, the incidence of pNETs is <1 per 100,000, but their prevalence is higher in autopsy samples, which suggests many cases may be subclinical (Table 7.1).310 Between 10 and 30% of pNETs are functioning, and of these, insulinomas and gastrinomas are the most common sub-types (Table 7.1). Overall gastrointestinal NETs are the second most prevalent malignancy in the GI tract, only surpassed by colon cancer.

In early studies, non-functioning pNETs were thought to present late in the disease course, often when liver metastases is already detectable in 60-85% of cases; however, more recent reports implicate an earlier occurrence of pNETs, during the asymptomatic/preclinical stage, and at a higher frequency than previously reported (Table 7.1).309

Table 7.1. Prevalence and incidence of pancreatic neuroendocrine tumor (pNET) subtypes and syndromes, worldwide.
Pancreatic neuroendocrine tumor subtypes and syndromes (pNETs) Data source Population Hormone Functioning syndrome Incidence

(per 100,000 per year)

Prevalence of all pNETs


Prevalence of functioning

pNETs (%)

pNETS SEER database US


N/A N/A 0.43


N/G N/A McKenna and Edil. 2014311
Literature review Worldwide,

Pancreatic tumors

N/A N/A ng 1–4% N/G McKenna and Edil. 2014311; D’Haese et al. 2014312; Milan and Yeo. 2012313
Literature review Worldwide, Neuroendocrine tumors N/A N/A N/G 7% N/G McKenna and Edil. 2014311
Literature review Worldwide, autopsy studies N/A N/A N/G 0.8–10% N/G Ito et al. 2012309 McKenna and Edil. 2014311; Berge et al. 1976314
Non-functioning pNET
Literature review Worldwide None Pancreatic polypeptideomas (PPomas): non-functioning-pNET 1-3 N/G N/G Ito et al. 2012309
Functioning pNETs
Gastrinoma Literature review Worldwide gastrin Zollinger-Ellison syndrome (ZES) 0.5-1.5 N/G 16–30% McKenna and Edil. 2014311; Ito et al. 2012309
Insulinoma Literature review Worldwide Insulin Insulinoma 1-3 N/G 35–40% Ito et al. 2013309; McKenna and Edil. 2014311
VIPomas Literature review Worldwide Basoactive intestinal peptide Verner-Morrison syndrome, Pancreatic cholera, WDHA syndrome 0.05-0.2 N/G <10% Ito et al. 2012309; McKenna and Edil. 2014311
Glucagonoma Literature review Worldwide Glucagon Glucagonoma 0.01-0.1 N/G <10% Ito et al. 2012309; McKenna and Edil. 2014311
Somatostatinoma Literature review Worldwide Somatastatina Somatastatinoma <0.1% N/G <5% Ito et al. 2012309; McKenna and Edil 2014311
ACTHomas Literature review Worldwide Adrenocorticotropic hormone (ACTH) ACTHoma <0.1% N/G N/G Ito et al. 2012309
pNET causing carcinoid syndrome Literature review Worldwide Serotonin


PET causing carcinoid syndrome <0.1% N/G N/G Ito et al. 2013315
pNET causing hypercalcemia Literature review Worldwide PTHrP PTHrPoma <0.1% N/G N/G Ito et al. 2012309

Abbreviations: N/A, not applicable; N/G, not given.

While most pNETs arise sporadically, less than 10% are associated with one of four inherited syndromes: multiple endocrine neoplasia 1 (MEN1), von Hippel Lindau disease (VHL), neurofibromatosis-1 (NF1), or tuberous sclerosis complex (TSC).309,311 Prevalence of familial forms of pNETs syndromes are shown in Table 7.2. Inherited cases of pNETs arise at an earlier age than sporadic disease.309 Approximately 50% of all MEN1 patients develop functioning pNETs by age 50 years, and Zollinger-Ellison syndrome in MEN1 patients develops a decade earlier than in sporadic gastrinomas.311 Clinical signs of Pnet in MEN1 patients are seen in only 20-80% of cases, while microscopic signs of disease are present in 80-100%, suggesting that there are many asymptomatic cases.311

Table 7.2. Prevalence of familial Pancreatic Neuroendocrine Tumor (pNET) syndromes from literature reviews, worldwide.
Familial pNET syndrome Prevalence (per 100,000 population) Prevalence of pNETs in the familial pNET syndrome Prevalence of pNET subtype (%) References
MEN1 1-10 Gastrinomas 54 Ito et al. 2012309; McKenna and Edil. 2014311; Sadowski and Triponez. 2015316
Insulinomas 18
Glucagonomas 3
Vipomas 3
GRFomas and


von Hippel-Lindau disease (VHL) 2-3 Single non-functioning pNET (98%) 10-17 Ito et al. 2012309; McKenna and Edil. 2014311
Neurofibromatosis-1 (NF-1) (Von Recklinghausen disease, VRH) ~25 Duodenal Ssomas,

Rare pNETs


Tuberosis sclerosis (TSC) 10 Uncommon Rarely develop functioning pNETS,

non-functioning pancreatic endocrine tumors (PET)

A number of methods for imaging analysis of pNETs have similar sensitivities: CT (62-83%), magnetic resonance imaging, MRI (85-100%), somatostatin receptor scintigraphy, SRS (75-100%), positron-emission tomography, PET (94-100%), and endoscopic ultrasonography, EUS (82%).311  While CT and MRI can identify 30-66% of insulinomas, endoscopic ultrasonography and EUS show a higher sensitivity for detecting insulinomas and are also more useful in identifying small tumors than CT and MRI. CT/MRI, octreotide scan and endoscopic ultrasound can be used to detect gastrinomas, glucagonomas and VIPomas.317

Recently, next generation sequencing (NGS) has been used to identify the frequency of novel somatic gene mutations associated with pNET (Table 7.3).

Table 7.3. Frequency of genetic mutations associated with pNETs, worldwide.
Gene Mutation frequency (%)
MEN1 44
mTOR 16

Source: McKenna and Edil. 2014311



Demographic data for pNETs is limited; however, they are reported to be more common in Caucasians  (84%), and in males (55%).311 The mean age at onset for pNETs is 55.2 years for functioning tumors, and 58.8 years for nonfunctioning tumors. The overall pNET incidence per 100,000 increased with age from 1 at 15-19 years, to peak at 76 at age 70-79 years, and reflected that of non-functional pNETs.318



In 2013, Ter-Minassian reported that of the 900 neuroendocrine tumor patients at a single institute, 23% had pNET with a 5-year disease-free survival rate of 57%, and a median disease-free survival of 5.8 years. The median duration before diagnosis was 3.4 months, with 19.5% reporting durations of 1-5 years, 2.5% of 5-10 years, and 2% of >10 years. The overall survival was related to the tumor site: with small bowel NET patients showing double the survival duration of pNET (Table 7.4). The authors also concluded that while most cases were diagnosed soon after clinical onset, prolonged time from onset to diagnosis was common.319

Table 7.4. Comparison of survival rates for neuroendocrine tumors (NETs).
Data source Method Population Site of neuroendocrine tumor Percentage of cohort (%) 5-year disease-free survival rates  (%) Median disease-free survival (years) Median estimated overall survival for metastatic patients (years) Median survival duration for metastatic patients *(years)
Gastrointestinal clinic at Dana-Farber Cancer Institute (DFCI) 2003-2010. Medical records or social security death index Prospective Institutional database study US, patients with resected, localized disease (n=354) All 100 56 5.8 8.0 5.2
Small bowel NET 38 57 5.8 10.1 7.9
Pancreatic NET (pNET) 23 42 4.1 5.9 3.9
Other site NETs (unknown primary site, bronchi, appendix, stomach, other origins)


39 N/G 7.3 5.9 3.7
Abbreviations: N/G, not given.
Note: *, calculated using modified Kaplan-Meier analysis that corrects for immortal time bias or left truncation bias in institutional databases.

Source: Ter-Minassian et al. 2013319

Ter-Minassian and colleagues in 2013 reported statistical bias for shorter survival times in Pnet patients (at a single institute) than those with small bowel carcinoids (Table 7.4). The authors suggested that most studies overestimated survival time because of variability in times of patients entering a study after diagnosis.319

Approximately 21% of patients presenting with pNETs have locally advanced disease, while 60% have metastatic disease.311,320 The chance of pNETS (except insulinomas) becoming malignant is estimated to be ~50%.309 pNETs are the most common underlying cause of MEN1-associated mortality.316 MEN1 patients have a 50% 15-year survival rate for metastatic gastrinomas. Small tumors (<2cm) have no difference in life-expectancy compared to MEN1 with no pNETS.311

In a retrospective study of 324 patients with pNETs, Ekeblad and colleagues reported a mean overall survival of 8.25 years, a 5-year survival rate of 64%, and a 10-year survival rate of 44%. While hereditary and non-functioning tumors were not associated with poor prognosis, tumor staging was associated (Table 7.5).321 Similarly, Wang and colleagues reported that resectability did not influence long-term survival for functioning or non-functioning pNETS (Table 7.5), and that prognosis was dependent on functioning status of the tumor, tumor stage, lymph node status, and pathological classification.322

Table 7.5. Survival rates for patients with neuroendocrine tumors (NETs), worldwide.
Data source Population Neuroendocrine tumors 5-year survival rate (%) 10-year survival rate (%) References
Literature review Pancreatic endocrine tumors (pNETs) Untreated liver metastases in patients with nonfunctioning pNET 30–40 McKenna et al. 2014311; Ehehalt et al. 2009323
After hepatic surgery of patients with nonfunctioning pNET metastases 47-76
SEER 13 database, 1973-2000 adults age³18 years (44.8% female) overall survival (n=1,483)


Functioning pNETs 47.6 33.7 Halfdanarson et al. 2008318; Ehehalt et al. 2009323
Nonfunctioning pNETs 31.3 17.0
Mayo Clinic, Rochester, 1927-1986 (60-year study) US, age range 8-82 years, median age 47 years (59% female) (n=224) All functioning insulinoma 88 Service et al. 1991324; Ehehalt et al. 2009323
Malignant insulinoma (5.8%) 29
Retrospective review of patients treated at Department of Surgery, University of Vienna, Austria Austria, mean age at operation 49.5 years, range 12-80 years, pNET surgically treated (n=100) Insulinoma 97 Schindl et al. 2000325; Ehehalt et al. 2009323
Gastrinoma 50
National Institute of Health, December 1981- August 1998 Zollinger-Ellison syndrome or malignant gastrinoma patients who underwent laparotomy, (n=151):

sporadic gastrinomas (n=123); MEN1 (n=28); mean follow up 8 ±4 years.

DFS = disease-free survival;

DSS = disease-specific survival

Sporadic gastrinoma



40 (DFS);

100 (DSS)



34 (DFS); 95 (DSS) Norton et al. 1999326




MEN1 4 (DFS)

100 (DSS)


0 (DFS)

86 (DSS)

Medical records of patients diagnosed and treated at tertiary referral center, retrospective study Sweden, patients at a single institution; median follow up 54 months (n=324) pNET 64 44 Ekebald et al. 2008321
Literature review Pancreatic endocrine tumors Insulinoma 97 Mansour and Chen. 2004327
Gastrinoma 60-70
Glucagonoma 50-60
VIPoma 80-90
Somatostatinoma 90
Non-functioning and PPoma 80-90
Cohort study Taiwan, pancreatic and peripancreatic tumors; n=93 Functional (n=39);

Nonfunctional (n=54)


(disease-specific survival)

86.4 Wang et al. 2011322
Unresectable (disease-specific survival) 65.6
SEER 9 registries, 1975-2006 children and adolescents, age <30 years (n=1,073) NETs 83-84 Navalkele et al. 2011328
Malignant carcinoids of appendix, lung, and hindgut 96-100
children and adolescents, females, age <30 years NETs of the cervix and ovary only (small cell carcinomas) 24-29



Diagnosis and treatment for NETs involves multidisciplinary teams for diagnosis, surgery, surveillance, chemotherapy, and targeted therapies.311,316,329 Familial disorders are more complicated to diagnose and treat than sporadic ones because of their earlier presentation and the presence of multiple tumor sites.311,315,316 Serotonin is secreted by ~70% of tumors in jejunum, ileum, proximal colon and appendix, but is uncommon in pNETs; and secreted by 10-35% of NETs in the stomach and respiratory tract. In some cases, measurement of Chromogranin A or serotonin in blood, or its breakdown product 5-hydroxyindoleactic acid (5-HIAA) in urine, can assist in diagnosis but low sensitivity and false positives are problematic, and new circulating biomarkers such as the NETest are being developed.329 As 90% of tumors are not associated with hypersecretion of hormones or peptides (non-functioning), the first recognizable symptom (the tumor mass) is usually identified later in disease progression.329 Additionally, with increased imaging, pNETs are increasingly found incidentally and observation (rather than surgery) has become a reasonable choice for small pNETs (<2cm). Detecting functioning tumors is also problematic; the hypersecretion of hormones can produce syndromes that mimic other disorders, potentially delaying correct diagnosis.329 Treatments for functioning NETs must control hormone excess as well as the tumor.311,329,330 In all cases of localized tumors, surgical resection should be offered, and if inoperable or in cases of metastases, combination therapy with surgical debulking, medical therapy to control hormone secretion and tumor growth are the treatments of choice. Table 7.6  summarizes health outcomes of treatment strategies for pNETs.

Table 7.6. Health outcomes measures for pancreatic neuroendocrine tumors (pNETs) from literature reviews.
pNETS Malignancy frequency Hormone-related symptoms Treatment Outcome References

(Zollinger-Ellison syndrome)

60–90% Peptic ulcer or GERD Need surgical resection for all pNET types

Proton-pump inhibitors for acute and long-term control (including omeprazole, olansoprazole.

>98% effective Ito et al. 2013315; McKenna and Edil. 2014311; Ehehalt et al. 2009323; Ramage et al. 2012329
Insulinomas 5–15% Hypoglycemia Surgical resection Surgery successful in >95% of cases Ito et al. 2013315; McKenna and Edil. 2014311; Sadowski and Triponez. 2015316
Diazoxide inhibits insulin release Hypoglycemia controlled in 50-60% of patients
VIPomas 70–90% Severe diarrhea, leads to dehydration and hypokalemia


Long-acting somatostatin analogues: octreotide-LAR, lanreotide-autogel Diarrhea controlled in >90% of patients Ito et al. 2013315; McKenna and Edil. 2014311; Burns and Edil. 2012330; Ramage et al. 2012329
Glucagonoma 60–75% Dermatitis, diabetes, diarrhea, deep vein thrombosis Long-acting somatostatin analogues: octreotide-LAR, lanreotide-autogel Life-threatening skin lesion necrolytic migratory erythmia (NME) controlled in 50-90%, improvement in weight loss, abdominal pain, diarrhea, diabetes does not improve Ito et al. 2013315; Burns and Edil. 2012330; Ramage et al. 2012329
Hypoglycemia Diazoxide Hypoglycemia controlled in 50-60% of patients Ito et al. 2013315
Table 7.7 Health outcome measures for current standards of care for neuroendocrine tumors (NETs) from literature reviews.
Disease Therapeutic strategy Outcome References
NETs Lung resection 67-96% 5-year survival, depending on histology and lymph node involvement; 5-year survival 87%, post-operative mortality 6% Ramage et al. 2012329
Liver resection Liver metastases curative in ~10% of cases, if confined to single lobe
Liver transplant for end-stage NET and uncontrollable symptoms, and unresponsive to other therapies Highest disease-free survival up to 77% at 1-year; actural disease-free survival 62% at 1-year and 23% at 5 years
Liver metastases Surgery if debulking of >90% of the tumor is possible: only 5-15% of patients are candidates Debulking of >90% of tumor can extend survival; resolution of symptoms in 96% in one study. Reoperationon recurrence can extend survival up to 70% after 10 years. McKenna and Edil. 2014311; D’Haese et al. 2014312
Pancreatic neuroendocrine neoplasms (pNENs) Surgery for curative resection, and to prevent or delay local or metastaic recurrence Survival benefit of 79 months for resected patients relative to nonresected (114 vs. 35 months) D’Haese et al. 2014312

Current standard of care strategies for NETs are outlined in Table 7.7. Surgery is the first-line therapy when NETs are resectable, and is also recommended for debulking of liver metastases and correction of cardiac valvular disease.311,329 Although surgery is the primary treatment, pNETs are more sensitive to chemotherapy than other NETs such as carcinoid tumors.311 The therapeutic approaches available for NET metastatic disease, advanced pNET, and carcinoids include surgery, radiotherapy, targeted molecular therapy and chemotherapy (Table 7.8). New cytotoxic agents namely taxanes, gemcitabine, pemetrexed, and topotecan have not been as successful, showing response rates under 10%.329 On February 26th 2016, the FDA approved the use of Afinitor (everolimus) in adult patients with progressive, non-functional gastrointestinal and lung NETs that are unresectable, locally advanced or metastatic (Table 7.8).331,332

Table 7.8. Health outcomes measures for advanced pNETs.
Therapeutic strategy Indication Studies Response Survival References
Chemotherapy pNET streptozotocin, 5-fluorouracil and doxorubicin In a US retrospective series of locally advanced and metastatic pancreatic endocrine carcinomas (n=84); 39% showed tumor response; with median duration 9.3 months 2-year overall survival rates was 74%; 2-year progression-free survival rate was 41% Kouvaraki et al. 2004333; McKenna and Edil. 2014311
temozolomide + capecitabine In a single-arm retrospective study of metastatic pancreatic endocrine carcinomas (n=30), tumor response rate was seen in 70% of patients 2-year survival rate of 92%: median progression-free survival of 18 months; grade 3 or 4 adverse events in 12% Strosberg et al. 2011334; McKenna et al. 2014311
Peptide receptor radiotherapy (PPRT) Gastroenteropancreatic NETs Lu-177 labelled Complete tumor response in2%; partial tumor response in 28%(n=504)


Minor tumor response (decrease in size by >25% but <50%) in 16%; median time to disease progression 40 months; median overall survival 48 months; median overall survival from diagnosis 128 months; Serious treatment toxicity 3.6%. Kwekkeboom et al. 2008335; McKEnna and Edil. 2014311
Targeted molecular therapy Progressing, advanced, or symptomatic pNETs Everolimus: Mtor signalling inhibitor US, low-or intermediate grade pNETs with radiologic progression <12 months; n=207 patients, n=203 placebo.  65% reduction in estimated risk of progression/death. 34% of treated patients alive and progression free for 18 months vs. 9% with placebo Median progression free survival of 11 months for everolimus, 4.6 months for placebo. Stable disease (by RECIST) in 73% for everolimus and 51% for placebo. Yao et al. 2011336; Ramage et al. 2012329
Sunitinib: tyrosine kinase inhibitor targets VEGF receptors France, phase III trial, n=171, advanced well differentiated pNETs, tumor response rate 9.3% for treatment vs. 0% placebo Median progression free survival 11.4 months for sunitinib vs. 5.5 months placebo Raymond et al. 2011337; McKenna and Edil. 2014311

Abbreviations: NETs, neuroendocrine tumors; pNETs, pancreatic neuroendocrine tumors.

Following a systematic review of the literature, Broder and colleagues reported that above-label use of octreotide-LAR may be warranted to control symptoms, and delay tumor progression, in NETs, particularly carcinoid syndrome and VIPomas.338 In all cases of NETs, long-term surveillance for recurrent disease is recommended.339,340,329


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