Pancreatic Neuroendocrine Tumors. An overview of the disease and its management

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1 Pancreatic Neuroendocrine Tumors An overview of the disease and its management

2 Introduction Pancreatic neuroendocrine tumors (pancreatic NET) comprise a group of neoplasms that are believed to arise from pancreatic endocrine cells found among the exocrine tissue of the pancreas. 1,2 Pancreatic NET are also called islet cell tumors because the endocrine cells of the pancreas are commonly located in clusters, anatomically called islets of Langerhans. 3 Pancreatic NET can also arise from cells other than islet cells and can originate outside of the pancreas, for example, in the duodenum. 2,4,5 Pancreatic NET have notably different tumor biology from pancreatic adenocarcinomas. They vary in clinical presentation and aggressiveness, and can present unique challenges for health care professionals who diagnose and manage patients with this uncommon cancer. This brochure provides an overview of pancreatic NET, covering the following topics: Types of pancreatic NET Epidemiology Classification Prognostic factors Staging and grading Biochemical analysis Imaging Management A word about neuroendocrine nomenclature This brochure uses the nomenclature established in the 2010 WHO Classification of Tumours of the Digestive System 6 to classify 2 broad types of neuroendocrine neoplasms: Neuroendocrine tumors (NET): well-differentiated neuroendocrine neoplasms that can be divided into grade 1 (G1) and grade 2 (G2), depending on proliferation and histology Neuroendocrine carcinomas: poorly differentiated grade 3 (G3) neuroendocrine neoplasms NET may be referred to using a variety of terms, such as carcinoids, carcinoid tumors, or endocrine tumors. These terms can be used interchangeably with NET as defined above. 2

3 The other pancreatic cancer Pancreatic NET are less common than pancreatic adenocarcinomas and other neoplasms of the exocrine pancreas. 7 Prevalence of major pancreatic cancer types among all pancreatic cancers Exocrine Pancreatic adenocarcinoma 61% to 70% 7-9 Endocrine Mucinous tumors 6% to 7% 7-9 Pancreatic NET 2% to 4% 7-9 Compared with tumors of the exocrine pancreas, pancreatic NET have distinctly different tumor biology and are associated with longer survival. 7 % Survival Months Kaplan-Meier curves comparing percent survival for 5 years in the pancreatic cancer histologic types: Adenocarcinoma (n=31,357) Mucinous tumors (n=2865) Endocrine tumors (n=1054) 7 Histology Groups Reproduced with permission of the American Association for Cancer Research, from Fesinmeyer MD et al, vol 14, 2005; permission conveyed through Copyright Clearance Center, Inc. 3

4 Types of pancreatic NET Most pancreatic NET are well-differentiated (grade 1 and grade 2) tumors 10,11 with diverse clinical presentations. 4,5 These presentations are often related to the ability of some pancreatic NET to secrete hormones or peptides, such as insulin, gastrin, glucagon, vasoactive intestinal polypeptide (VIP), or somatostatin, causing characteristic hormonal syndromes. 3,4 However, not all pancreatic NET are associated with secretion of hormone products related to clinical symptoms; these tumors are considered to be clinically nonfunctional. 4,12,13 Characteristics of Pancreatic NET Type of pancreatic NET 5 Incidence (cases per million) 14 Hormone/ peptide associated with symptoms 4,5,15 Major signs/ symptoms 4,5,15,16 Prevalence of metastases Nonfunctional 1-2 a None May be first diagnosed due to mass effect, weight loss, hepatomegaly, abdominal mass, or incidentally >50% 5 Functional b Insulinoma Insulin Hypoglycemia <15% 5 Gastrinoma Gastrin Recurrent peptic ulcer, abdominal pain, diarrhea (also known as Zollinger-Ellison syndrome) <50% 5 Glucagonoma Glucagon Diabetes/glucose intolerance, rash (migratory necrolytic erythema), thromboembolic disease Majority 5 VIPoma 0.10 Vasoactive intestinal polypeptide (VIP) Severe watery diarrhea, electrolyte disturbances (also known as Verner-Morrison syndrome; pancreatic cholera; or watery diarrhea, hypokalemia, and achlorhydria [WDHA] syndrome) Majority 5 Somatostatinoma <0.10 Somatostatin Diabetes, cholelithiasis, diarrhea Majority 5 a Incidence of nonfunctional NET is probably higher due to the fact that autopsies reveal undiagnosed nonfunctional NET. b Other exceedingly rare hormonal syndromes can occur 5 but are not covered in this monograph. 4

5 Epidemiology The reported overall annual incidence of malignant pancreatic NET is believed to be approximately 0.32 per 100,000 individuals, based on statistics from the largest database, the US SEER Program (Surveillance, Epidemiology, and End Results Program of the National Cancer Institute) registries ( ). 17 The most common pancreatic NET are nonfunctional pancreatic NET, insulinomas, and gastrinomas; other types, such as glucagonomas, somatostatinomas, and VIPomas, are rare. 5 Pancreatic NET can be sporadic or associated with genetic syndromes, 5 such as multiple endocrine neoplasia type 1 (MEN 1), von Hippel-Lindau syndrome (VHL), von Recklinghausen disease (neurofibromatosis [NF-1]), and tuberous sclerosis complex (TSC). 18 MEN 1 is the genetic syndrome most frequently associated with pancreatic NET. 5,18 MEN 1 is an autosomal dominant condition typically associated with neoplasms that develop in the pituitary gland, parathyroid glands, and endocrine pancreas. 15 Patients with MEN 1 usually have multiple pancreatic NET, whereas those with sporadic pancreatic NET generally have solitary neoplasms. 5,19 The relative frequency of MEN 1 associated pancreatic NET, in descending order, is nonfunctional pancreatic NET, gastrinomas, insulinomas, glucagonomas, VIPomas, and somatostatinomas. 5,18 In patients with MEN 1, nonfunctional pancreatic NET may be microscopic and asymptomatic. 5,18 Prevalence in Patients With Pancreatic NET (in surgical series) Nonfunctional a 58%-71% 16,20 Functional b Insulinoma 23%-33% 16,20 Gastrinoma 3%-5% 16,20 Glucagonoma 2%-3% 16,20 VIPoma 0.6%-1.0% 16,21 Somatostatinoma 0.05% 21 a The frequency with which nonfunctional pancreatic NET are being identified and treated is increasing due to an increase in detection rather than a true increase in incidence. 16 b Other exceedingly rare hormonal syndromes can occur 4 but are not covered in this monograph. 5

6 Classification Stage The World Health Organization (WHO) 2010 classification system follows that of the American Joint Committee on Cancer (AJCC) and International Union Against Cancer (UICC) for pancreatic adenocarcinoma The tumor-node-metastasis (TNM) classification embraced by WHO is different from the one used by the European Neuroendocrine Tumor Society (ENETS). The WHO system is for well-differentiated NET only. The ENETS system is also meant for high-grade neoplasms. 24 WHO TNM Classification of Tumors of the Pancreas 25 T-Primary Tumor Stage Grouping TX T0 Tis T1 T2 T3 T4 Primary tumor cannot be assessed No evidence of primary tumor Carcinoma in situ, includes PanIN-3 Tumor limited to the pancreas, 2 cm in greatest dimension Tumor limited to the pancreas, >2 cm in greatest dimension Tumor extends beyond the pancreas Tumor involves the celiac axis or the superior mesenteric artery Stage T N M Stage 0 Tis N0 M0 Stage IA T1 N0 M0 Stage IB T2 N0 M0 Stage IIA T3 N0 M0 Stage IIB T1 N1 M0 T2 N1 M0 T3 N1 M0 Stage III T1 Any N M0 Stage IV Any T Any N M1 N-Regional Lymph Nodes NX Regional lymph nodes cannot be assessed Adapted from Bosman FT, Carniero F, Hruban RH, Theise ND. World Health Organization Classification of Tumours of the Digestive System. IARC, Lyon, PanIN, pancreatic intraepithelial neoplasia. N0 N1 No regional lymph-node metastasis Regional lymph-node metastasis M-Distant Metastasis M0 M1 No distant metastasis Distant metastasis 6

7 Grade Pancreatic NET should also be classified by grade, a fundamental predictor of outcome that estimates the biologic aggressiveness (ie, potential for metastatic spread) of the neoplasm. 5,26 The WHO grading system is one of many grading systems, which differ in both terminology and criteria. 5,6,26 To address these differences, a multidisciplinary group of specialists in the field of NET recently recommended that the grading system used must be specified on the pathology report. 26 ENETS Modified Clinical, Radiological, and Pathological TNM Staging of Pancreatic Endocrine Tumors 27 Stage T a N b M c I T1 or T2 N0-NX M0 II T3 N0-NX M0 III T4 N0-NX M0 Any T N1 IV Any T Any N M1 a T1, tumor limited to the pancreas and size <2 cm; T2, tumor limited to the pancreas and size between 2 and 4 cm; T3, tumor limited to the pancreas and size >4 cm; T4, tumor invasion of any adjacent structure (including duodenum, common bile duct and peripancreatic fat), documented by histology or clinical/imaging signs of malignancy (jaundice or duodenal bleeding due to infiltration, dilation of main pancreatic or common bile duct). b NX, lymph nodes not assessed; N0, absence of lymph node metastasis; N1, invasion of regional lymph nodes. c M0, absence of distant metastasis; M1, presence of distant metastasis. Adapted from Scarpa A et al with permission of Nature Publishing Group WHO Grading of Pancreatic Neuroendocrine Neoplasms 6,28 Grade Low-grade NET G1 Intermediate-grade NET G2 High-grade neuroendocrine carcinoma G3 Criteria <2 mitoses/10 HPF AND/OR <3% Ki-67 index 2-20 mitoses/10 HPF AND/OR 3% - 20% Ki-67 index >20 mitoses/10 HPF AND/OR >20% Ki-67 index HPF, high-power field. 7

8 Prognosis In pancreatic NET, prognostic factors include location of the primary tumor, disease stage, patient age, and years since diagnosis; however, the most important predictor of outcome is stage. 3 Disease Stage at Diagnosis Survival probability Extent of disease and survival from time of diagnosis in patients with well-differentiated (grade 1 and grade 2) pancreatic NET. Median duration of survival in patients with localized, regional, and distant disease was 124, 70, and 23 months, respectively (P<0.001). 3 Localized disease (n=167) Regional disease (n=289) Distant disease (n=558) Months Reproduced with permission from Springer Science + Business Media. Ann Surg Oncol, vol 14, 2007, p3496, Yao JC et al, Figure 3. This underscores the need for greater awareness of and vigilance for diagnostic indicators and the importance of making an informed, multidisciplinary diagnosis as early as possible in the course of disease. Disease stage at diagnosis and patient prognosis vary across different types of pancreatic NET. For example, the majority of patients with glucagonoma, VIPoma, and somatostatinoma present with metastases at diagnosis. 5 However, in patients with gastrinoma, metastases are present in fewer than 50%, and in insulinoma, metastases are present in fewer than 15%. 5 This finding has clinical implications, because the presence of distant metastases is an important predictor of outcome in patients with pancreatic NET. 21 When Bilimoria et al analyzed outcomes in 4793 patients with pancreatic NET and applied the AJCC staging system, distant metastasis was found to be the only predictor of survival (P<0.0001) and was associated with a 2-fold increased likelihood of death. 22 8

9 Prognosis (cont) When the US SEER database from was used to specifically analyze 1157 patients with pancreatic NET, the longest survival (124 months) was in patients with localized disease (n=167). 3 The majority of patients had distant disease (n=558) and the shortest median duration of survival (23 months). Grade and patient age Patient age and neoplasm grade are other prognostic factors to consider. 21 In 2008, Bilimoria and colleagues compared patients 55 years of age or younger with those aged 55 to 75 years and with those aged 75 years or older. Both the middle age and the oldest age groups had an increased risk of death after resection (hazard ratios, 1.57 and 3.04, respectively; P<0.0001). Likewise, patients with high-grade pancreatic neuroendocrine neoplasms had a 2-fold increase in the postresection risk of death compared with those who had low-grade pancreatic NET (hazard ratio, 2.03; P<0.0001). 21 Prognostic biomarkers Like other NET, pancreatic NET generally produce and secrete marker proteins, such as chromogranin A (CgA), and express somatostatin receptors (SSTRs). 29,30 CgA and SSTR Expression in Pancreatic NET Elevated CgA levels (% sensitivity) Functional 50% 29 Nonfunctional 57% 29 Expression of high-density SSTRs Insulinomas 50%-70% 30 Gastrinomas 80%-100% 30 Glucagonomas 80%-100% 30 VIPomas 80%-100% 30 Consensus and clinical management guidelines variously recognize the prognostic significance of CgA in patients with NET. Elevated plasma CgA levels may be associated with poor overall prognosis; one prospective database analysis found that CgA levels elevated twice or higher the normal limit were associated with shorter survival times in patients with metastatic NET (adjusted HR 2.7 [1.9, 4.0], P<0.001). 31 Conversely, early decreases following treatment may be associated with favorable outcomes. Care should be taken in measuring and interpreting CgA results. Levels may also be elevated due to proton pump inhibitor therapy; and in patients with kidney or liver failure, hypertension, or chronic gastritis. 15 Elevated 24-hour urinary 5-hydroxyindoleacetic acid (5-HIAA) levels can be a surrogate marker of possible progression or treatment response, but this measurement is not as useful in patients with pancreatic NET that do not secrete serotonin. 28 9

10 Prognosis (cont) Prognosis compared with other types of NET Retrospective analyses of cancer registries indicate that patients with pancreatic NET present with distant metastases more often than patients with any other NET. 3,17,32 In the US SEER database, patients who had pancreatic NET with distant metastases had a median survival of 24 months, compared with median survival of 33 months across all NET sites with distant metastases. 17 Studies in large treatment centers report longer median survival times for patients with pancreatic NET. In one such recent series, Strosberg et al evaluated all cases of differentiated, metastatic pancreatic NET seen at the center between the years 1999 and 2003, measuring survival from time of diagnosis of distant metastases. 33 In this analysis, the median overall survival for patients with metastatic pancreatic NET was 70 months; more than half of all patients survived for more than 5 years. Impact of disease stage across NET types A prospective study comparing prognostic factors and survival in 156 patients with pancreatic NET or gastrointestinal (GI) NET (jejunum, rectum, appendix, duodenum, stomach, and colon) found that patients diagnosed with pancreatic NET had a poorer prognosis relative to patients with other NET. 34 In this study, pancreatic primary tumor site was significantly associated with poor outcome in both univariate and multivariate analyses (P = and 0.017, respectively), 34 and survival rates were significantly worse in patients with pancreatic NET compared to those with GI NET. 34 The prevalence of advanced disease was relatively, but not statistically, higher in patients with pancreatic NET compared to those with GI NET (55% vs 35%, respectively). 34 The prevalence of advanced disease, combined with the observation that 61.2% of patients with pancreatic NET had no associated hormonal syndrome, led the authors to conclude that diagnosis is usually late in patients with pancreatic NET due to the lack of specific symptoms. 34 Conversely, a second, larger prospective study of prognostic factors in 399 patients found that the occurrence of metastases in pancreatic NET was 77%, a rate lower than that among patients with NET of the small intestine or colon, 35 suggesting that a pancreatic NET may be diagnosed at an earlier stage than a NET of these other sites. 10

11 Biochemical analysis The clinical presentation of a pancreatic NET largely depends on the functional status of the neoplasm. 4,5,36 In a functional pancreatic NET, symptoms are usually determined by the type of hormone secreted, 4,5 whereas in a nonfunctional pancreatic NET, symptoms generally reflect tumor growth or spread. 4,5 Measurements of hormones and other secreted peptides can be useful in both diagnostic and disease monitoring applications. 5 Hormone measurements may be related to secretion by a particular neoplasm (eg, insulin, proinsulin, gastrin, glucagon, somatostatin) Other markers are nonspecific and are produced and secreted by most NET, such as CgA, 5-hydroxyindoleacetic acid (5-HIAA), and pancreatic polypeptide (PP). 36,37,40 For diagnostic accuracy and ongoing disease management, biomarker results should be confirmed with imaging studies and biopsy, when appropriate. 41 Nonfunctional pancreatic NET Because nonfunctional pancreatic NET are not associated with a hormonal syndrome, symptoms at presentation are indicative of mechanical problems caused by the tumor s bulk or metastases (eg, jaundice, abdominal pain, weight loss, diarrhea). 4,5,16,40 However, pancreatic NET can become very large without causing any symptoms, 40 and often are discovered incidentally during surgery or abdominal imaging. 2,5,16,40 Nonfunctional pancreatic NET may not secrete peptides that cause specific clinical syndromes, but they are not necessarily biologically silent 12 and, in fact, can secrete a number of peptides, 4,5,12 for example, CgA, 5-HIAA, and PP. 4,5,15 When measuring these biomarkers and interpreting the results, it is important to keep in mind that these biomarkers are also secreted by other types of NET. 5,36,40 Other conditions that can increase CgA levels and potentially cause a false-positive result include chronic atrophic gastritis, chronic inflammatory diseases, renal or hepatic failure, arterial hypertension, and proton-pump inhibitor (PPI) therapy. 42 For PP, both physiologic (eg, meals, age) and pathologic (eg, kidney failure, inflammatory diseases) conditions can cause false-positive results. 42 Therefore, an elevated CgA or PP level alone is not diagnostic of a pancreatic NET. 4,5 11

12 Insulinomas Insulinomas are typically small (<1 cm), single tumors that are almost always (>99%) intrapancreatic in location. 5 Insulinoma patients usually present with symptoms of hypoglycemia, especially neuroglycopenic symptoms (eg, confusion, altered consciousness). 5 Insulinomas in non MEN 1 patients can be treated by laparoscopic approach, and, if they can be localized preoperatively, can be cured in 70% to 100% of cases. 5 Clinical manifestations of insulinoma are related to hypoglycemia secondary to excessive, unregulated secretion of insulin. 12,43 Inappropriate hyperinsulinemia causes neuroglycopenic symptoms (eg, visual disturbances, confusion, abnormal behavior, and, in extreme cases, loss of consciousness resulting in coma). 12,40 In addition, catecholamines released in response to the hypoglycemia cause adrenergic symptoms (eg, perspiration, anxiety, heart palpitations, hunger). 40,44 Weight gain is common, since patients learn early in the disease process that eating resolves their symptoms. 40,43 The diagnosis of insulinoma requires demonstration of hypoglycemia in the presence of inappropriate hyperinsulinemia. 12 Biochemical workup for 6 parameters should take place during a 72-hour fast. 37 Biochemical Workup for Insulinomas (during a 72-hour fast) 37 Biomarker Glucose Insulin Proinsulin C-peptide β-hydroxybutyrate Sulfonylurea Levels in insulinoma Blood glucose levels 2.2 mmol/l ( 40 mg/dl) Concomitant insulin levels 6 μu/ml ( 36 pmol/l) Levels 5 pmol/l Levels 200 pmol/l Levels 2.7 mmol/l Absence of sulfonylurea (metabolites) in plasma and/or urine Gastrinomas Gastrinomas can occur as isolated, sporadic neoplasms or as multiple, MEN 1 associated neoplasms 5,45 and usually are located in the pancreas or the duodenum. 46 Patients typically present with abdominal pain related to peptic ulcer disease (PUD), diarrhea, and gastroesophageal reflux disease. 5,47 Gastrinomas can cause a clinical syndrome known as Zollinger-Ellison syndrome (ZES). 4,46 ZES should be suspected if the PUD is recurrent, resistant to treatment, severe, or familial; the PUD is associated with complications, endocrinopathies, prominent gastric folds, or hypergastrinemia; or Helicobacter pylori and nonsteroidal anti-inflammatory drug (NSAID) use are absent. 5,38 A diagnosis of ZES should be suspected if there is evidence of hypergastrinemia in the presence of gastric acid hypersecretion. 5,46 12

13 Biochemical Workup for Gastrinoma Biomarker Fasting serum gastrin (FSG) Gastric ph Basal acid output Rationale Hypergastrinemia is present at the time of acid hypersecretion in patients with gastrinomas 5 Gastric ph <2 in patients with ZES in the absence of antisecretory drugs can help in the differential diagnosis by demonstrating inappropriate hypergastrinemia 5,38,48 Assessment in patients with known hypergastrinemia confirms inappropriate hypergastrinemia 5 Secretin stimulation test This test can help to diagnose gastrinoma 5 Gastric ph, basal acid output, and a secretin stimulation test should be performed after cessation of PPIs, if possible. 38 There can be some risk in stopping PPIs in patients suspected of having a gastrinoma 5 ; these patients should be managed at a center experienced in diagnosing ZES. 5,40 Glucagonomas Glucagonomas are typically large and occur almost entirely within the pancreas. 4,5,12 Glucagonomas secrete excessive amounts of glucagon, causing glucose intolerance, weight loss, and a pathognomonic rash known as necrolytic migratory erythema. This rash is characterized by raised erythematous patches that begin in areas of friction, such as the perineum, and eventually can involve the trunk, extremities, and other parts of the body. 5,12 Although necrolytic migratory erythema is found in up to 90% of patients with glucagonomas, it also can occur in cirrhosis, pancreatitis, and celiac disease. Thus, diagnosis of a glucagonoma depends on the demonstration of pathologic hyperglucagonemia (glucagon levels elevated to 500 to 1000 pg/ml; normal range <50 pg/ml). 4,5,12 VIPomas VIPomas cause a distinct clinical syndrome known by several names, including Verner-Morrison syndrome, pancreatic cholera, and WDHA (for watery diarrhea, hypokalemia, and achlorhydria) syndrome. 5 Patients typically present with severe, large-volume, watery diarrhea, hypokalemia, and hypochlorhydria. The definitive diagnosis requires demonstration of elevated serum concentrations of VIP (>200 pg/ml) and localization of a pancreatic NET in the presence of large-volume secretory diarrhea. 4,12 Somatostatinomas Somatostatinomas cause a clinical syndrome characterized by diabetes mellitus, cholelithiases, diarrhea, and steatorrhea. 4,12 Diagnosis is based on the demonstration of hypersomatostatinemia in the presence of a pancreatic or duodenal mass and at least some features of the clinical syndrome. 4,5,12 13

14 Imaging Imaging studies are used during all phases of the diagnosis and management of patients with pancreatic NET to localize and stage the neoplasm, to guide management plans (ie, curative resection, debulking, medical management only), to monitor neoplasm growth, and for follow-up after therapy. 4,5,12 A number of different modalities are available for the imaging of pancreatic NET. 5 Because the characteristics of each pancreatic NET subtype vary, a combination of imaging studies may be utilized. 2 Modalities Available for Imaging of Pancreatic NET Modality Rationale for use/application Computed tomography (CT) Magnetic resonance imaging (MRI) Somatostatin receptor scintigraphy (SRS) Endoscopic ultrasound (EUS), esophagogastroduodenoscopy (EGD) Intraoperative localization Positron emission tomography (PET) At least 1 conventional imaging method is usually available at most centers 5 CT with contrast is the most frequently used initial imaging method 5 MRI can be considered in patients who are potential surgical candidates for a more accurate assessment of hepatic disease burden Radiolabeled tracers bind to somatostatin receptors that are overexpressed on pancreatic NET cell surfaces 5 Combined with single-photon emission CT (SPECT) imaging is more sensitive than conventional imaging for detection of primary pancreatic NET and distant metastases 5,49 Allows quick total body scanning 5 Helpful in localizing insulinomas, which are frequently missed by conventional imaging and SRS 5 Can identify 90% of intrapancreatic NET 5 Can detect small nonfunctional pancreatic NET in patients with MEN 1 or von Hippel-Lindau (VHL) syndrome 5 EGD can help identify Zollinger-Ellison syndrome in patients with suspected gastrinoma, and also duodenal NET and submucosal duodenal lesions Intraoperative ultrasonography is the most effective method to visualize small tumors, eg, insulinomas 49 Endoscopic transillumination is recommended for small duodenal pancreatic NET 5 PET, especially when combined with CT, is more sensitive than SRS and other modalities in some applications PET with 5-HTP or -dopa can be an option for detection of small tumors 49 14

15 Modalities Available for Imaging of Pancreatic NET (cont) Modality Computed tomography (CT) Magnetic resonance imaging (MRI) Limitation/disadvantage Conventional imaging often misses small primary pancreatic NET (especially insulinomas and duodenal gastrinomas) and small liver metastases 5 Somatostatin receptor scintigraphy (SRS) False-positive localizations can occur in up to 12% of patients 5 Unable to provide details about the location of the tumor in relationship to surrounding structures 2 Many insulinomas lack somatostatin receptor 2, 2 leading to negative SRS studies Endoscopic ultrasound (EUS), esophagogastroduodenoscopy (EGD) Operator-dependent 2 Unable to evaluate hepatic and distant metastases 2 Intraoperative localization Intraoperative localization can only be performed during surgery 5 Positron emission tomography (PET) Less sensitive than other imaging modalities 49 15

16 Management Optimal management of pancreatic NET requires an in-depth understanding of the disease process and may incorporate a variety of specialists from multiple disciplines (eg, pathologists, endocrinologists, radiologists, and medical, radiation, and surgical oncologists) with expertise in the use of specific biochemical, radiologic, and surgical methods. 2,3,15 Management of localized disease Hormonal syndromes should be controlled prior to surgery. For example, glucose levels in patients with insulinomas should be stabilized with diet and/or diazoxide. Gastric hypersecretion in patients with gastrinomas should be treated with PPIs. 15 All localized pancreatic NET should be resected, if possible, because surgery is the only curative option for a pancreatic NET. 2,4,5,12 Exceptions include patients who have another medical condition that limits life expectancy or increases surgical risk 5,12 and, in some situations, patients who have MEN 1 or VHL. 5,18 Surgery for patients with MEN 1 or VHL is rarely curative since their tumors are often too small and numerous for complete surgical resection. 5,18 Recommended Surgical Approaches for Select Locoregional Tumor Types Nonfunctioning 15 Small ( 2 cm) Enucleation with/without regional nodes Distal pancreatectomy with/without regional nodes/splenectomy Pancreatoduodenectomy with/without regional nodes Observation Large (> 2 cm) Pancreatoduodenectomy with regional nodes Distal pancreatectomy with regional nodes and splenectomy Gastrinoma 15 Occult Observation Exploratory surgery (eg, intraoperative ultrasound) Duodenum Duodenectomy and intraoperative ultrasound with local resection/enucleation and periduodenal node dissection Head Enucleation and periduodenal node dissection Pancreatoduodenectomy Insulinoma 15 Distal Distal pancreatectomy with/without splenectomy Exophytic or peripheral Enucleation; consider laparoscopic resection Deep/invasive/close proximity to pancreatic duct Pancreatoduodenectomy of the head Spleen-preserving distal pancreatectomy; consider laparoscopic resection Glucagonoma 15 VIPoma 15 Pancreatoduodenectomy of the head with peripancreatic lymph nodes Distal pancreatectomy and lymph node dissection with splenectomy Pancreatoduodenectomy of the head with peripancreatic lymph nodes Distal pancreatectomy and lymph node dissection with splenectomy 16

17 Management of advanced disease Options for patients with advanced pancreatic NET include surgical resection of hepatic metastases; hepatic arterial embolization; radiofrequency ablation and cryoablation, either alone or in conjunction with cytoreductive surgery; traditional chemotherapy; and investigational approaches, including liver transplantation. 5,40 Management of Advanced Pancreatic NET Management options Comment(s) managing my condition Surgical resection of hepatic metastases In cases of malignant pancreatic NET in which 90% of the visible tumor could be removed 5,28,49 Hepatic arterial embolization/ chemoembolization Radiofrequency ablation and cryoablation, either alone or in conjunction with cytoreductive surgery When surgery is not an option, these modalities may provide palliation in patients who have hepatic metastases and an otherwise preserved performance status, disease primarily confined to the liver, and a patent portal vein 5,28,49 When surgery is not an option, these techniques may be used in carefully selected patients with advanced pancreatic NET 5,28,49 Traditional chemotherapy Targeted therapies Peptide receptor radiotherapy (PRRT) Either alone or in combination in appropriate patients with advanced progressive disease 5,28,49 May be appropriate in some newly diagnosed patients with high-volume disease; recommended in patients with advanced, progressive disease 28 Using radiolabeled somatostatin analogs, PRRT is used for inoperable or metastatic NET 5,50 Clinical studies Clinical studies may be appropriate according to each one s specific protocol and are subject to the discretion of the treating physician 28 17

18 Follow-up Disease recurrence has been reported in 21% to 42% of patients with resected pancreatic NET. As such, the NCCN Clinical Practice Guidelines In Oncology (NCCN Guidelines ) recommend that patients with localized disease should be followed up 3 to 12 months after resection and every 6 to 12 months thereafter with a physical examination, appropriate biomarkers, and structural (eg, CT, MRI) and functional imaging studies. 15 The North American Neuroendocrine Tumor Society (NANETS) also suggests follow-up with chest x-ray. 5 Patients with metastatic disease are generally followed more frequently, as dictated by their requirement for concurrent therapies. Patients who are asymptomatic with stable, low-burden advanced disease can be followed up with biomarker and imaging assessments every 3 to 12 months until clinically significant progression occurs. Conclusion Pancreatic NET are uncommon tumors that, depending on subtype and histologic features, vary in clinical presentation and aggressiveness. They are distinct from exocrine pancreatic cancer and call for different clinical approaches to diagnosis, characterization, therapeutic intervention, and ongoing surveillance. Multidisciplinary expertise may help ensure optimal patient management of pancreatic NET. Definitive diagnosis also calls for biochemical, imaging, and histopathologic assessments that span various clinical disciplines. Perhaps most importantly, early detection and identification of pancreatic NET are key to getting patients into treatment earlier in the course of disease, when therapeutic options and the potential for positive outcomes are improved. 18

19 References 1. Vortmeyer AO, Huang S, Lubensky I, Zhuang Z. Non-islet origin of pancreatic islet cell tumors. J Clin Endocrinol Metab. 2004;89(4): Ong SL, Garcea G, Pollard CA, et al. A fuller understanding of pancreatic neuroendocrine tumours combined with aggressive management improves outcome. Pancreatology. 2009;9(5): Yao JC, Eisner MP, Leary C, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol. 2007;14(12): Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 2008;135(5): Kulke MH, Anthony LB, Bushnell DL, et al. NANETS treatment guidelines: well-differentiated neuroendocrine tumors of the stomach and pancreas. Pancreas. 2010;39(6): Rindi G, Arnold R, Bosman FT, et al. Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman FT, Carniero F, Hruban RH, Theise ND, eds. WHO Classification of Tumours of the Digestive System. 4th ed. 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