Materiały źródłowe

• #1 Key Statistics for Pancreatic Neuroendocrine Tumor | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/about/key-statistics.html

  Pancreatic neuroendocrine tumors (pNETs) are rare. Less than 2% of all cancers found in the pancreas each year are pNETs. […] The number of pNETs diagnosed each year; however, has been rising over time. This is thought to be partly because they are being found more often incidentally (by accident), when imaging tests such as CT or MRI scans of the abdomen are done for other reasons. […] Most people with pNETs are older, with the average age at diagnosis being 60.

• #2 Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8118193/

  The purpose of our review is to explore global epidemiologic trends of gastroenteropancreatic (GEP) neuroendocrine tumors (NETs). Specifically, we sought to examine whether there are differences in incidence, prevalence, distribution (by primary tumor site, tumor grade, tumor stage at presentation), and overall survival of GEP NETs between different regions of the world. […] GEP NET incidence rates are rising steadily in North America, Asia, and Europe, though this rise appears to be most profound in North America. […] The incidence and prevalence of NETs continues to rise globally, with the greatest rates of increase in nations such as the USA, Canada, and Norway. […] In the USA alone, the incidence of NETs has increased more than 6-fold over the last 4 decades, with a predominant rise in localized tumors rather than metastatic tumors; current disease prevalence of NETs in the USA approximates 170,000 patients.

• #3 Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8118193/

  GEP NETs represent the most common NET subtype, comprising 55-70% of all NETs. […] The largest retrospective population-based study querying the SEER database identified 64,971 NET patients between 1973 and 2012. Among primary tumor sites, GEP NETs demonstrated the highest incidence rate, with 3.56 cases per 100,000. […] The incidence rates of GEP NECs increased consistently from 1.5 cases per 1,000,000 in 1973 to 4.6 cases per 1,000,000 in 2012. […] The presence of metastatic disease at diagnosis ranged from 40 to 76%, with pancreatic NECs most likely to present with metastatic disease. […] The maximum incidence rate was observed in patients 70, where incidence rates reached 16-17 cases per 100,000. […] The observed 5-year overall survival (OS) rates for rectal, appendiceal, small intestinal, gastric, colon, and pancreatic NETs were 88.5%, 81.3%, 68.1%, 64.1%, 54.6%, and 37.6%, respectively.

• #4 Recent epidemiology of patients with gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NEN) in Japan: a population-based study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07581-y

  The worldwide prevalence and incidence of neuroendocrine neoplasms (NEN) have been increasing recently, although few studies have analyzed data on the current situation of NENs in Japan. […] A total of 6735 individuals were diagnosed with GEP-NEN in Japan in 2016. Annual onset incidence was 0.70/100,000 for pancreatic NEN and 2.84/100,000 for gastrointestinal NEN. […] In Japan, only two studies have reported on the incidence of NEN. Ito et al. estimated annual incidences in 2005 of 1.01 per 100,000 population for pancreatic NEN (PanNEN), and 2.10 per 100,000 for gastrointestinal NEN (GI-NEN) in Japan. […] In 2016, the Cancer Registry Promotion Act took effect which required all hospitals in Japan to submit the basic data of newly encountered cancer patients to the national cancer registry.

• #5 Recent epidemiology of patients with gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NEN) in Japan: a population-based study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07581-y

  Based on data derived from the NCR2016, the total number of patients treated for GEP-NENs in 2016 was recorded as 6735 and the age-adjusted overall incidence was 3.53 per 100,000 population. […] An apparent male predominance tended to be seen in the incidence of NENs, especially in the upper GI. The overall incidence of NENs among males was 2.09 per 100,000 population and that in females was 1.39 per 100,000 population. […] The disease was incidentally diagnosed in 51.8% of patients with no symptoms on a visit to the hospital for a health checkup or by chance. […] This study found that the overall incidence of GEP-NENs in Japan was 3.53 per 100,000 population in 2016. […] The current analysis revealed that 20.4% of PanNENs and 26.6% of GI-NENs were NEC, including MANEC, suggesting that aggressive phenotypes are more prevalent than expected.

• #6 An updated analysis of the epidemiologic trends of neuroendocrine tumors in Taiwan | Scientific Reports

  https://www.nature.com/articles/s41598-021-86839-2

  The incidence of neuroendocrine tumors (NETs) has been increasing in recent decades. […] The incidence of NETs was 0.244 per 100,000 in 1996 and increased to 3.162 per 100,000 in 2015. […] The most common site of NETs was rectum (29.65%), followed by lung/bronchus (17.22%), and pancreas (10.71%). […] The survival of pancreatic NET has shown a recent improvement. […] Among the common sites of NETs, an improved survival of pancreatic NETs diagnosed between 2010 and 2015 compared to those diagnosed between 2004 and 2009 was observed. […] The incidence of pancreatic NET increased from 0.017 per 100,000 in 1996 to 0.446 per 100,000 in 2015 with an APC of 28.04. […] The overall survival of pancreatic NETs improved after introduction of targeted therapies whereas the survival improvement of other gastrointestinal tract and pulmonary NETs awaits evaluation in the near future.

• #7 Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2735065/

  The crude annual overall incidence of PNETs (per 1000000) was 2.2 (1.8 in females and 2.6 in males), and the incidence increased with advancing age at diagnosis. The incidence of PNETs in both sexes also increased over the study period (by 0.05 cases per 1000000 per year, P 0.0001). The observed increase in incidence was greater in males (0.07/1000000 per year, P 0.0001) than in females (0.03/1000000 per year, P = 0.0014). […] Survival has increased over time: patients diagnosed in the later tertiles of the observation period (19811990 and 19912000) had longer OS than patients diagnosed in the earliest tertile (19731980) (P = 0.01). The increased survival retained statistical significance after adjusting for other prognostic predictors in a multivariate analysis. The survival increase is not explained by stage migration as patients in the later tertiles were as likely to be diagnosed with regionally advanced or metastatic disease (P = 0.21).

• #8 Epidemiology, Pathogenesis, and Prognosis of Pancreatic Neuroendocrine Tumors | SpringerLink

  https://link.springer.com/10.1007/978-3-030-41683-6_36

  Pancreatic neuroendocrine tumors (NETs) account for 7% of pancreatic tumors. Most cases of NETs are sporadic. Pancreatic NETs are classified clinically into two groups: functional and nonfunctional. Patients with functional tumors present with variety of symptoms as the result of hormone or active peptides production. Among functional NETs, insulinoma is the most common type; the majority of tumors are benign; however, lesions larger than 2 cm carry higher malignant potential. Gastrinoma is the second most common functional NET, and up to 90% of the tumors are malignant. Due to advances in diagnostic modalities and utility of endoscopic ultrasound, there has been an increase in early detection of pancreatic NETs. Lymphovascular invasion, perineural invasion, higher tumor grade, advanced tumor stage, distant metastasis, and older age at the time of diagnosis are considered poor prognostic factors for disease-free survival. […] Considering mTOR pathway plays a crucial role in pathogenesis of NETs, mTOR inhibitor for management of pancreatic NETs has been shown beneficial.

• #9 Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2735065/

  Pancreatic neuroendocrine tumors (PNETs) are uncommon neoplasms that can present with symptoms of hormone overproduction. We evaluated the incidence, prognosis, and temporal trends of PNETs. […] We analyzed all cases of PNETs registered in the Surveillance, Epidemiology, and End Results database from 1973 to 2000. Age-adjusted incidence and survival rates were calculated and survival trends over time were evaluated. […] We identified 1483 cases of PNETs. The crude annual incidence per 1000000 was 1.8 in females and 2.6 in males and increased with advancing age. The incidence increased over the study period. Most patients (90.8%) had nonfunctional tumors. Advanced stage, higher grade, and age were the strongest predictors of worse survival. Patients with functional tumors had better outcomes than patients with nonfunctional tumors in both univariate and multivariate analysis (P = 0.004). Survival time increased over the period from 1973 to 2000. No differences were seen in the distribution of stage or age at diagnosis among time periods.

• #10 Surgical Management of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/2072-6694/15/7/2006

  Surgical resection remains the primary curative-intent modality for localized PNET, and resection may be associated with improved oncologic outcomes even in some cases of metastatic disease. In addition to oncologic benefits, tumor resection may alleviate symptoms secondary to the hormone secretion for functional PNETs, and resection of primary functioning PNETs is associated with improved overall survival for all stages of disease. The variable behavior of these tumors precludes a single surgical strategy, with management options ranging from nonoperative observation to pancreas-sparing procedures, pancreatectomy with lymphadenectomy, and occasionally metastasectomy. […] Nonfunctional PNETs comprise the majority of all PNETs. Nonfunctional PNETs have a range of malignant potential, in that these tumors can be slow and insidious, and also locally invasive or aggressively metastatic. Nonfunctional PNETs may metastasize to the liver, bone, peritoneum, adrenal gland, brain, and spleen. The 5-year overall survival for nonfunctional PNETs has been reported to be 26–58%. Given that nonfunctional PNETs do not secrete hormones, they often are found incidentally and may therefore present with advanced disease.

• #11 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. […] Many neuroendocrine tumors arise sporadically, whereas some are associated with genetic syndromes, such as multiple endocrine neoplasia type 1 (MEN1). […] Approximately 22% of patients with PNETs have functional tumors, many of which are insulinomas. […] Risk for metastases varies by type; most insulinomas follow an indolent course, but other PNET types, such as gastrinomas, demonstrate a higher risk for metastases. […] Genetic testing for hereditary syndromes should be considered for all patients with PNETs. […] Multiple endocrine neoplasia type 1 (MEN1) is characterized by pancreatic neuroendocrine tumors (PNETs), in addition to pituitary adenomas and hyperparathyroidism.

• #12 Surgical Management of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/2072-6694/15/7/2006

  Pancreatic neuroendocrine tumors (PNETs) are rare malignancies originating from the endocrine tissues of the pancreas. PNETs represent approximately 2% of all pancreatic neoplasms, yet the overall incidence appears to be increasing. This observation is perhaps a result of increased recognition in the setting of improved quality of cross-sectional imaging. PNETs are mainly sporadic, though approximately 10% occur in the context of genetic predispositions including multiple endocrine neoplasia type 1 (MEN1), von Hippel Lindau (VHL) disease, tuberous sclerosis complex (TSC1 and TSC2), and neurofibromatosis (NF1). PNETs can present with a range of clinical behaviors; they may occur as small benign lesions, slow-growing indolent tumors with a favorable prognosis, locally invasive lesions, or as widespread metastatic disease. PNETs are categorized as either functional or nonfunctional based on the ability to secrete biologically active hormones. Functional PNETs secrete specific hormones with subsequent characteristic symptoms and are far less prevalent than nonfunctional PNETs. Nonfunctional lesions have previously thought to comprise approximately 50–85% of all PNETs, though the frequency is likely greater given increased recognition of small, indolent lesions. Overall, the prevalence of nonfunctioning PNETs is on the rise, specifically an increased incidence of small nonfunctional PNETs (less than 2 cm).

• #13 Pancreatic neuroendocrine tumors | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/endocrine-tumours-of-the-pancreas?embed_domain=hackmd.io%252f%2540yipuafecsl2jsu8smr5njq%252fbnjhjgjghjghjghfavicon.icofavicon.icofavicon.icofavicon.ico&lang=us

  Pancreatic neuroendocrine tumors (pNET), also known as endocrine tumors of the pancreas, arise from pancreatic ductal stem cells and include some distinct tumors that match the cell type of origin. […] Overall, pancreatic neuroendocrine tumors have an incidence of 0.001% and account for 1-2% of pancreatic neoplasms. They occur most commonly at ages 30-60 with no clear gender predilection. […] Most tumors are sporadic. Approximately 10% are associated with multiple endocrine neoplasia type 1 (MEN1), von Hippel Lindau disease, tuberous sclerosis, and neurofibromatosis type 1. […] Surgical resection for low-grade localized PNETs can be curative and should be considered for all symptomatic/functional PNETs and non-function PNETs 2 cm in size. Non-functional PNETs may be suitable for active surveillance if 1-2 cm in size.

• #14 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. […] The majority of PNETs are nonfunctional tumors. Nonfunctional tumors do not produce any hormones so they do not cause any hormone-related symptoms. As a result, these tumors are typically diagnosed once the tumor is advanced and is causing symptoms such as pain or jaundice. […] 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.

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

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

  Patients with MEN1 often have multiple PNETs, and insulinomas and gastrinomas are the most frequently seen PNETs in patients with MEN1. […] Approximately 50% of patients with MEN1 will be diagnosed with functioning pancreatic neoplasms (although such neoplasms may be benign), and 20-55% of patients with MEN1 will develop nonfunctioning PNETs. […] Laboratory testing for PNETs is appropriate for diagnosing patients with a pancreatic mass or signs and symptoms of a functional syndrome associated with PNETs. […] 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.

• #16 Pancreatic Neuroendocrine Tumor | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/p/pancreatic-neuroendocrine-tumor.html

  A pancreatic neuroendocrine tumor is a rare type of cancer that starts in the pancreas. […] These tumors are fairly rare. Healthcare providers don’t know why people develop them. […] You are at higher risk for pancreatic NETs if you: Have certain inherited genetic syndromes (those passed in families), such as: MEN1 (multiple endocrine neoplasia type 1) syndrome, Neurofibromatosis type 1, Von Hippel-Lindau syndrome, Drink a lot of alcohol, Smoke, Have type 2 diabetes, Have chronic pancreatitis (inflammation of the pancreas). […] Nonfunctioning pancreatic NETs don’t cause symptoms until they’re big enough to put pressure on other organs, such as the bile ducts. […] Blood tests and imaging scans can help diagnose these tumors. […] Surgery is the main treatment.

• #17 Management of pancreatic neuroendocrine tumors in patients with MEN 1 – Sadowski – Gland Surgery

  https://gs.amegroups.org/article/view/5404/6403

  Pancreatic neuroendocrine tumors (PNETs) are frequent and can be non-functional (NF) in patients with multiple endocrine neoplasia type 1 (MEN1). Their identification is of clinical importance because malignant PNETs are reported to be the most common cause of death in patients with MEN1. Once the diagnosis of MEN1 is established in an individual based on clinical manifestations and/or genetic testing results, an active surveillance program is instituted for early detection and treatment of MEN1-associated disease, especially for tumor sites with malignant potential such as gastrointestinal and pancreatic NETs. The incidence of PNETs in patients with MEN1 varies between 30-80% in different studies. The natural history of NF-PNETs in patients with MEN1 is not well established. NF-PNETs are not associated with any clinical syndrome and include cases with elevated pancreatic polypeptide or glucagon but no symptoms. The identification of these NF-PNETs is of clinical importance because, malignant PNETs are reported to be the most common cause of death in patients with MEN1. Therefore the radiological screening for NF-PNETs in MEN1 should begin by the age of 10 years. To date, the recommendation for surgical resection in NF-PNETs has been based on tumor size, as a higher rate of metastases was found in patients with larger tumors. The most important adverse prognostic factor for survival in patients with MEN1 related PNETs is the presence of liver metastases and other distant disease. In conclusion, PNETs are frequent and can be NF in patients with MEN1. Their identification is of clinical importance because malignant PNETs are reported to be the most common cause of death in patients with MEN1. To date, the recommendation for surgical resection in NF-PNETs is based on tumor size, as a higher rate of metastases was found in patients with larger tumors.

• #18 Epidemiology, treatment and outcomes of gastroenteropancreatic neuroendocrine neoplasms | Scientific Reports

  https://www.nature.com/articles/s41598-024-81518-4

  To investigate incidence, treatment patterns and outcomes of gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) in the United States. Overall survival was evaluated using averaged Cox regression. GEP-NEN incidence increases, likely due to improved detection and diagnosis. Treatment patterns have evolved to follow the latest international guidelines and site-specific improvement in survival is noted. Although GEP-NEN are considered rare, several large population-based studies have reported an increasing incidence over the past four decades, possibly attributable to increased use of cross-sectional imaging and endoscopic procedures and more widely adopted classification schemes. In our cohort, treatment effectiveness varied by stage and grade: across all strata, patients treated with surgical resection demonstrated longest overall survival. This is in line with international guidelines that recommend surgical resection as the mainstay of treatment for low grade local or locoregional GEP-NEN. The addition of systemic therapy was most effective in high stage G3 NEN. Factors associated with improved survival on multivariate analysis included having higher income ($63,333), having private insurance, Medicare, or other government insurance, and receiving treatment at an academic center.

• #19 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. […] Among patients with MEN-1 syndrome, gastrinomas were the most prevalent pNENs (ranging from 20-61%), followed by insulinomas (ranging from 7-31%), and glucagonomas (ranging from 1-5%). All patients with MEN-1 syndrome should undergo active surveillance throughout their lives to reduce the risk of malignant transformation of pNENs.

• #20 Active Surveillance May Benefit Certain Patients With Small Nonfunctional Pancreatic NETs

  https://www.oncnursingnews.com/view/active-surveillance-may-benefit-certain-patients-with-small-nonfunctional-pancreatic-nets

  This retrospective review demonstrates the increased use of active surveillance in patients with small nonfunctional pancreatic neuroendocrine tumors. […] Results from a retrospective review demonstrated significant increases in the use of observation in patients with small nonfunctional pancreatic neuroendocrine tumors (NF-pNETs) from 2015 to 2020, as published in JAMA Surgery. […] These findings highlight a growing nationwide trend in the initial nonoperative management of small NF-pNETs, study authors wrote. […] Currently, experts are uncertain of the metastatic potential of NF-pNETs, and the potential morbidity linked with pancreatic surgery, as noted in the study. […] Further research on the long-term efficacy of active surveillance, cost-effectiveness strategies, and further refining patient-related risk factors other than tumor size will ultimately guide the evidence-based and personalized management of small NF-pNETs, study authors concluded.

• #21 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/

  A systemic review and meta-analysis of 344 patients with small, nonfunctional pNENs reported that 22% of patients experienced an increase in tumor size during surveillance, but only 12% of patients ultimately needed surgical resection. […] In recent years, EUS-guided radiofrequency and ethanol ablation have emerged as novel techniques for the treatment of patients with pNENs.

• #22 SEER Inquiry System – Question 20120063 Details

  https://seer.cancer.gov/seer-inquiry/inquiry-detail/20120063/

  According to the World Health Organization (WHO) pancreatic neuroendocrine tumors (NET) are malignant. They are reportable. […] For pancreas primaries, code NET, G1 (well differentiated) to 8240/3; NET G2 (moderately differentiated) to 8249/3; and nonfunctional NET, GI or G2 to 8150/3. […] The histology code for neuroendocrine carcinoma (NEC) is 8246/3, large cell NEC is 8013/3 and small cell NEC is 8041/3.

• #23 Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8118193/

  The 5-year OS rates were 93.5%, 66%, and 43% for grade 1, grade 2, and NEC patients, respectively. […] The age-adjusted incidence rate of all GEP NET tumors increased from 1996 to 2008, though not in a uniform fashion. […] The crude annual incidence of all GEP NENs was 5.83 cases per 100,000. […] The crude annual incidence of GEP NENs rose from 2.37 cases per 100,000 in 2003 to 8.35 cases per 100,000 in 2009; there was an increasing linear trend in crude incidence rates during the study period. […] The magnitude of rise in GEP NET incidence rates appears to be most profound in North America. […] The most prevalent primary sites of GEP NETs appear to differ between North America, Asia, and Europe.

• #24 The Role of Surgery for Pancreatic Neuroendocrine Tumors | Anticancer Research

  https://ar.iiarjournals.org/content/42/2/629

  According to the North American Neuroendocrine Tumor Society (NANETS) guidelines, patients with poorly differentiated NEC should not be considered as surgical candidates due to the aggressive disease biology and poor prognosis of NECs. In contrast, patients with localized, well-differentiated grade 3 PNETs can be considered for resection in the context of multimodal treatment, such as neoadjuvant therapy. […] In 2018, Li et al. performed a Surveillance, Epidemiology, and End Results database analysis of 2,350 patients who underwent oncological resection between 2004-2014. They showed that the 5-year overall survival rate for patients with stage I PNETs was 89.9%, for stage II PNETs was 82.6%, for stage III PNETs was 75.8%, and for stage IV PNETs was 56.9%. […] Although no definitive consensus was reached among the experts in the NANETS guidelines, the majority deemed there to be a benefit of primary tumor resection in cases with unresectable metastatic disease.

• #25 Survival for neuroendocrine cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/non-functioning/survival

  The following statistics are for survival for the different stages of all types of pancreatic neuroendocrine cancer. We have statistics for: […] More than 85 out of 100 people (more than 85%) survive for 5 years or more […] 85 out of 100 people (85%) survive for 5 years or more […] Around 70 out of 100 people (around 70%) survive for 5 years or more […] Around 25% out of 100 people (around 25%) survive for 5 years or more […] Around 70 out of 100 people (around 70%) survive for 5 years or more […] Around 60 out of 100 people (around 60%) survive for 5 years or more […] Almost 35 out of 100 people (almost 35%) survive for 5 years or more […] Around 10 out of 100 people (around 10%) survive for 5 years or more.

• #26 The Role of Surgery for Pancreatic Neuroendocrine Tumors | Anticancer Research

  https://ar.iiarjournals.org/content/42/2/629

  The NANETS guidelines recommend observation for non-functional PNETs 1 cm and a patient-oriented management for those 1-2 cm depending on age, comorbidities, grade, growth, extent of the required operation, and patient preference. The ENETS and the National Comprehensive Cancer Network guidelines state that observation of non-functional PNETs 2 cm with EUS, MRI, or CT scan, every 6-12 months is a reasonable option. […] Overall, according to the current body of evidence, surgical resection is a reasonable option for well-selected patients with functional PNETs of any size or non-functional PNETs 2 cm. For patients with non-functional PNETs 1-2 cm, a patient-oriented approach should be used based upon age, comorbidities, tumor location, differentiation, and staging.

• #27 Role of surgery in pancreatic neuroendocrine tumor – Wong – Gland Surgery

  https://gs.amegroups.org/article/view/18046/html

  Although several guidelines exist to guide the treatment of functioning and non-functioning PNETs. It is generally accepted that tumours 2 cm and functioning tumours should be resected but the management of non-functioning tumours 2 cm remains controversial. Further controversy exists as to the approach of resection (open vs. laparoscopic) and the management of PNETs with liver metastasis which occurs in up to 80% of patients. The aim of this paper is to review the current evidence of surgical resection in PNETs, the role of laparoscopic surgery and the management of liver metastasis. […] In general, it is recommended that PNET which is symptomatic, functional, size 2 cm or with presence of aggressive features like pancreatic duct dilation should undergo pancreatic resection. However, it is controversial whether surgery should be recommended for PNET 2 cm as primary treatment. Some surgeons recommended upfront surgery, while other guidelines recommended active surveillance for asymptomatic small PNET. Most less than 2 cm PNET were likely in benign and indeterminate risk in malignancy. Only 6% of incidental found smaller than 2 cm non-functioning PNET is confirmed to be malignancy. Small PNET (2 cm) were slowing growing with indolent course, it is considered to be amenable to observation.

• #28 Surgical Management of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/2072-6694/15/7/2006

  A National Cancer Database (NCDB) study of over 2000 PNET patients demonstrated active surveillance can be reasonably pursued for tumors less than 1 cm. Similarly, a surveillance, epidemiology, and end results (SEER) database study found the likelihood of aggressive behavior in nonfunctioning PNETs less than or equal to 2 cm was low, and there was no survival benefit with resection. Several retrospective database studies evaluating the role of active surveillance for small (less than or equal to 2 cm) nonfunctional PNETs suggest that a nonoperative approach is safe. Importantly, all patients followed nonoperatively require definitive diagnosis via either fine needle aspiration (FNA) or somatostatin-receptor imaging. […] While data remain limited, there are several expert society guidelines that have addressed the management of small, nonfunctional PNETs. Per a recent consensus statement by the North American Neuroendocrine Tumor Society (NANETS), asymptomatic patients with tumors less than 1 cm and imaging consistent with PNET can be observed. However, for tumors 1–2 cm, it is advised that management be based on patient comorbidities, tumor grade, extent of resection if surgery is pursued, patient preference, and access to follow-up care. The National Comprehensive Cancer Network (NCCN) recommendations suggest that tumors less than or equal to 2 cm can be observed; however, evidence is stronger for surveillance of tumors less than or equal to 1 cm. The Canadian National Expert Group consensus on nonfunctional PNET surgical management states for tumors less than 2 cm, active surveillance can reasonably be pursued; specifically, tumors should be solitary lesions with no evidence of invasive disease, have low Ki67, and continue to demonstrate stability on serial imaging and biochemical monitoring every 6 months. The European Neuroendocrine Tumor Society (ENETS) recommends active surveillance for nonfunctional PNETs that are less than or equal to 2 cm. Tumors should be low-grade (G2 or less), asymptomatic, and without radiographic evidence suspicious for malignancy. However, surgery is recommended if the tumor is symptomatic or patient’s preference is for resection. Active surveillance includes imaging every 6 to 12 months.

• #29 Role of surgery in pancreatic neuroendocrine tumor – Wong – Gland Surgery

  https://gs.amegroups.org/article/view/18046/html

  However, there were limitations with the current evidence supporting active surveillance. As PNET is a slow-growing tumor, studies with follow-up up to 3 to 4 years cannot reflect the long-term prognosis. It is important in stratifying patients for surgical interventions, especially young individuals who had found a small PNET. Young patients are likely to live long but have to cope with the stress and the presence of the disease. As the majority of reasons for pancreatic resection is tumor growth, there is a cumulative need for operation in long-term follow up. On the other hand, nearly all studies were retrospective in nature. They did not state the criteria in selecting surgery or observation. The treatment decision was based on patients age, pre-morbid conditions and patients decision. It was reflected by the fact that patients in observation were usually older.

• #30

  http://www.bccancer.bc.ca/books/neuroendocrine-tumors/follow-up-of-neuroendocine-tumors

  Follow-up of Neuroendocrine Tumours: Low-risk Resected Disease […] Low risk of recurrence […] Routine surveillance imaging or lab work is not recommended. Clinical follow up at the discretion of the treating physician. […] High-risk Resected Disease […] Surveillance per the table below if any of the following high risk features: primary greater than 2 cm, nodal involvement, high Ki67 (5%) […] If high risk and grade 3 disease period of recommended follow-up is 5 years […] If high risk and grade 1-2 period of recommended follow-up is 10 years […] Unresected disease (metastatic or residual-positive post-debulking)

• #31

  https://journals.lww.com/10.1097/XCS.0000000000000105

  Pancreatic neuroendocrine tumor (PNET) survival outcomes differ by race. Current recommendations for surveillance of PNETs less than 2 cm in size are based on low malignant potential and low rates of lymph node metastases (LNM). […] The current recommendation for surveillance of PNETs of less than 2 cm in size is likely based on a low rate of LNM seen in a predominantly White population. The incidence of LNM in Black patients with tumors less than 2 cm in size is clinically relevant and concerning. Current guidelines may not be universally applicable, and a more aggressive approach to resection in Black patients with small PNETs may be warranted. […] Black patients with pancreatic neuroendocrine tumors are more likely than White patients to have lymph node metastases with smaller tumors; current size-based guidelines are not likely applicable to this patient population.

• #32 Relative Survival Rates of Pancreatic Neuroendocrine Tumors, Carcinomas – Gastroenterology Advisor

  https://www.gastroenterologyadvisor.com/news/pancreatic-neuroendocrine-tumors-carcinomas-incidence-and-survival-rates/

  In this analysis of population-based German pancreatic cancer registries, neuroendocrine tumors had the best prognosis, followed by neuroendocrine carcinomas and adenocarcinomas. […] In conclusion, the distinction of neuroendocrine neoplasms in neuroendocrine tumors and neuroendocrine carcinomas by the WHO results in two prognostically clearly distinct subgroups that should always be analyzed separately in terms of survival, the study authors wrote.

• #33 Active surveillance in metastatic pancreatic neuroendocrine tumors: A 20-year single-institutional experience

  https://www.wjgnet.com/2307-8960/full/v8/i17/3751.htm

  In this study, we retrospectively reviewed the clinicopathological data of patients with liver metastatic NF-PanNETs who received active surveillance in our institution over the past 20 years. Additionally, we established a nomogram to predict the suitable characteristics for active surveillance in liver metastatic NF-PanNET patients. […] The median TTP (mTTP) was 10 mo in 76 patients with liver metastatic NF-PanNETs. Multivariate analysis showed that the largest axis of the liver metastasis 5 mm (P = 0.04), non-resection of the primary tumor (P = 0.024), and T3-T4 stage (P = 0.028) were associated with a shorter TTP. […] Patients with favorable factors had a time to progressive disease of more than two years without systemic treatment; further studies with a larger sample size and a control are required. […] Active surveillance may only be safe for metastatic NF-PanNET patients with favorable risk factors, and other patients progressed rapidly without treatment.

• #34

  https://insight.jci.org/articles/view/160130

  Within the last decade, immunotherapy targeting immune checkpoint molecules has emerged as a highly effective treatment option in a subset of patients across a range of immunogenic cancers with elevated baseline T cell infiltration, including melanomas and nonsmall cell lung cancers, or in cancers with mismatch repair deficiencies. […] However, early clinical data with respect to immune checkpoint blockade has been less promising in patients with PNETs. […] The wide variety of morphologies, behaviors, and genetic heterogeneity observed among PNETs has contributed to inconsistent reports of prognostic association for each of these markers. […] To investigate early changes accompanying disease progression to metastasis, we performed RNA-Seq analysis on surgically excised primary tumors originating from both localized and metastatic PNETs.

• #35

  https://insight.jci.org/articles/view/160130

  We identified potential therapeutic options for PNET management and delineated the mechanism by which increased T cell recruitment to the tumor could be optimized. […] The data presented here demonstrate that the tumor-immune microenvironment is different between localized and metastatic PNETs in the primary pancreas tissue. […] Our findings support the growing body of literature that suggests a more complex TME among PNETs, with definitive increases in the percentage of CD69+, CCR5+, and PDCD1+ T cells among localized, and likely metastatic, tumors compared with peripheral blood. […] Our analysis also demonstrates a potential avenue of immunogenic optimization via TME modulation with vorinostat.

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