Materiały źródłowe

• #1 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5719919/

  Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. […] For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. Chromogranin A is the most sensitive marker but has insufficient specificity. In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. […] Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. Positron emission tomography (PET) scan with 18F-fluorodeoxyglucose cannot visualize PNETs, but PET with 68-Ga DOTATATE can. Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy.

• #2 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. […] For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. […] In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. […] Multiphasic magnetic resonance imaging can detect PNETs smaller than 2 cm and small liver metastasis compared with other modalities. […] Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. […] Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs.

• #3 Tests for Pancreatic Neuroendocrine Tumor | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/how-diagnosed.html

  Certain signs and symptoms might suggest that a person could have a pancreatic neuroendocrine tumor (NET), but they will need tests to confirm the diagnosis. […] In taking your medical history, the doctor will ask questions about your general health, lifestyle habits, symptoms, and risk factors. […] Your doctor will also examine you to look for signs of a pancreatic neuroendocrine tumor (pNET) or other health problems. […] If the exam results are abnormal, your doctor will probably order tests, such as imaging, labs, or other procedures, to help find the problem. […] A CT scan uses x-rays taken from different angles, which are combined by a computer to make detailed pictures of the organs. […] This test is most often used to look at the belly (abdomen) to see the pancreas clearly and if the pNET has spread to nearby lymph nodes or other organs such as the liver.

• #4 Pancreatic Neuroendocrine Tumors: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/21970-pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors (pancreatic NETs, also called islet cell tumors) are rare tumors that start in your pancreas endocrine cells. They can be cancerous (malignant) or benign (noncancerous). Without treatment, malignant tumors may spread to other areas of your body. […] A healthcare provider will do a physical examination. Theyll ask about your symptoms. They may ask when your symptoms started and if theyre getting worse. Theyll also ask if anyone in your biological family has certain inherited disorders that increase pancreatic NET risk. […] Providers use these test results to see if a tumor is functioning or non-functioning. Functioning tumors release unusually large amounts of hormones. Non-functioning tumors dont release hormones. Test results also help providers identify the type of pancreatic neuroendocrine tumor.

• #5 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. […] For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. […] In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. […] Multiphasic magnetic resonance imaging can detect PNETs smaller than 2 cm and small liver metastasis compared with other modalities. […] Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. […] Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs.

• #6 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. […] MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 2-3 cm. […] Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs. […] Contrast-enhanced EUS is helpful in categorizing small hypervascular PNETs, which are incidentally found on other imaging modalities. […] Early detection through advanced imaging modalities and proper classification and staging with the recently updated classification system are important in providing prognostic information and decision for the management of PNETs.

• #7 Pancreatic Neuroendocrine Tumors (PNETs) – Pancreatic Cancer Action Network

  https://pancan.org/facing-pancreatic-cancer/about-pancreatic-cancer/types-of-pancreatic-cancer/endocrine-pancreatic-neuroendocrine-tumors/

  Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs) account for less than 10% of all pancreatic tumors. They may be benign or malignant and they tend to grow slower than exocrine tumors. […] Pancreatic neuroendocrine tumors are either functional (produce hormones) or nonfunctional (produce no hormones). […] As a result, these tumors are typically diagnosed once the tumor is advanced and is causing symptoms such as pain or jaundice. […] Nonfunctional islet cell tumors are usually malignant. They are hard to detect. […] About 30-75% of people with MEN1 will develop pancreatic neuroendocrine tumors. Gastrinomas are the most common pancreatic neuroendocrine tumors in individuals with MEN1. The second most common are insulinomas. The tumors in the pancreas may be malignant and generally appear in individuals in their 30s or 40s.

• #8 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. […] Endocrine testing, imaging, and histological evidence are necessary to accurately diagnose PNETs. […] A complete diagnosis should establish the PNET nature, assess the tumor grade, identify the primary and metastatic loci, and determine whether the tumor is functioning. […] If hormonal hypersecretion syndrome is suspected, appropriate biochemical testing is performed to determine hormonal hypersecretion and followed by imaging, endoscopy, and biopsy. […] Biochemical testing should ideally be performed even if hormonal hypersecretion syndrome is not evident because it could be at the subclinical stage, and the hypersecreted hormones can be used as tumor markers during follow-up evaluations.

• #9 Pancreatic Neuroendocrine Tumors – PNETs | Choose the Right Test

  https://arupconsult.com/content/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors (PNETs) are rare tumors of pancreatic islet cells that account for approximately 10% of pancreatic tumors by prevalence. […] Laboratory testing for PNETs involves evaluation of specific biochemical markers, as determined by the clinical presentation. Genetic testing for hereditary syndromes should be considered for all patients with PNETs. […] In cases of suspected pancreatic neuroendocrine tumors (PNETs), the clinical presentation helps to determine which biochemical markers to test. […] Genetic testing should be considered in all patients with PNETs. […] Histopathologic testing, including immunohistochemical (IHC) tests, also has a role in a workup for PNETs. […] Follow-up testing is recommended 12 weeks to 12 months after resection (and sooner if symptoms appear) and every 6-12 months thereafter for up to 10 years. Monitoring should include biochemical marker testing, particularly if a functional syndrome was diagnosed. […] Once a PNET diagnosis has been established, patients should be monitored for the development of other conditions associated with MEN1.

• #10 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5719919/

  Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. […] For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. Chromogranin A is the most sensitive marker but has insufficient specificity. In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. […] Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. Positron emission tomography (PET) scan with 18F-fluorodeoxyglucose cannot visualize PNETs, but PET with 68-Ga DOTATATE can. Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy.

• #11 Diagnostic work-up of gastroenteropancreatic neuroendocrine tumors | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-diagnostic-work-up-gastroenteropancreatic-neuroendocrine-tumors-S1807593222024048

  Neuroendocrine tumors are a heterogeneous group of malignancies that present a diagnostic challenge. The diagnosis is based on histopathology, imaging, and circulating biomarkers. The histopathology should contain specific neuroendocrine markers such as chromogranin A, synaptophysin, and neuron-specific enolase and also an estimate of the proliferation by Ki-67 (MIB-1). Standard imaging procedures consist of computed tomography or magnetic resonance imaging together with somatostatin receptor scintigraphy. 68Ga-DOTA-octreotate scans will in the future replace somatostatin receptor scintigraphy because they have higher specificity and sensitivity. Other positron imaging tomographic scanning tracers that will come into clinical use are 18F-DOPA and 11C-5HTP. […] The initial diagnostic approach in patients with NETs includes histological examination, which is always required before therapeutic decisions are made. Clinicians should also consider performing repetitive biopsies to reassess the prognosis if the disease course changes significantly. The following investigations are also required: (a) immunohistochemical markers and detailed histological analysis; (b) assessment of the primary tumor and the extent of extrahepatic spread by imaging, including patterns of hepatic metastases; and (c) biochemical assessment of functionality and general tumor markers.

• #12 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  Pancreatic neuroendocrine neoplasms (pNENs) and carcinoid tumors develop from the islets of Langerhans in the pancreas and enterochromaffin cells in the gastrointestinal tract, respectively. […] The incidence of GEP-NENs has expanded over the past few decades, likely due to increased emphasis on screening and widespread use of cross-sectional imaging with computed tomography (CT) and magnetic resonance imaging (MRI). […] Also, recent technological advancements in endoscopy have facilitated the early diagnosis of neuroendocrine neoplasms. […] In this review, we will summarize the clinical presentations and diagnostic criteria of each functional GEP-NEN. […] The most dependable test to diagnose insulinomas is the measurement of serum glucose, proinsulin, and C-peptide levels after an overnight fast and every 4 hours for up to 72 hours.

• #13 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  Gastrinomas can be diagnosed by measuring the gastric pH and fasting serum gastrin (FSG) levels. […] To diagnose a VIPoma, patients must present with secretory diarrhea and plasma VIPs 500 pg/mL. […] Clinical documentation of necrolytic migratory erythema with a fasting plasma glucagon value 500 pg/mL can confirm the diagnosis. […] Somatostatinomas are usually diagnosed after immunohistochemical staining for somatostatin. […] In patients with suspected carcinoid syndrome, measuring urinary 5-Hydroxyindoleacetic acid (5HIAA) for 24 hours (the normal value for urinary 5-HIAA ranges 3-15 mg/day) is the recommended initial test, with a sensitivity and specificity of 73% and 100%, respectively. […] Among many imaging modalities used to screen for early diagnosis of pNENs in MEN-1 syndrome patients, Endoscopic Ultrasound (EUS) outperformed CT, MRI, and somatostatin receptor scintigraphy (SRS).

• #14 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment – NCI

  https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq

  Other kinds of lab tests are used to check for the specific type of pancreatic NETs. […] The following tests and procedures may be used: Fasting serum gastrin test; Basal acid output test; Secretin stimulation test; Somatostatin receptor scintigraphy. […] Fasting serum glucose and insulin test; Fasting serum glucagon test; Serum VIP (vasoactive intestinal peptide) test; Fasting serum somatostatin test. […] Certain factors affect prognosis (chance of recovery) and treatment options. […] Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The type of cancer cell; Where the tumor is found in the pancreas; Whether the tumor has spread to more than one place in the pancreas or to other parts of the body; Whether the patient has MEN1 syndrome; The patient’s age and general health; Whether the cancer has just been diagnosed or has recurred (come back).

• #15 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  Gastrinomas can be diagnosed by measuring the gastric pH and fasting serum gastrin (FSG) levels. […] To diagnose a VIPoma, patients must present with secretory diarrhea and plasma VIPs 500 pg/mL. […] Clinical documentation of necrolytic migratory erythema with a fasting plasma glucagon value 500 pg/mL can confirm the diagnosis. […] Somatostatinomas are usually diagnosed after immunohistochemical staining for somatostatin. […] In patients with suspected carcinoid syndrome, measuring urinary 5-Hydroxyindoleacetic acid (5HIAA) for 24 hours (the normal value for urinary 5-HIAA ranges 3-15 mg/day) is the recommended initial test, with a sensitivity and specificity of 73% and 100%, respectively. […] Among many imaging modalities used to screen for early diagnosis of pNENs in MEN-1 syndrome patients, Endoscopic Ultrasound (EUS) outperformed CT, MRI, and somatostatin receptor scintigraphy (SRS).

• #16 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment – NCI

  https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq

  Other kinds of lab tests are used to check for the specific type of pancreatic NETs. […] The following tests and procedures may be used: Fasting serum gastrin test; Basal acid output test; Secretin stimulation test; Somatostatin receptor scintigraphy. […] Fasting serum glucose and insulin test; Fasting serum glucagon test; Serum VIP (vasoactive intestinal peptide) test; Fasting serum somatostatin test. […] Certain factors affect prognosis (chance of recovery) and treatment options. […] Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following: The type of cancer cell; Where the tumor is found in the pancreas; Whether the tumor has spread to more than one place in the pancreas or to other parts of the body; Whether the patient has MEN1 syndrome; The patient’s age and general health; Whether the cancer has just been diagnosed or has recurred (come back).

• #17 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. […] MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 2-3 cm. […] Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs. […] Contrast-enhanced EUS is helpful in categorizing small hypervascular PNETs, which are incidentally found on other imaging modalities. […] Early detection through advanced imaging modalities and proper classification and staging with the recently updated classification system are important in providing prognostic information and decision for the management of PNETs.

• #18 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. […] Endocrine testing, imaging, and histological evidence are necessary to accurately diagnose PNETs. […] A complete diagnosis should establish the PNET nature, assess the tumor grade, identify the primary and metastatic loci, and determine whether the tumor is functioning. […] If hormonal hypersecretion syndrome is suspected, appropriate biochemical testing is performed to determine hormonal hypersecretion and followed by imaging, endoscopy, and biopsy. […] Biochemical testing should ideally be performed even if hormonal hypersecretion syndrome is not evident because it could be at the subclinical stage, and the hypersecreted hormones can be used as tumor markers during follow-up evaluations.

• #19 Pancreatic Neuroendocrine Tumor

  https://www.ahn.org/services/cancer/types/pancreatic-neuroendocrine-tumor-pnet

  Blood and urine tests may reveal elevated levels of hormones secreted by the tumor, such as chromogranin A, gastrin, insulin, glucagon, or vasoactive intestinal peptide (VIP). […] Imaging studies play a crucial role in diagnosing and staging pancreatic neuroendocrine tumors (PanNETs). They help to locate the tumor, determine its size and extent, and assess whether it has spread to other parts of the body (metastasis). […] While imaging studies are essential for detecting pancreatic masses, a biopsy is generally necessary for confirming a diagnosis of PanNET, determining its grade and type, and guiding treatment decisions.

• #20 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5719919/

  PNETs are divided into functional and non-functional tumors. About 90% of PNETs are classified as non-functional tumors, and the remaining 10% as functional tumors. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs. Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 23 cm. Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs.

• #21 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. […] MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 2-3 cm. […] Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs. […] Contrast-enhanced EUS is helpful in categorizing small hypervascular PNETs, which are incidentally found on other imaging modalities. […] Early detection through advanced imaging modalities and proper classification and staging with the recently updated classification system are important in providing prognostic information and decision for the management of PNETs.

• #22 Computed Tomography in the Diagnosis of Neuroendocrine Tumors of the Pancreas

  https://www.sgo-iasgo.com/article/computed-tomography-in-the-diagnosis-of-neuroendocrine-tumors-of-the-pancreas

  Neuroendocrine tumors of the pancreas (NETP) are rare malignancies that originate from pancreatic neuroendocrine cells and may be functional in nature through the clinical picture secondary to hormonal hypersecretion. […] This article presents two cases diagnosed with NETP, of two men aged 58 and 77 years respectively in the Hospital, detected by CT and subsequently confirmed histopathologically, who presented with non-specific acute abdominal symptoms with altered general condition and fever. […] CT scan showed the presence of multiple NETP in both cases with liver metastasis in the 77-year-old patient. […] From a diagnostic point of view, the most used and reliable method remains CT with contrast substance to which the use of MRI is added. […] The preferred method for imaging diagnosis and to appreciate the functional character of TNEP remains 68GA-DOTATOC-PET/CT, but the most accurate results are obtained as a result of using the alternative method with 18F-FDG PET/CT in which the activity can be observed and agglomeration of protein analogs of somatostatin.

• #23 Computed Tomography in the Diagnosis of Neuroendocrine Tumors of the Pancreas

  https://www.sgo-iasgo.com/article/computed-tomography-in-the-diagnosis-of-neuroendocrine-tumors-of-the-pancreas

  Confirmation of the diagnosis of certainty is done by direct intraoperative or percutaneous echo-guided biopsy. […] Computed tomography (CT) should be performed with a multidetector CT scanner with 1.5 mm slice thickness and multiplanar reconstructions with the use of CT contrast. […] Studies demonstrate an outstanding performance of CT imaging in the diagnosis and detection of TNEP with a sensitivity and specificity of approximately 73% and 96% of cases, respectively. […] On the other hand, MRI can be used alternatively for the detection of TNEP as well as for abdominal staging, CT can be used alternatively or additionally, when there is clinical suspicion for a small TNEP, equivocal imaging morphology or not detectable by CT. […] The importance of computed tomography was highlighted in both cases presented.

• #24 Current Diagnostics and Treatments for Pancreatic Neuroendocrine Tumors Upper Gastrointestinal Cancer – GMKA – Global Medical Knowledge Alliance

  https://gmka.org/current-diagnostics-and-treatments-for-pancreatic-neuroendocrine-tumors/

  The diagnosis of PNETs tends to be delayed due to the ambiguity of symptoms one may experience. […] In order to accurately diagnose PNETs, imaging and histological evidence from a biopsy are required. Hormonal testing is required if there is clinical suspicion based on history. Computerized tomography (CT) is the gold standard imaging study for PNETs. […] The pancreas should be examined with intravenous iodinated contrast typically utilizing a specialized pancreas protocol CT which includes imaging in three phases, early arterial phase, later arterial phase, which provides the best imaging of the pancreatic parenchyma, and venous phase, which provides optimal liver enhancement. […] CT has been found to have a specificity of 96% and a sensitivity of 73% for the diagnosis of PNET. […] Localization of PNETs with the method listed above has a relatively high failure rate. The gold standard for both biopsy and histological diagnosis is endoscopic ultrasound, as this method is very sensitive to PNETs. […] A helpful tool is somatostatin receptor scintigraphy. The somatostatin analog of choice is Gallium- 68. A 68-Gallium DOTATATE PET-CT scan allows for clearer imaging and higher spatial resolution when compared to other forms of imaging for both poorly- and well-differentiated tumors.

• #25 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. […] For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. […] In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. […] Multiphasic magnetic resonance imaging can detect PNETs smaller than 2 cm and small liver metastasis compared with other modalities. […] Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. […] Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs.

• #26 Imaging presentation of pancreatic neuroendocrine neoplasms | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1007/s13244-018-0658-6

  Pancreatic neuroendocrine neoplasms (P-NENs) are the second most common solid pancreatic neoplasms. P-NENs have a wide range of imaging features presentations and they can be detected with typical and atypical imaging presentations. Typical and atypical appearances can be explained by pathologic correlations. P-NENs are generally hypervascular lesions, showing a typical enhancement behavior after contrast media injection during imaging methods, but they could also have different imaging features, creating some difficulty in differential diagnosis. For this reason, radiologists should be aware of different imaging presentations of these neoplasms. Radiological evaluation has a critical role in P-NENs identification, characterization, and staging of these neoplasms, especially in those cases in which surgery is the treatment of choice. The present paper shows, indicating the underlying pathologic correlations, typical and atypical presentations of NENs.

• #27 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. […] MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 2-3 cm. […] Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs. […] Contrast-enhanced EUS is helpful in categorizing small hypervascular PNETs, which are incidentally found on other imaging modalities. […] Early detection through advanced imaging modalities and proper classification and staging with the recently updated classification system are important in providing prognostic information and decision for the management of PNETs.

• #28 Pancreatic neuroendocrine tumours: spectrum of imaging findings – Bicci – Gland Surgery

  https://gs.amegroups.org/article/view/52017/html

  With the advent of Radiomics, the new method in which the information is hidden inside the radiological images and can be extracted using advanced texture and shape analysis, it has been possible to extract lots of information regarding the structure we need to study. More information in the characterization of these different grades can be obtained with MRI imaging. The pancreas has a hyperintense signal on T1-weighted imaging, because of the amount of protein inside the parenchyma, therefore pNETs tend to appear hypointense in contrast with pancreatic parenchyma on un-enhanced T1-weighted images. On T2-weighted images, pNETs can be hyperintense, even if a lower signal can also be present. Diffusion weighted imaging (DWI) may also help in tumour detection. […] pNETs can be hyperfunctioning, with the production of a lot of different hormones such as insulin, glucagon, gastrin, vasoactive intestinal peptide (VIP) and somatostatin, that can create various clinical manifestation. This subtype of tumours tends to present earlier, with clinical signs and symptoms. At CT examination, these lesions are isodense at baseline, homogeneously hyperdense in the pancreatic phase, and hypodense in the venous one because of their washout phenomena. In order to distinguish between typical and atypical insulinomas, multiphase imaging is essential. MRI shows a homogeneously enhanced lesion with low signal on T1-weighted imaging and high signal on T2-weighted one.

• #29 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. […] MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 2-3 cm. […] Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs. […] Contrast-enhanced EUS is helpful in categorizing small hypervascular PNETs, which are incidentally found on other imaging modalities. […] Early detection through advanced imaging modalities and proper classification and staging with the recently updated classification system are important in providing prognostic information and decision for the management of PNETs.

• #30 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  EUS despite the fact that compared to computed tomography and magnetic resonance imaging (MRI) it is much more dependent on the operators experience is currently considered to be the most sensitive method of detecting pancreatic neuroendocrine tumours, especially small ones with diameter 2 cm (even 12 mm). […] The sensitivity of EUS is 94100% in small insulin-secreting tumours. […] The role of surgical treatment in patients diagnosed with ZES but having negative results of preoperative imaging examinations (lack of tumour identification and location) remains debatable. […] The method of surgical treatment of PanNETs depends on the degree of tumour malignancy, its size, location, infiltration of adjacent organs, the presence of distant metastases, the patients general condition, and the ability to control clinical symptoms. […] The decision regarding PanNET treatment method should be made by a multidisciplinary team of physicians experienced in the treatment of neuroendocrine tumours.

• #31

  https://journals.lww.com/10.1097/MCG.0000000000000152

  Pancreatic neuroendocrine tumors (PNETs) are rare pancreatic neoplasms comprising only 1% to 2% of all pancreatic tumors. […] However, a significant proportion of PNETs may not be visualized on conventional imaging such as computed tomography, magnetic resonance imaging, and somatostatin receptor scintigraphy. Endoscopic ultrasound (EUS) has become an integral part of the diagnosis of PNETs because of its high sensitivity for detecting, localizing, and diagnosing PNETs. EUS-guided tissue acquisition provides histologic and immunologic confirmation, and may also allow prognostication about tumor behavior. […] In addition to preoperative assessment of these tumors, EUS has also been shown to have an important role in nonoperative management of small nonfunctional PNETs. Finally, recent developments suggest that interventional EUS may be used to aid intraoperative localization of PNETs and to deliver therapeutic agents for the treatment of PNETs.

• #32 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  EUS allows a detailed examination of the pNENs, providing information on the location and size of the lesion that will direct treatment and management plans for the patients. […] EUS is an especially powerful diagnostic tool when the size of pNENs is 20 mm. […] A recent systemic review and meta-analysis on 864 patients revealed that the Ki-67 index of EUS-FNA extracted tissue and surgically biopsied tissues matched 80.3%, proving that grading from EUS-FNA extracted pancreatic tissues is dependable. […] One study revealed that the overall sensitivity and specificity of contrast-enhanced EUS in detecting pNENs were 78.9% and 98.7%, respectively, which has similar diagnostic accuracy to CT scans. […] In recent years, somatostatin receptor positron emission tomography/computed tomography with gallium-68 radiolabeled peptides (68Ga-SSR-PET/ CT) has emerged as a new method in diagnosing GEP-NENs.

• #33 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  EUS allows a detailed examination of the pNENs, providing information on the location and size of the lesion that will direct treatment and management plans for the patients. […] EUS is an especially powerful diagnostic tool when the size of pNENs is 20 mm. […] A recent systemic review and meta-analysis on 864 patients revealed that the Ki-67 index of EUS-FNA extracted tissue and surgically biopsied tissues matched 80.3%, proving that grading from EUS-FNA extracted pancreatic tissues is dependable. […] One study revealed that the overall sensitivity and specificity of contrast-enhanced EUS in detecting pNENs were 78.9% and 98.7%, respectively, which has similar diagnostic accuracy to CT scans. […] In recent years, somatostatin receptor positron emission tomography/computed tomography with gallium-68 radiolabeled peptides (68Ga-SSR-PET/ CT) has emerged as a new method in diagnosing GEP-NENs.

• #34 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. […] For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. […] In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. […] Multiphasic magnetic resonance imaging can detect PNETs smaller than 2 cm and small liver metastasis compared with other modalities. […] Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. […] Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs.

• #35 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5719919/

  PNETs are divided into functional and non-functional tumors. About 90% of PNETs are classified as non-functional tumors, and the remaining 10% as functional tumors. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs. Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 23 cm. Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs.

• #36 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  EUS allows a detailed examination of the pNENs, providing information on the location and size of the lesion that will direct treatment and management plans for the patients. […] EUS is an especially powerful diagnostic tool when the size of pNENs is 20 mm. […] A recent systemic review and meta-analysis on 864 patients revealed that the Ki-67 index of EUS-FNA extracted tissue and surgically biopsied tissues matched 80.3%, proving that grading from EUS-FNA extracted pancreatic tissues is dependable. […] One study revealed that the overall sensitivity and specificity of contrast-enhanced EUS in detecting pNENs were 78.9% and 98.7%, respectively, which has similar diagnostic accuracy to CT scans. […] In recent years, somatostatin receptor positron emission tomography/computed tomography with gallium-68 radiolabeled peptides (68Ga-SSR-PET/ CT) has emerged as a new method in diagnosing GEP-NENs.

• #37 Diagnosis, Management and Theragnostic Approach of Gastro-Entero-Pancreatic Neuroendocrine Neoplasms

  https://www.mdpi.com/2072-6694/15/13/3483

  Gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs) are the most common neuroendocrine tumors, with the ability of overexpressing somatostatin receptors (SSTRs) on the cells’ surface. […] The theragnostic approach, which is able to combine diagnosis and therapy, is widely applied in GEP-NENs patients through the administration of radiolabeled SSTR analogs. […] The SSTRs overexpression in GEP-NENs constitutes an ideal target for diagnosis and therapy with radiolabeled somatostatin analogs. […] In this context, nuclear medicine techniques, such as Positron Emission Tomography (PET) and Single Photon Emission Computed Tomography (SPECT), have been proposed as crucial tools in the management of GEP-NENs. […] The theragnostic approach is able to combine diagnosis and therapy by using the same molecule for the identification of a therapeutic target that can be treated with the same compound.

• #38 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  One study revealed that the sensitivity and specificity of 68Ga-SSR-PET/CT in localizing neuroendocrine tumors were 93% and 96%, respectively, outperforming CT scans. […] Assessing the extent of metastasis is essential for a comprehensive diagnosis as it can drastically influence the management of the pNENs. […] The management of pNENs is multidisciplinary, which involves octreotide (somatostatin analogs), sunitinib (tyrosine kinase inhibitor), everolimus (an mTor inhibitor), peptide receptor radionuclide therapy, and chemotherapy depending on grading and extent of metastatic disease at the time of diagnosis. […] Surgical resection remains the only curative treatment for pNENs, although the relapse rate is high. […] Therefore, functional pNENs with tumor size 20 mm are generally recommended for surgical resection.

• #39 Pancreatic Neuroendocrine Tumors – Pancreas Disease | UCLA Health

  https://www.uclahealth.org/medical-services/pancreas-disease/pancreatic-conditions/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors are often slow-growing. Even when tumors cannot be removed, patients can live a long time with few symptoms. […] Our doctors diagnose pancreatic neuroendocrine tumors with imaging tests and by analyzing biopsy samples in the lab. People with a family history of other cancers may also be at risk for pancreatic neuroendocrine tumors. Because of this connection, we also provide genetic counseling to help patients get diagnosed earlier. […] UCLA offers some of the most advanced imaging and diagnosis available for pancreatic neuroendocrine tumors, including: […] DOTA-TATE Gallium-68 PET scans: This is the most sensitive and accurate test available for pancreatic NETs. UCLA is one of just a few hospitals in the country offering this innovative test. […] Specialized CT (computerized tomography) and MRI (magnetic resonance imaging): We use advanced CT and MRI procedures that provide clearer images than standard CT and MRI imaging available at other facilities. We have specialized procedures for imaging both the pancreas and bile duct.

• #40 Current update on imaging for pancreatic neuroendocrine neoplasms

  https://www.wjgnet.com/2218-4333/full/v12/i10/897.htm

  Current and emerging radiotracers have broadened the utility of functional imaging in evaluating panNEN. […] Fluorine-18 fluorodeoxyglucose positron emission tomography (PET)/CT and somatostatin receptor imaging such as Gallium-68 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acidoctreotate PET/CT may be useful for improved identification of panNEN in comparison to anatomic modalities. […] These new techniques can also play a direct role in optimizing the selection of treatment for individuals and predicting tumor response based on somatostatin receptor expression. […] In addition, emerging methods of radiomics such as texture analysis may be a potential tool for staging and outcome prediction in panNEN, however further investigation is required before clinical implementation. […] Imaging plays a critical role in the diagnosis and management of pancreatic neuroendocrine neoplasms.

• #41 Current update on imaging for pancreatic neuroendocrine neoplasms

  https://www.wjgnet.com/2218-4333/full/v12/i10/897.htm

  Enhancement patterns and diffusion-weighted imaging aid the detection and classification of these lesions. […] Contrast-enhanced magnetic resonance imaging is useful for the evaluation of hepatic metastases. […] Dual-tracer positron emission tomography/computed tomography with Gallium-68 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acidoctreotate and Fluorine-18 fluorodeoxyglucose may be particularly useful for distinguishing grade 3 pancreatic neuroendocrine tumor from pancreatic neuroendocrine carcinoma. […] Furthermore, these advanced imaging techniques can help in the staging and detection of distant metastases. […] Evaluation of somatostatin receptor expression and metabolic activity with functional imaging can help select optimal treatment.

• #42 Diagnosis, Management and Theragnostic Approach of Gastro-Entero-Pancreatic Neuroendocrine Neoplasms

  https://www.mdpi.com/2072-6694/15/13/3483

  Current guidelines recommend functional imaging by using radiolabeled somatostatin analogs in order to localize primary tumors and detect sites of metastatic disease, for re-staging of patients with known disease, to monitor the effects of therapy, including surgery, radiotherapy, chemotherapy or somatostatin analog therapy. […] The combined evaluation of PET/CT imaging by 68Ga-DOTA-peptides and 18F-FDG in patients with GEP-NENs has been largely investigated. […] The presence of 18F-FDG uptake correlated with a higher risk of progression of disease. […] The absence of 18F-FDG uptake at baseline evaluation does not exclude the occurrence of positive scans during follow-up. […] The identification of lesions showing areas of match or mismatch at metabolic and receptorial imaging (flip-flop phenomenon) is a powerful tool in prognostic stratification of GEP-NENs patients.

• #43 Classification, clinical presentation, diagnosis, and staging of pancreatic neuroendocrine neoplasms – UpToDate

  https://www.uptodate.com/contents/classification-epidemiology-clinical-presentation-localization-and-staging-of-pancreatic-neuroendocrine-neoplasms

  Classification, clinical presentation, diagnosis, and staging of pancreatic neuroendocrine neoplasms […] The clinical presentation, diagnostic imaging studies, and staging of well-differentiated pancreatic NETs is presented here. […] Diagnostic approach […] Imaging studies […] Computed tomography […] Magnetic resonance imaging […] Endoscopic ultrasonography […] Somatostatin receptor-based imaging studies […] Arterial stimulation with venous sampling […] Intraoperative localization techniques […] Laboratory studies […] Functioning tumors […] Nonfunctioning tumors […] Pancreatic neuroendocrine tumors (NETs) are rare neoplasms that arise in the endocrine tissues of the pancreas. […] They can secrete various peptide hormones, including insulin, gastrin, glucagon, and vasoactive intestinal peptide, resulting in a myriad of clinical syndromes. […] Approximately 50 to 75 percent of pancreatic NETs are nonfunctioning (ie, unassociated with a hormonal syndrome).

• #44

  https://rasayely-journals.com/index.php/hpbci/article/view/55

  Selective Arterial Calcium Stimulation With Hepatic Venous Sampling Differentiates Insulinoma From Nesidioblastosis. […] Recent standardization of treatment strategy for pancreatic neuroendocrine tumors. […] Diagnostic imaging of gastrointestinal neuroendocrine neoplasms with a focus on ultrasound. […] Radiofrequency Ablation of Pancreatic Neuroendocrine Tumors: A Pilot Study of Feasibility, Efficacy, and Safety. […] Endoscopic ultrasound-guided radiofrequency ablation of pancreatic neuroendocrine tumors: a systematic review of the literature.

• #45 Diagnosed Differently From Other Tumors of The Pancreas – Pancreatic Cancer  |  Johns Hopkins Pathology

  https://pathology.jhu.edu/pancreas/neuroendocrine/diagnosis

  Examination of a biopsy or the entire tumor under the microscope is the gold standard for establishing the diagnosis of a neuroendocrine tumor. Neuroendocrine tumors usually have a distinct microscopic appearance, and this appearance can be used to not only determine that the tumor is a neuroendocrine tumor, but also the specific type of neuroendocrine tumor. […] Special stains, called immunohistochemical stains, can be used to demonstrate the production of hormones, such as insulin and glucagon, in tissue sections made from biopsies or resected tumors. […] For this reason, the accurate pathologic examination of neuroendocrine tumors is critical. […] Elevated levels of the hormones produced by neuroendocrine tumors can often be detected in the blood by special tests that can be run on blood samples taken from a patient.

• #46 Tests for Pancreatic Neuroendocrine Tumor | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/how-diagnosed.html

  Like CT scans, MRI scans show detailed images of soft tissues in the body. […] Ultrasound tests use sound waves to create images of organs such as the pancreas. […] These imaging tests look at the pancreatic ducts and bile ducts to see if they are blocked, narrowed, or dilated. […] Scans using small amounts of radioactivity and special cameras can be helpful looking for pNETs. […] Certain blood tests may help diagnose and check the response to treatment. […] In many cases, the only way to know for sure if a person has a pNET is to remove cells from the tumor and look at them in the lab. […] For this type of biopsy, a doctor inserts a thin, hollow needle through the skin over the abdomen and into the pancreas to remove a small piece of a tumor. […] Doctors can also biopsy a tumor during an endoscopy.

• #47 Tests for Pancreatic Neuroendocrine Tumor | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/how-diagnosed.html

  Like CT scans, MRI scans show detailed images of soft tissues in the body. […] Ultrasound tests use sound waves to create images of organs such as the pancreas. […] These imaging tests look at the pancreatic ducts and bile ducts to see if they are blocked, narrowed, or dilated. […] Scans using small amounts of radioactivity and special cameras can be helpful looking for pNETs. […] Certain blood tests may help diagnose and check the response to treatment. […] In many cases, the only way to know for sure if a person has a pNET is to remove cells from the tumor and look at them in the lab. […] For this type of biopsy, a doctor inserts a thin, hollow needle through the skin over the abdomen and into the pancreas to remove a small piece of a tumor. […] Doctors can also biopsy a tumor during an endoscopy.

• #48 Pancreatic Neuroendocrine Tumor: Types, Symptoms, Outlook & More

  https://www.healthline.com/health/pancreatic-islet-cell-tumor

  How is a pancreatic neuroendocrine tumor diagnosed? There are several ways your doctor may diagnosis a PNET. The first step is to complete an overall physical exam and a review of your medical and family history. The next step is to either run a blood test or perform an imaging technique to look for a tumor. Blood tests look for increased levels of hormones and glucose levels in the bloodstream. Increased levels can indicate a PNET. Imaging tests allow your doctor to visualize your pancreas and look for the presence of a tumor. These tests may include an ultrasound, an MRI, or a CT scan. Another way to get an image of the pancreas is to perform minor exploratory surgery. Your doctor may want to insert a small camera, or endoscope, into the abdomen to view your pancreas. They could also opt to take a small sample of the tissue for a biopsy.

• #49 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  Tumor biopsy is critical for PNET diagnosis, not only to demonstrate the neuroendocrine nature of the tumor but also to preliminarily grade the tumor and to perform immunocytochemical staining for hormones and islet markers, which is useful for determining the pancreatic origin of liver metastases. […] Currently, the best predictor of PNET behavior is tumor grade; therefore, the cytologic examination of the biopsied tumor sample should classify the tumor as a well-differentiated endocrine tumor (low grade of malignancy), a well-differentiated endocrine carcinoma (intermediate grade), or a poorly differentiated endocrine carcinoma (high grade).

• #50 Pancreatic neuroendocrine tumor – Wikipedia

  https://en.wikipedia.org/wiki/Pancreatic_neuroendocrine_tumor

  PanNETs are a type of neuroendocrine tumor, representing about one-third of gastroenteropancreatic neuroendocrine tumors (GEP-NETs). […] Because symptoms are non-specific, diagnosis is often delayed. […] Measurement of hormones including pancreatic polypeptide, gastrin, proinsulin, insulin, glucagon, and vasoactive intestinal peptide can determine if a tumor is causing hypersecretion. […] Multiphase CT and MRI are the primary modalities for morphologic imaging of PNETs. […] Notably, while many malignant lesions are hypodense in contrast-enhanced studies, the liver metastases of PNETs are hypervascular and readily visualized in the late arterial phase of the post-contrast CT study. […] However, morphological imaging alone is not sufficient for a definite diagnosis. […] On biopsy, immunohistochemistry is generally positive for chromogranin and synaptophysin.

• #51 Diagnostic work-up of gastroenteropancreatic neuroendocrine tumors | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-diagnostic-work-up-gastroenteropancreatic-neuroendocrine-tumors-S1807593222024048

  Neuroendocrine tumors are a heterogeneous group of malignancies that present a diagnostic challenge. The diagnosis is based on histopathology, imaging, and circulating biomarkers. The histopathology should contain specific neuroendocrine markers such as chromogranin A, synaptophysin, and neuron-specific enolase and also an estimate of the proliferation by Ki-67 (MIB-1). Standard imaging procedures consist of computed tomography or magnetic resonance imaging together with somatostatin receptor scintigraphy. 68Ga-DOTA-octreotate scans will in the future replace somatostatin receptor scintigraphy because they have higher specificity and sensitivity. Other positron imaging tomographic scanning tracers that will come into clinical use are 18F-DOPA and 11C-5HTP. […] The initial diagnostic approach in patients with NETs includes histological examination, which is always required before therapeutic decisions are made. Clinicians should also consider performing repetitive biopsies to reassess the prognosis if the disease course changes significantly. The following investigations are also required: (a) immunohistochemical markers and detailed histological analysis; (b) assessment of the primary tumor and the extent of extrahepatic spread by imaging, including patterns of hepatic metastases; and (c) biochemical assessment of functionality and general tumor markers.

• #52

  https://journals.lww.com/jpancreatology/fulltext/2024/06000/consensus_on_the_clinical_diagnosis_and_treatment.1.aspx

  The World Health Organization (WHO) 2017 classifications for neuroendocrine neoplasms (NENs) subdivided grade 3 pancreatic neuroendocrine neoplasms (pNENs) into G3 well-differentiated pancreatic neuroendocrine tumors (G3 pNETs) and poorly differentiated pancreatic neuroendocrine carcinomas (pNECs), according to the mitotic count, Ki-67 index, and cell differentiation. […] Therefore, in the WHO classification proposed in 2017 and 2019, patients with Ki-67 20% and well-differentiated morphology were defined as G3 pNETs, separate from pNECs. As a new type of pNET, the diagnosis and treatment of G3-grade pNETs remain challenging. […] The pathological diagnosis of G3 NET is clearly introduced in the Chinese Pathological Diagnosis Consensus on Gastrointestinal and Pancreatic Neuroendocrine Tumors (2020).

• #53 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  The biochemical diagnostics of gastrinoma is discussed in the second part of the recommendations in the article Gastroduodenal neuroendocrine neoplasms including gastrinoma update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours). […] The minimal histopathological report regarding PanNENs should include: histological type of the tumour divided into WD-NET, large or small cell NEC, or MiNEN; grade of histological maturity (G) relating to highly differentiated neoplasms (PanNET G1, PanNET G2, PanNET G3) and NEC; pTNM stage according to the 8th edition of AJCC/UICC pTNM classification of 2017 (it is important to provide the affiliation of the classification in each case); assessment of surgical margins. […] The presence of multiple ulcers of atypical location (distal duodenum or jejunum) during the examination of the upper gastrointestinal tract, the coexistence of severe oesophagitis, and recurrence of ulcers after pharmacological or surgical treatment suggest that diagnostics towards gastrinoma is advisable.

• #54 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5719919/

  Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. […] For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. Chromogranin A is the most sensitive marker but has insufficient specificity. In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. […] Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. Positron emission tomography (PET) scan with 18F-fluorodeoxyglucose cannot visualize PNETs, but PET with 68-Ga DOTATATE can. Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy.

• #55 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5719919/

  PNETs are divided into functional and non-functional tumors. About 90% of PNETs are classified as non-functional tumors, and the remaining 10% as functional tumors. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs. Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 23 cm. Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs.

• #56 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5719919/

  PNETs are divided into functional and non-functional tumors. About 90% of PNETs are classified as non-functional tumors, and the remaining 10% as functional tumors. […] A detailed history taking of symptoms caused by mass effect and hormone overproduction is essential for the diagnosis of PNETs. Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 23 cm. Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs.

• #57

  https://journals.lww.com/jpancreatology/fulltext/2024/06000/consensus_on_the_clinical_diagnosis_and_treatment.1.aspx

  The World Health Organization (WHO) 2017 classifications for neuroendocrine neoplasms (NENs) subdivided grade 3 pancreatic neuroendocrine neoplasms (pNENs) into G3 well-differentiated pancreatic neuroendocrine tumors (G3 pNETs) and poorly differentiated pancreatic neuroendocrine carcinomas (pNECs), according to the mitotic count, Ki-67 index, and cell differentiation. […] Therefore, in the WHO classification proposed in 2017 and 2019, patients with Ki-67 20% and well-differentiated morphology were defined as G3 pNETs, separate from pNECs. As a new type of pNET, the diagnosis and treatment of G3-grade pNETs remain challenging. […] The pathological diagnosis of G3 NET is clearly introduced in the Chinese Pathological Diagnosis Consensus on Gastrointestinal and Pancreatic Neuroendocrine Tumors (2020).

• #58 Diagnostic work-up of gastroenteropancreatic neuroendocrine tumors | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-diagnostic-work-up-gastroenteropancreatic-neuroendocrine-tumors-S1807593222024048

  Immunohistochemical determination of CgA and synaptophysin as well as proliferation marker Ki-67 (MIB-1) is mandatory. […] Staging is performed with the familiar tumor node metastasis (TNM) approach according to the anatomical location of the tumors, and this approach is recommended by the WHO, the American Joint Committee on Cancer and the International Union Against Cancer. […] A standard computed tomographic (CT) scan of the chest, abdomen, and pelvis or magnetic resonance image is mandatory, and should be complemented by somatostatin receptor scintigraphy including single photon emission computer tomography (SPECT)-SRS and triphasic CT. […] The minimal biochemical work-up for NETs includes circulating chromogranin A and assessment of a specific marker to assess functionality, such as urinary 5-HIAA evaluation in carcinoid syndrome.

• #59 Grades, types and stages of pancreatic neuroendocrine cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/grades-types-stages

  A specialist doctor (pathologist) looks at the cancer cells under a microscope. This tells them: […] Your doctor uses the differentiation to diagnose you with one of the following: pancreatic neuroendocrine tumour (pancreatic NET) these are well differentiated cancers […] pancreatic neuroendocrine carcinoma (pancreatic NEC) these are poorly differentiated cancers. […] The stage of a cancer tells you about its size and whether it has spread. Knowing the stage can help your doctor decide which treatment you need. […] You have tests and scans which give some information about the stage of the cancer. Sometimes it’s not possible to be certain about the stage until after surgery. […] There are different ways to stage pancreatic neuroendocrine cancer. Doctors usually use the TNM system or number system.

• #60

  https://www.xiahepublishing.com/2771-165X/JCTP-2022-00005

  Raising the awareness of the characteristic morphology of individual variants is the most effective approach to prevent a potential misdiagnosis. The present review discussed examples of selective PanNET morphologic variants, with focus on the specific cytomorphology and corresponding differential diagnosis. […] The most important differential diagnosis of this variant is clear cell renal cell carcinoma (CCRCC), particularly because patients with VHL have an increased risk for development of CCRCC, and because CCRCC is the most common metastatic malignancy in the pancreas. This variant should also be distinguished from ectopic adrenal tissues in the pancreas or adrenocortical neoplasm, among others. Due to the significant cytomorphologic overlapping among the three entities, the combination of immunohistochemical stains would be crucial for making the distinctions. Typically, PanNETs are immunoreactive for cytokeratins and neuroendocrine markers. […] Recognizing the cytomorphologic and immunophenotypic features of these variants in FNA specimens is critically important to make a correct diagnosis, preventing diagnostic pitfalls, and guiding the appropriate clinical management.

• #61

  https://www.xiahepublishing.com/2771-165X/JCTP-2022-00005

  Morphologic variants of well-differentiated pancreatic neuroendocrine tumors (PanNETs) are uncommon. These variants may mimic non-PanNETs, and when under recognized, it would lead to misdiagnosis by fine-needle aspiration (FNA) biopsy. The present report describes the unique cytomorphologic features and diagnostic clues of pigmented, pleomorphic, clear cell/lipid-rich and oncocytic variants of well-differentiated PanNETs. The differential diagnoses of each morphologic variant are also discussed. Ancillary immunohistochemical studies with appropriate markers are crucial in the diagnostic work-up. Raising the awareness of PanNET morphologic variants is essential for preventing diagnostic pitfalls, and rendering an accurate diagnosis during the FNA diagnostic work-up of pancreatic lesions. […] The preferred preoperative approach for the diagnosis of PanNET is endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). Well-differentiated PanNETs have unique cytomorphologic features. The classic features include single and dyscohesive clusters of relatively uniform tumor cells with eosinophilic cytoplasm, eccentrically located nuclei (plasmacytoid appearance), and speckled (salt and pepper) chromatin. With the recognition of these cytomorphologic features, the application of confirmatory immunohistochemistry studies with neuroendocrine markers has resulted in accurate cytological diagnoses.

• #62 Pancreatic neuroendocrine tumours: spectrum of imaging findings – Bicci – Gland Surgery

  https://gs.amegroups.org/article/view/52017/html

  Non-functional pNETs are usually malignant (90%), approximately 50-60% of gastrinomas show these characteristics, whereas 90% of insulinomas are benign. They can generate metastases above all in lymph nodes, bones and liver. The differential diagnosis with pNETs, involve some other lesions. It has to be remembered also that accessory spleens are commonly located in the pancreatic tail and with their high enhancement it can be difficult to distinguish them from pNETs. […] pNETs are rare tumours but their incidence is growing because of our better radiological detection especially through CT and MRI. The management of patients with pNET is multidisciplinary and often multimodal: this underlines the importance of the radiologists as a fundamental figure that must be aware of the typical/atypical imaging findings or mimics of pNETs, according to up-to-date literature, in order to facilitate the initial diagnosis and to help in the management in patients with such pancreatic lesions.

• #63 Pancreatic Neuroendocrine Tumors | Types and Treatment | University of Michigan Rogel Cancer Center

  https://www.rogelcancercenter.org/endocrine-cancer/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs) account for less than 5% of all pancreatic tumors. […] The Multidisciplinary Endocrine Tumor Program at the University of Michigan is composed of endocrinologists, endocrine surgeons, radiologists, nuclear medicine specialists and oncologists who specialize in the diagnosis and treatment of these rare pancreatic endocrine tumors. […] Several tests are required to confirm the diagnosis of an insulinoma because there are several other reasons for low blood sugar. Once the diagnosis is confirmed with blood tests, imaging studies such as CT scan or ultrasound are used to localize the tumor. […] After the diagnosis of gastrinoma is confirmed with blood tests, imaging studies including CT scan and ultrasound can help to localize the tumor prior to surgical removal.

• #64 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  Treatment of PanNENs requires a multidisciplinary team of specialists in the field of neuroendocrine neoplasms. […] It includes blood tests for the hormonal biochemical markers secreted by PanNENs. It is used in differential diagnosis, assessment of treatment effectiveness, and prognosis. […] The characteristics of biochemical biomarkers specific and non-specific for PanNENs are included in the first part of the recommendations. […] The diagnosis of insulinoma is facilitated by identification of the Whipple triad: clinical symptoms suggestive of hypoglycaemia after fasting; symptoms accompanied by hypoglycaemia; relief of symptoms soon after taking simple carbohydrates. […] The gold standard in the diagnosis of insulinoma is still the 72-hour fasting test, although some studies suggest that the 48-hour test may be sufficient.

• #65 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  Treatment of PanNENs requires a multidisciplinary team of specialists in the field of neuroendocrine neoplasms. […] It includes blood tests for the hormonal biochemical markers secreted by PanNENs. It is used in differential diagnosis, assessment of treatment effectiveness, and prognosis. […] The characteristics of biochemical biomarkers specific and non-specific for PanNENs are included in the first part of the recommendations. […] The diagnosis of insulinoma is facilitated by identification of the Whipple triad: clinical symptoms suggestive of hypoglycaemia after fasting; symptoms accompanied by hypoglycaemia; relief of symptoms soon after taking simple carbohydrates. […] The gold standard in the diagnosis of insulinoma is still the 72-hour fasting test, although some studies suggest that the 48-hour test may be sufficient.

• #66 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  Pancreatic neuroendocrine neoplasms (pNENs) and carcinoid tumors develop from the islets of Langerhans in the pancreas and enterochromaffin cells in the gastrointestinal tract, respectively. […] The incidence of GEP-NENs has expanded over the past few decades, likely due to increased emphasis on screening and widespread use of cross-sectional imaging with computed tomography (CT) and magnetic resonance imaging (MRI). […] Also, recent technological advancements in endoscopy have facilitated the early diagnosis of neuroendocrine neoplasms. […] In this review, we will summarize the clinical presentations and diagnostic criteria of each functional GEP-NEN. […] The most dependable test to diagnose insulinomas is the measurement of serum glucose, proinsulin, and C-peptide levels after an overnight fast and every 4 hours for up to 72 hours.

• #67 Pancreatic Neuroendocrine Tumors | Types and Treatment | University of Michigan Rogel Cancer Center

  https://www.rogelcancercenter.org/endocrine-cancer/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs) account for less than 5% of all pancreatic tumors. […] The Multidisciplinary Endocrine Tumor Program at the University of Michigan is composed of endocrinologists, endocrine surgeons, radiologists, nuclear medicine specialists and oncologists who specialize in the diagnosis and treatment of these rare pancreatic endocrine tumors. […] Several tests are required to confirm the diagnosis of an insulinoma because there are several other reasons for low blood sugar. Once the diagnosis is confirmed with blood tests, imaging studies such as CT scan or ultrasound are used to localize the tumor. […] After the diagnosis of gastrinoma is confirmed with blood tests, imaging studies including CT scan and ultrasound can help to localize the tumor prior to surgical removal.

• #68 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  Gastrinomas can be diagnosed by measuring the gastric pH and fasting serum gastrin (FSG) levels. […] To diagnose a VIPoma, patients must present with secretory diarrhea and plasma VIPs 500 pg/mL. […] Clinical documentation of necrolytic migratory erythema with a fasting plasma glucagon value 500 pg/mL can confirm the diagnosis. […] Somatostatinomas are usually diagnosed after immunohistochemical staining for somatostatin. […] In patients with suspected carcinoid syndrome, measuring urinary 5-Hydroxyindoleacetic acid (5HIAA) for 24 hours (the normal value for urinary 5-HIAA ranges 3-15 mg/day) is the recommended initial test, with a sensitivity and specificity of 73% and 100%, respectively. […] Among many imaging modalities used to screen for early diagnosis of pNENs in MEN-1 syndrome patients, Endoscopic Ultrasound (EUS) outperformed CT, MRI, and somatostatin receptor scintigraphy (SRS).

• #69 Pancreatic Neuroendocrine Tumors | Types and Treatment | University of Michigan Rogel Cancer Center

  https://www.rogelcancercenter.org/endocrine-cancer/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs) account for less than 5% of all pancreatic tumors. […] The Multidisciplinary Endocrine Tumor Program at the University of Michigan is composed of endocrinologists, endocrine surgeons, radiologists, nuclear medicine specialists and oncologists who specialize in the diagnosis and treatment of these rare pancreatic endocrine tumors. […] Several tests are required to confirm the diagnosis of an insulinoma because there are several other reasons for low blood sugar. Once the diagnosis is confirmed with blood tests, imaging studies such as CT scan or ultrasound are used to localize the tumor. […] After the diagnosis of gastrinoma is confirmed with blood tests, imaging studies including CT scan and ultrasound can help to localize the tumor prior to surgical removal.

• #70 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  The biochemical diagnostics of gastrinoma is discussed in the second part of the recommendations in the article Gastroduodenal neuroendocrine neoplasms including gastrinoma update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours). […] The minimal histopathological report regarding PanNENs should include: histological type of the tumour divided into WD-NET, large or small cell NEC, or MiNEN; grade of histological maturity (G) relating to highly differentiated neoplasms (PanNET G1, PanNET G2, PanNET G3) and NEC; pTNM stage according to the 8th edition of AJCC/UICC pTNM classification of 2017 (it is important to provide the affiliation of the classification in each case); assessment of surgical margins. […] The presence of multiple ulcers of atypical location (distal duodenum or jejunum) during the examination of the upper gastrointestinal tract, the coexistence of severe oesophagitis, and recurrence of ulcers after pharmacological or surgical treatment suggest that diagnostics towards gastrinoma is advisable.

• #71 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  Gastrinomas can be diagnosed by measuring the gastric pH and fasting serum gastrin (FSG) levels. […] To diagnose a VIPoma, patients must present with secretory diarrhea and plasma VIPs 500 pg/mL. […] Clinical documentation of necrolytic migratory erythema with a fasting plasma glucagon value 500 pg/mL can confirm the diagnosis. […] Somatostatinomas are usually diagnosed after immunohistochemical staining for somatostatin. […] In patients with suspected carcinoid syndrome, measuring urinary 5-Hydroxyindoleacetic acid (5HIAA) for 24 hours (the normal value for urinary 5-HIAA ranges 3-15 mg/day) is the recommended initial test, with a sensitivity and specificity of 73% and 100%, respectively. […] Among many imaging modalities used to screen for early diagnosis of pNENs in MEN-1 syndrome patients, Endoscopic Ultrasound (EUS) outperformed CT, MRI, and somatostatin receptor scintigraphy (SRS).

• #72 Pancreatic Neuroendocrine Tumors : Diagnosis and Management with an Emphasis on Endoscopy – Practical Gastro

  https://practicalgastro.com/2023/01/26/pancreatic-neuroendocrine-tumors-diagnosis-and-management-with-an-emphasis-on-endoscopy/

  Gastrinomas can be diagnosed by measuring the gastric pH and fasting serum gastrin (FSG) levels. […] To diagnose a VIPoma, patients must present with secretory diarrhea and plasma VIPs 500 pg/mL. […] Clinical documentation of necrolytic migratory erythema with a fasting plasma glucagon value 500 pg/mL can confirm the diagnosis. […] Somatostatinomas are usually diagnosed after immunohistochemical staining for somatostatin. […] In patients with suspected carcinoid syndrome, measuring urinary 5-Hydroxyindoleacetic acid (5HIAA) for 24 hours (the normal value for urinary 5-HIAA ranges 3-15 mg/day) is the recommended initial test, with a sensitivity and specificity of 73% and 100%, respectively. […] Among many imaging modalities used to screen for early diagnosis of pNENs in MEN-1 syndrome patients, Endoscopic Ultrasound (EUS) outperformed CT, MRI, and somatostatin receptor scintigraphy (SRS).

• #73 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  In this paper, we present the current guidelines for the diagnostics and management of pancreatic neuroendocrine neoplasms (PanNENs) developed by Polish experts providing care for these patients in everyday clinical practice. […] In oncological diagnostics, in addition to biochemical tests, molecular identification with the use of NETest liquid biopsy and circulating microRNAs is gaining importance. Both anatomical and functional examinations (including new radiopharmaceuticals) are used in imaging diagnostics. Histopathological diagnosis along with immunohistochemical examination still constitute the basis for therapeutic decisions. Whenever possible, surgical procedure is the treatment of choice. Pharmacological management including biotherapy, radioisotope therapy, targeted molecular therapy, and chemotherapy are important methods of systemic therapy.

• #74 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  In this paper, we present the current guidelines for the diagnostics and management of pancreatic neuroendocrine neoplasms (PanNENs) developed by Polish experts providing care for these patients in everyday clinical practice. […] In oncological diagnostics, in addition to biochemical tests, molecular identification with the use of NETest liquid biopsy and circulating microRNAs is gaining importance. Both anatomical and functional examinations (including new radiopharmaceuticals) are used in imaging diagnostics. Histopathological diagnosis along with immunohistochemical examination still constitute the basis for therapeutic decisions. Whenever possible, surgical procedure is the treatment of choice. Pharmacological management including biotherapy, radioisotope therapy, targeted molecular therapy, and chemotherapy are important methods of systemic therapy.

• #75

  https://rasayely-journals.com/index.php/hpbci/article/view/55

  For unresectable or metastatic disease, locoregional therapy and systemic treatment are options. […] In this review, we discuss the clinical presentation, diagnosis, and potential management options of pNETs. […] Low accuracy of chromogranin A for diagnosing early-stage pancreatic neuroendocrine tumors. […] The NETest: The Clinical Utility of Multigene Blood Analysis in the Diagnosis and Management of Neuroendocrine Tumors. […] The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Medical Management of Pancreatic Neuroendocrine Tumors. […] The role of endoscopy in the evaluation and management of patients with solid pancreatic neoplasia. […] Incremental benefit of preoperative EUS for the detection of pancreatic neuroendocrine tumors: a meta-analysis.

• #76 Pancreatic neuroendocrine tumours: spectrum of imaging findings – Bicci – Gland Surgery

  https://gs.amegroups.org/article/view/52017/html

  With the advent of Radiomics, the new method in which the information is hidden inside the radiological images and can be extracted using advanced texture and shape analysis, it has been possible to extract lots of information regarding the structure we need to study. More information in the characterization of these different grades can be obtained with MRI imaging. The pancreas has a hyperintense signal on T1-weighted imaging, because of the amount of protein inside the parenchyma, therefore pNETs tend to appear hypointense in contrast with pancreatic parenchyma on un-enhanced T1-weighted images. On T2-weighted images, pNETs can be hyperintense, even if a lower signal can also be present. Diffusion weighted imaging (DWI) may also help in tumour detection. […] pNETs can be hyperfunctioning, with the production of a lot of different hormones such as insulin, glucagon, gastrin, vasoactive intestinal peptide (VIP) and somatostatin, that can create various clinical manifestation. This subtype of tumours tends to present earlier, with clinical signs and symptoms. At CT examination, these lesions are isodense at baseline, homogeneously hyperdense in the pancreatic phase, and hypodense in the venous one because of their washout phenomena. In order to distinguish between typical and atypical insulinomas, multiphase imaging is essential. MRI shows a homogeneously enhanced lesion with low signal on T1-weighted imaging and high signal on T2-weighted one.

• #77 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  In this paper, we present the current guidelines for the diagnostics and management of pancreatic neuroendocrine neoplasms (PanNENs) developed by Polish experts providing care for these patients in everyday clinical practice. […] In oncological diagnostics, in addition to biochemical tests, molecular identification with the use of NETest liquid biopsy and circulating microRNAs is gaining importance. Both anatomical and functional examinations (including new radiopharmaceuticals) are used in imaging diagnostics. Histopathological diagnosis along with immunohistochemical examination still constitute the basis for therapeutic decisions. Whenever possible, surgical procedure is the treatment of choice. Pharmacological management including biotherapy, radioisotope therapy, targeted molecular therapy, and chemotherapy are important methods of systemic therapy.

• #78 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. […] MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 2-3 cm. […] Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs. […] Contrast-enhanced EUS is helpful in categorizing small hypervascular PNETs, which are incidentally found on other imaging modalities. […] Early detection through advanced imaging modalities and proper classification and staging with the recently updated classification system are important in providing prognostic information and decision for the management of PNETs.

• #79 Pancreatic Neuroendocrine Tumors: State-of-the-Art Diagnosis and Management

  https://www.cancernetwork.com/view/pancreatic-neuroendocrine-tumors-state-art-diagnosis-and-management

  The incidence of pancreatic neuroendocrine tumors (PNETs) has significantly increased in recent years, although this may reflect better detection and improved diagnosis, in addition to a true increase in incidence. […] The workup of an incidental pancreatic mass or of symptoms consistent with a PNET is focused on arriving at a diagnosis, determining functionality, and delineating extent of disease. […] In the workup of PNETs, cross-sectional imaging plays a major role in characterizing the primary tumor and determining the extent of disease. The sensitivity of CT and MRI for the detection of PNETs exceeds 80%, and both modalities are more sensitive than an octreotide-based scintigraphic study. […] EUS is an invaluable adjunct to CT or MRI and has superior resolution. The sensitivity of EUS alone may exceed 90%, with tumor resolution as small as 2 mm; its sensitivity approaches 100% when combined with cross-sectional imaging.

• #80 Pancreatic Neuroendocrine Tumors: State-of-the-Art Diagnosis and Management

  https://www.cancernetwork.com/view/pancreatic-neuroendocrine-tumors-state-art-diagnosis-and-management

  Functional imaging in NETs takes advantage of high levels of somatostatin receptor 2 (SSTR2) expression. […] In NETs, tumor markers are useful for diagnosis, but they often play a more important role in prognostication, assessment of response to treatment, and detection of recurrence. […] CT and MRI, in combination with tumor markers, are the preferred modalities for surveillance and monitoring progression. […] Multiple classification and grading systems have been proposed over the last few decades in an effort to better categorize NETs according to their clinical behavior; these have emphasized grade and differentiation. […] The only known cure for functional or nonfunctional PNETs is surgical resection. […] Surgical resection is the gold standard for all locoregional PNETs 2 cm.

• #81 Pancreatic Neuroendocrine Tumors | Types and Treatment | University of Michigan Rogel Cancer Center

  https://www.rogelcancercenter.org/endocrine-cancer/pancreatic-neuroendocrine-tumors

  Surgical removal of the tumor is the primary treatment after imaging studies are completed. […] Blood tests and imaging studies are needed to confirm the diagnosis. […] The challenge in the diagnosis of these types of tumors is to distinguish them from pancreatic adenocarcinoma which has a worse prognosis. […] Surgery is the primary treatment for non-functional neuroendocrine tumors after blood tests and imaging studies are complete. […] The treatment of pancreatic neuroendocrine tumors requires a multidisciplinary approach with medical, surgical, oncologic and radiological treatments. The Multidisciplinary Endocrine Tumor Program at the University of Michigan is composed of endocrinologists, endocrine surgeons, radiologists, nuclear medicine specialists and oncologists who specialize in all aspects of diagnosis and treatment of pancreatic endocrine tumors.

• #82 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  EUS despite the fact that compared to computed tomography and magnetic resonance imaging (MRI) it is much more dependent on the operators experience is currently considered to be the most sensitive method of detecting pancreatic neuroendocrine tumours, especially small ones with diameter 2 cm (even 12 mm). […] The sensitivity of EUS is 94100% in small insulin-secreting tumours. […] The role of surgical treatment in patients diagnosed with ZES but having negative results of preoperative imaging examinations (lack of tumour identification and location) remains debatable. […] The method of surgical treatment of PanNETs depends on the degree of tumour malignancy, its size, location, infiltration of adjacent organs, the presence of distant metastases, the patients general condition, and the ability to control clinical symptoms. […] The decision regarding PanNET treatment method should be made by a multidisciplinary team of physicians experienced in the treatment of neuroendocrine tumours.

• #83 Pancreatic neuroendocrine tumor – Wikipedia

  https://en.wikipedia.org/wiki/Pancreatic_neuroendocrine_tumor

  PanNETs are a type of neuroendocrine tumor, representing about one-third of gastroenteropancreatic neuroendocrine tumors (GEP-NETs). […] Because symptoms are non-specific, diagnosis is often delayed. […] Measurement of hormones including pancreatic polypeptide, gastrin, proinsulin, insulin, glucagon, and vasoactive intestinal peptide can determine if a tumor is causing hypersecretion. […] Multiphase CT and MRI are the primary modalities for morphologic imaging of PNETs. […] Notably, while many malignant lesions are hypodense in contrast-enhanced studies, the liver metastases of PNETs are hypervascular and readily visualized in the late arterial phase of the post-contrast CT study. […] However, morphological imaging alone is not sufficient for a definite diagnosis. […] On biopsy, immunohistochemistry is generally positive for chromogranin and synaptophysin.

• #84 Pancreatic Neuroendocrine Cancer vs. Pancreatic Cancer – NETRF

  https://netrf.org/2018/11/07/pancreatic-neuroendocrine-cancer-vs-pancreatic-cancer/

  What is the difference between a pancreatic neuroendocrine tumor and a pancreatic tumor? […] Though they occur in the same organ, these are two different types of cancers. Pancreatic adenocarcinoma and neuroendocrine cancers have different: […] Tests […] Pancreatic neuroendocrine cancer is sometimes called islet cell carcinoma. […] Neuroendocrine tumors arise from endocrine cells in the pancreas, which cluster together like an island and are called islet cells. […] One in two neuroendocrine tumor patients is misdiagnosed. On average, people have symptoms for five years before learning they have a neuroendocrine tumor. […] Given delays in diagnosis, rates of metastases, and misdiagnosis, those affected by NETs advocate for increased education and awareness. […] Advocates for improvements in NET diagnosis and treatment stress the importance of referring to the cancer type, not site.

• #85

  https://journals.lww.com/jpancreatology/fulltext/2024/06000/consensus_on_the_clinical_diagnosis_and_treatment.1.aspx

  The consensus also recommends that when pathologically diagnosing G3 pNETs, if tumors with G1/G2 components are present simultaneously, the percentage of tumors of different grades should be reported, to facilitate subsequent clinical individualized treatment. […] IHC staining of characteristic biomarkers can help distinguish G3 pNETs from pNECs. […] The consensus recommends a second biopsy when pathological diagnosis is not clear, if possible. […] Molecular imaging plays an important role in the diagnosis and treatment choice of G3 pNETs. It is recommended to perform dual-tracer scans of SSTR-PET and FDG-PET during the initial diagnosis of G3 pNETs. […] The choice of treatment should be based on the hormone secretion status, SSTR expression, Ki-67 index, tumor burden, tumor growth rate, tumor blood supply, and patients general condition.

• #86 Pancreatic Neuroendocrine Cancer vs. Pancreatic Cancer – NETRF

  https://netrf.org/2018/11/07/pancreatic-neuroendocrine-cancer-vs-pancreatic-cancer/

  What is the difference between a pancreatic neuroendocrine tumor and a pancreatic tumor? […] Though they occur in the same organ, these are two different types of cancers. Pancreatic adenocarcinoma and neuroendocrine cancers have different: […] Tests […] Pancreatic neuroendocrine cancer is sometimes called islet cell carcinoma. […] Neuroendocrine tumors arise from endocrine cells in the pancreas, which cluster together like an island and are called islet cells. […] One in two neuroendocrine tumor patients is misdiagnosed. On average, people have symptoms for five years before learning they have a neuroendocrine tumor. […] Given delays in diagnosis, rates of metastases, and misdiagnosis, those affected by NETs advocate for increased education and awareness. […] Advocates for improvements in NET diagnosis and treatment stress the importance of referring to the cancer type, not site.

• #87 Diagnosis of Pancreatic Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7055

  Laboratory tests for functional PNETs include hormone levels for specific symptoms and tumor markers, such as CgA and NSE. […] For localization and staging of PNETs, multiphasic contrast-enhanced CT is considered the imaging study of choice. […] MRI has been suggested to be more sensitive in detecting small tumors and liver metastasis than other modalities. […] Somatostatin receptor scintigraphy is often used when functional PNETs, except insulinomas, are suspected clinically but tumors are not localized on cross-sectional images. […] EUS provides high-resolution ultrasonic images of the pancreas and can detect small tumors less than 2-3 cm. […] Its sensitivity is equal to or superior to multidetector CT or MRI for the detection of PNETs. […] Contrast-enhanced EUS is helpful in categorizing small hypervascular PNETs, which are incidentally found on other imaging modalities. […] Early detection through advanced imaging modalities and proper classification and staging with the recently updated classification system are important in providing prognostic information and decision for the management of PNETs.

• #88 Diagnosis, Management and Theragnostic Approach of Gastro-Entero-Pancreatic Neuroendocrine Neoplasms

  https://www.mdpi.com/2072-6694/15/13/3483

  The PRRT protocols consist on the systemic administration of a radiopharmaceutical composed of a β-emitting radionuclide, chelated to a specific somatostatin analog. […] In GEP-NENs patients with high SSTR expression and advanced disease, PRRT has been demonstrated to be an effective systemic treatment. […] The efficacy and safety of 177Lu-DOTATATE were first evaluated by Kwekkeboom et al. in 310 and 504 patients, respectively. […] The NETTER-1 trial confirmed the good tolerability of the treatment; grade 3 or 4 neutropenia, thrombocytopenia and lymphopenia occurred in 1%, 2% and 9% of the PRRT patients, respectively. […] Recent advances in functional imaging and radiometabolic therapy improved diagnosis and management of patients with GEP-NENs.

• #89 Pancreatic neuroendocrine neoplasms — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki — uaktualnione zasady diagnostyki i leczenia (rekomendowane przez P

  https://journals.viamedica.pl/endokrynologia_polska/article/view/EP.a2022.0050

  EUS despite the fact that compared to computed tomography and magnetic resonance imaging (MRI) it is much more dependent on the operators experience is currently considered to be the most sensitive method of detecting pancreatic neuroendocrine tumours, especially small ones with diameter 2 cm (even 12 mm). […] The sensitivity of EUS is 94100% in small insulin-secreting tumours. […] The role of surgical treatment in patients diagnosed with ZES but having negative results of preoperative imaging examinations (lack of tumour identification and location) remains debatable. […] The method of surgical treatment of PanNETs depends on the degree of tumour malignancy, its size, location, infiltration of adjacent organs, the presence of distant metastases, the patients general condition, and the ability to control clinical symptoms. […] The decision regarding PanNET treatment method should be made by a multidisciplinary team of physicians experienced in the treatment of neuroendocrine tumours.

• #90 Pancreatic Neuroendocrine Tumors | Types and Treatment | University of Michigan Rogel Cancer Center

  https://www.rogelcancercenter.org/endocrine-cancer/pancreatic-neuroendocrine-tumors

  Surgical removal of the tumor is the primary treatment after imaging studies are completed. […] Blood tests and imaging studies are needed to confirm the diagnosis. […] The challenge in the diagnosis of these types of tumors is to distinguish them from pancreatic adenocarcinoma which has a worse prognosis. […] Surgery is the primary treatment for non-functional neuroendocrine tumors after blood tests and imaging studies are complete. […] The treatment of pancreatic neuroendocrine tumors requires a multidisciplinary approach with medical, surgical, oncologic and radiological treatments. The Multidisciplinary Endocrine Tumor Program at the University of Michigan is composed of endocrinologists, endocrine surgeons, radiologists, nuclear medicine specialists and oncologists who specialize in all aspects of diagnosis and treatment of pancreatic endocrine tumors.

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