Nonfunctioning Pancreatic Tumors Research Articles

Aptasensor for Quantifying Pancreatic Polypeptide

Herein, a simple colorimetric aptasensor based on unmodified gold nanoparticles (AuNPs) has been developed for quantifying pancreatic polypeptide (PP), a peptide hormone associated with nonfunctional neuroendocrine pancreatic tumor (NF-PNET). First, several PP-specific aptamers have been screened by systematic evolution of ligands by exponential enrichment. The generated aptamers showed specificity and affinity higher than those reported so far. The sensing platform was constructed based on the optical properties of AuNPs as well as the adsorption of single-stranded DNA aptamers. The high sensitivity of the developed aptasensor was achieved by optimizing the sensing conditions, including salt tolerance, time of reaction, and optimal aptamer concentration. The developed aptasensor showed a wide linear dynamic range (1–60 nM) with a limit of detection of 521 pM. Furthermore, the performance of the aptasensor was evaluated for the detection of PP in human serum and showed recovery values ranging between 92.4...

The Pancreatic Endocrine Tumors - Experience of First Surgical Clinic Iasi.

Pancreatic neuroendocrine tumors (PNETs) are rare and characterized by widely variable clinical presentation and often challenging surgical management. Methods: Retrospective study conducted during the last 15 years at the First Surgical Clinic of the Iasi St Spiridon University Hospital, and which included all the patients diagnosed with pancreatic endocrine tumors by immunohistochemistry. Results: There were 26 cases diagnosed with PNET. The male/female ratios was 7/19 and mean age 41.93 +- 2.48 years (range 20-79 years). Of the PNET cases 13 were insulinomas, 5 gastrinomas, 2 gastrinomas associated with other endocrine neoplasms (Wermer syndrome), 5 non-functional endocrine pancreatic tumors and 1 ACTHoma. Clinical manifestations depended on tumor type: hypoglycemia and Whipple triad for insulinoma, Zollinger Ellison syndrome and complicated peptic ulcer (hemorrhage, perforation) for gastrinoma, Cushing syndrome for ACTHoma. Biological diagnosis included biological markers (e.g. insulin, gastrin and cortisol). Tumor site and size at diagnosis were determined by ultrasound, CT-scan, angiography, PETscan, octreoscan and intraoperative ultrasound. Surgical procedures for PNET insulinomas were: tumor resection - 6 cases; left splenopancreatectomy - 3 cases; left spleen-preserving pancreatectomy - 2 cases; pancreaticoduodenectomy - 2 cases. We also present 4 cases of gastrinoma with multiple ulcers and multiple surgical interventions for hemorrhage and perforation with peritonitis. The two patients with Wermer syndrome also had ulcers complicated with hemorrhage and peritonitis and parathyroid adenoma. Nonfunctional pancreatic endocrine tumors were diagnosed in 5 women of which in 3 the tumors were located in the pancreatic tail (in which splenopancreatectomy and left pancreatectomy with spleen preservation were performed) and in 2 in the pancreatic head (in which pancreaticoduodenectomy and Beger type operation were performed). Conclusions: Knowledge of clinical signs of secreting tumors and exploring the patients are of crucial importance for management of PNETs. Immunohistochemistry is mandatory for confirming the diagnosis and assessing the proliferation and biological behavior of the tumor, thus facilitating the administration of specific therapy. Aggressive surgical treatment is indicated, even in advanced stages.

Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate

Distal pancreatectomy is the most frequent operation for insulinomas complicating multiple endocrine neoplasia type 1 insulinoma, although there are conditions for which a different operative approach might be preferable. In this article, we report the operative experience of a referral center for multiple endocrine neoplasia type 1 insulinoma. Twelve patients underwent operations between 1992 and 2015: 8 underwent a distal pancreatic resection, and 4 underwent a pancreatoduodenectomy. Enucleation of other macroadenomas present in the remnant pancreas was performed in 9 out of these 12 patients. Operative complications (2 pancreatic fistulas and 2 cases of pancreatitis) occurred in 4 of the 8 distal pancreatic resections. In 1 patient, reoperation was required to resolve the complications of the first operation. At pathologic analysis, multiple insulinomas were found in 5 patients, lymph-nodal metastasis positive for insulin in 2 patients, multiple nonfunctioning pancreatic tumors in all patients, glucagonoma in 4 patients, and gastrinoma in the duodenum or lymph nodes in 4 patients. All the patients were treated successfully for the hypoglycemic/hyperinsulinemic syndrome with no clinical recurrence at a mean follow-up of 85months (range 4-242months). Recurrent nonfunctioning pancreatic tumor macroadenomas in the remnant pancreas occurred in only 1 of the 12 patients, and no progression of the gastrinomas was observed. None of the patients developed diabetes mellitus. Resection of the most severely affected part of the pancreas, whether left or right, associated with enucleation of concomitant macroadenomas in the preserved pancreas is recommended for the treatment of hypoglycemic/hyperinsulinemic syndrome and to prevent malignant progression of nonfunctioning pancreatic tumors in patients with multiple endocrine neoplasia type 1. If the head of the pancreas is the most affected site and the Zollinger-Ellison syndrome is concomitant, then pancreatoduodenectomy should be preferred over distal pancreatectomy.

Predicting Metastasis in Non-Functioning Pancreatic Tumors with Reappraisal of the Tumor Size Significance: A Population Based Analysis

Predicting Metastasis in Non-Functioning Pancreatic Tumors with Reappraisal of the Tumor Size Significance: A Population Based Analysis

Double Gastrojejunocolic Fistula as a Late Complication of Pancreatic Head Resection for a Non-Functioning Pancreatic Tumor

Pancreatic head resection represents one of the most demanding procedures in abdominal surgery; however, several complications may develop after this type of surgery such as fistulas (internal and/or external), gastrointestinal tract hemorrhage, and abdominal infections. The relaparotomy rate, as well as low perioperative mortality, may be present. The reported postoperative complication rate may range from 23 to 57%, even when an expert surgeon performs the resection. However, late complications of pancreatic head resection are rare. A case of double gastrojejunocolic fistula, which developed in a patient who had undergone pancreatic head resection some years earlier, is herein reported. The patient was admitted to our hospital for abdominal pain associated with nausea andvomiting. She also complained of diarrhea after food ingestion, even in small quantities, and referred the presence of undigested food in the feces and a weight loss of 10 kg in the previous three months. The patient was treated conservatively. We should be aware that symptoms of fistulas due to pancreatic head resections may be diagnosed several years after surgery.

Pancreatic Adenocarcinoma in a Patient With Multiple Endocrine Neoplasia 1 Syndrome

To the Editor We present the rare occurrence of a concurrent pancreatic neuroendocrine tumor (pNET) and pancreatic ductal adenocarcinoma (PDAC) in a patient with multiple endocrine neoplasia 1 (MEN1) syndrome. It is important for clinicians to consider the possibility of PDAC in patients with MEN1 because aggressive early surgical intervention provides the only chance of cure. Multiple endocrine neoplasia 1 is characterized by parathyroid adenomas, pNETs, and anterior pituitary tumors.1 Multiple endocrine neoplasia 1–associated pNETs are usually slowly progressive and associated with low malignant potential. In the context of MEN1, pancreatic NET size correlates with prognosis and the presence of metastases,2 and lesions of more than 2 cm are associated with greater genetic instability and malignant behaviour.3 The European Neuroendocrine Tumour Society recommendations for management of pancreatic lesions in a patient with MEN1 state that early diagnosis and surgical excision of MEN1-related pNET improve survival, preventing or delaying the development of distant metastases.4 It is mandatory to operate on MEN1-related nonfunctioning pancreatic tumors with metastases, size of more than 2 cm, or yearly increased size of more than 0.5 cm. Management of pNETs of less than 2 cm is controversial, current recommendation being intensive surveillance to avoid repeated intervention where lesions are typically multiple and behave in an indolent fashion.

Metastatic Insulinoma Following Resection of Nonsecreting Pancreatic Islet Cell Tumor: A Case Report and Review of the Literature.

A 56-year-old woman presented to our clinic for recurrent hypoglycemia after undergoing resection of an incidentally discovered nonfunctional pancreatic endocrine tumor 6 years ago. She underwent a distal pancreatectomy and splenectomy, after which she developed diabetes and was placed on an insulin pump. Pathology showed a pancreatic endocrine neoplasm with negative islet hormone immunostains. Two years later, computed tomography scan of the abdomen showed multiple liver lesions. Biopsy of a liver lesion showed a well-differentiated neuroendocrine neoplasm, consistent with pancreatic origin. Six years later, she presented to clinic with 1.5 years of recurrent hypoglycemia. Laboratory results showed elevated proinsulin, insulin levels, and c-peptide levels during a hypoglycemic episode. Computed tomography scan of the abdomen redemonstrated multiple liver lesions. Repeated transarterial catheter chemoembolization and microwave thermal ablation controlled hypoglycemia. The unusual features of interest of this case include the transformation of nonfunctioning pancreatic endocrine tumor to a metastatic insulinoma and the occurrence of atrial flutter after octreotide for treatment.

Incidental intraoperative discovery of a pancreatic neuroendocrine tumor associated with chronic pancreatitis

Pancreatic neuroendocrine tumors are a rare entity with an incidence between 2 per million to 5 per 100 000. Association with pancreatitis (acute or chronic) is rare and is considered to be determined by the tumoral obstruction of pancreatic ducts, but sometimes occurs without any apparent relationship between them. Non-functional neuroendocrine pancreatic tumors are usually diagnosed when either very large or metastatic. Small ones are occasionally diagnosed when imagery is performed for other diagnostic reasons. Intraoperative discovery is even rarer and poses problems of differential diagnosis with other pancreatic tumors. Association with chronic pancreatitis is rare and usually due to pancreatic duct obstruction by the tumor. We describe the case of a patient with a small non-functioning neuroendocrine tumor in the pancreatic tail accidentally discovered during surgery for delayed traumatic splenic rupture associated with chronic alcoholic pancreatitis. The tumor of 1.5cm size was well differentiated and confined to the pancreas, and was resected by a distal splenopancreatectomy.ConclusionsSurgeons should be well aware of the rare possibility of a non-functional neuroendocrine tumor in the pancreas, associated with chronic pancreatitis, surgical resection being the optimal treatment for cure. Histopathology is of utmost importance to establish the correct diagnosis, grade of differentiation, malignancy and prognosis.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2114470176676003.

Can treatment using radiolabelled somatostatin analogue increase the survival rate in patients with non-functioning neuroendocrine pancreatic tumours?

The aim of the study was to assess the effectiveness of peptide receptor radionuclide therapy (PRRT) in patients with non-functioning neuroendocrine pancreatic tumours (NFPNTs) and to compare survival rates in patients with NFPNTs and in patients with other neuroendocrine tumours (NETs) treated using radiolabelled somatostatin analogue in our Department. We would like to analyze factors potentially determining the effectiveness of the therapy and also to assess the myelo- and nephrotoxicity. Fourteen patients with disseminated disease and/or inoperable NFPNT were qualified to PRRT based on positive SRS (somatostatin receptor scintigraphy). There were 5 men and 9 women, with Karnofsky's index>70%. In the whole group of patients, partial response was observed in 21.4%, stabilization of the disease in 42.9%, and progression of the disease in 35.7% of patients. Mean observation time was 19±13 months, mean time to progression was 12±9 months, and mean time to death was 16±9 months. Six patients died--four of them due to progression of the disease, two due to myocardial infarction. After PRRT we did not observe clinically significant haemotoxicity and/or nephrotoxicity. 1. Peptide receptor radionuclide therapy may be a safe and effective treatment option in patients with NFPNTs, leading to stabilization or regression of the disease in the majority of patients. 2. There is no statistically significant difference in survival rate between patients with NFPNTs and NETs of other localization treated with PRRT.

Magnetic resonance imaging versus endoscopic ultrasonography for the detection of pancreatic tumours in multiple endocrine neoplasia type 1

ObjectiveIn multiple endocrine neoplasia type 1, the main risk factor for metastases is pancreatic tumour size. We and others recommend limiting surgery to non-functioning pancreatic tumors ≥20mm or growing, based on their size measured with endoscopic ultrasonography. Because endoscopic ultrasonography is invasive, we compared endoscopic ultrasonography (EUS) to non-invasive magnetic resonance imaging (MRI) for the detection of pancreatic tumours ≥10mm in multiple endocrine neoplasia type 1 patients. MethodsA prospective study was performed in nine participating centres; 90 patients with multiple endocrine neoplasia type 1 underwent EUS and MRI with gadolinium infusion. Gastroenterologists and radiologists were blinded to the results, magnetic resonance images were reviewed centrally. ResultsEUS detected 86 tumours ≥10mm, and 48 (53.3%) patients had at least one tumour ≥10mm. MRI detected 67 tumours ≥10mm, and 46 (51.1%) patients had at least one tumour ≥10mm. EUS and MRI agreement was moderate for detection of tumours ≥10mm (Kappa coefficient=0.49), and for selection of patients with tumours ≥10mm (Kappa coefficient=0.55). EUS and MRI missed 11/24 and 4/24 lesions ≥20mm, respectively. EUS failed to identify 9/57 (15.7%) patients with pancreatic tumours ≥10mm, and MRI failed to identify 11/57 (19.3%) patients with pancreatic tumours ≥10mm. ConclusionsEUS and MRI are complementary and should be performed at initial evaluation in multiple endocrine neoplasia type 1 patients. Whether follow-up should be based on either technique or both, requires further evaluation.

Impact of CT-based diagnostic imaging on management and outcome of nonfunctioning pancreatic tumors

Sporadic malignant non-functioning pancreatic endocrine tumors (NF-PETs) are an important subset of pancreatic neoplasms. The aim of this study was to assess the impact of improved imaging on these features in a tertiary referral centre within a 20-year follow-up. From 1988 to 2009, 51 patients were treated for sporadic malignant NF-PETs. Forty-one patients who underwent tumor resection were retrospectively attributed according to the date of the initial diagnosis, group 1: 1988-1999 vs. group 2: 2000-2009. Cross-sectional imaging led to positive prediction of NF-PETs in all patients. Curative resection was achieved in 76%. Synchronous metastases were present in 56% with a positive prediction of 43%. In group 1, the mean reported CT-determined tumor size was 56 vs. 54mm in group 2 (p = 0.89). Synchronous metastases were present in 61% in group 1 vs. 57% (p = 0.99) in group 2. Metachronous metastases were recorded in 39% in group 1 vs. 43% (p = 0.84) in group 2. The mean interval from initial resection to diagnosis of metastatic disease was significantly shorter (p = 0.01) in patients from group 1 (14 vs. 61months). Cumulative 5- and 10-year survival rates were 77% and 72% in group 1 vs. a 5-year survival rate of 66% in group 2. So far, improved CT-based imaging has no impact on earlier detection of initial synchronous metastases in sporadic malignant NF-PETs, while metachronous metastases are detected earlier.

Attenuated expression of menin and p27 Kip1 in an aggressive case of multiple endocrine neoplasia type 1 (MEN1) associated with an atypical prolactinoma and a malignant pancreatic endocrine tumor

Tumors in multiple endocrine neoplasia type 1 (MEN1) are generally benign. Since information on the pathogenesis of MEN1 in malignant cases is limited, we conducted genetic analysis and compared the expression of menin, p27(Kip1)(p27)/CDKN1B and p18(Ink4C)(p18)/CDKN2C with levels in benign cases. We describe the case of a 56 year-old male with an atypical prolactinoma and malignant pancreatic neuroenocrine tumor. At age 50, he had undergone transsphenoidal surgery to remove a prolactinoma. However, the tumor relapsed twice. Histological analysis of the recurrent prolactinoma revealed the presence of prolactin, a high MIB-1 index (32.1 %), p53-positive cells (0.2%), and an unusual association with FSH-positive cells. A few years later, he was also found to have a non-functioning pancreatic tumor with probable metastasis to the extradullar region. The metastatic region tested positive for chromogranin and CD56, and negative for prolactin, with 1.2 % of cells p53-positive. Although genetic analyses of the MEN1, p27, and p18 genes demonstrated no mutation, numbers of menin, p27 and p18 immuno-positive cells were significantly down-regulated in the recurrent prolactinoma, but that of p18 was intact in the metastatic region. Furthermore, MEN1 and p27 mRNA levels of the recurrent prolactinoma were down-regulated, particularly the MEN1 mRNA level, compared to levels in 10 cases of benign prolactinoma, while the p18 mRNA level was similar to that of normal pituitary. The tumor in this case may be a subtype of MEN1 showing more aggressive and malignant features probably induced by low levels of menin and p27.

Giant Malignant Insulinoma Which Developed from a Non-Functioning Pancreatic Tumor Over a Long Period of Time

Insulinomas are the most common hormone-producing pancreatic neuroendocrine tumors (NETs), which are usually benign, solitary and small. We describe herein a patient with a giant insulinoma (>10 cm in diameter) with concomitant thyroid tumor as detected by Somatostatin receptor scintigraphy (SRS). A 50-year-old man presented hypoglycemic symptoms 20 years after the first detection of a pancreatic tumor, which was ameliorated by administration of a somatostatin analogue, octreotide. SRS showed abnormal uptake by the insulinoma as well as by the thyroid tumor. RT-PCR and immunohistochemical study revealed abundant expression of somatostatin receptor (SSTR)-1, -2, and -5 in his insulinoma and SSTR-1 and -2 in his thyroid follicular neoplasm. This is a rare case of a slow-growing pancreatic well-differentiated neuroendocrine carcinoma over a long period of time to become a symptomatic giant insulinoma. Furthermore, SRS proves to be a useful tool for localization of insulinoma as well as concomitant thyroid neoplasm with predominant expression of SSTRs.

Risk Factors and Causes of Death in MEN1 Disease. A GTE (Groupe d’Etude des Tumeurs Endocrines) Cohort Study Among 758 Patients

The natural history of multiple endocrine neoplasia type 1 (MEN1) is known through single-institution or single-family studies. We aimed to analyze the risk factors and causes of death in a large cohort of MEN1 patients. Overall, 758 symptomatic MEN1 patients were identified through the GTE network (Groupe d'étude des Tumeurs Endocrines), which involves French and Belgian genetics laboratories responsible for MEN1 diagnosis and 80 clinical reference centers. The causes of death were analyzed. A frailty model, including time-dependent variables, was used to assess the impact of each clinical lesion, except for hyperparathyroidism, on survival. The median follow-up was 6.3 years. Female gender, family history of MEN1, and recent diagnosis were associated with a lower risk of death. Compared with nonaffected patients, those with thymic tumors (hazard ratio [HR] = 4.64, 95% CI = 1.73-12.41), glucagonomas-vipomas-somatostatinomas (HR = 4.29, 95% CI = 1.54-11.93), nonfunctioning pancreatic tumors (HR = 3.43, 95% CI = 1.71-6.88), and gastrinoma (HR = 1.89, 95% CI = 1.09-3.25) had a higher risk of death after adjustment for age, gender, and diagnosis period. The increased risk of death among patients with adrenal tumors was not significant, but three patients died from aggressive adrenal tumors. Pituitary tumors, insulinomas, and bronchial tumors did not increase the risk of death. The proportion of MEN1-related deaths decreased from 76.8 to 71.4% after 1990. The prognosis of MEN1 disease has improved since 1980. Thymic tumors and duodenopancreatic tumors, including nonsecreting pancreatic tumors, increased the risk of death. Rare but aggressive adrenal tumors may also cause death. Most deaths were related to MEN1. New recommendations on abdominal and thoracic imaging are required.

Treatment and prognosis of nonfunctional pancreatic endocrine tumor

Imaging techniques of nonfunctional pancreatic endocrine tumors (NFPET) including ultra-sonography, CT, magnetic resonance imaging and endoscopic ultrasonography (EUS). EUS is more sensitive at finding pancreatic head tumor. Surgery is the only treatment modality with the potential of cure. Tumors should be resected if volum is sporadic or more than 2 cm in multiple endocrine neoplasia Ⅰ. The treatment for/iver metastases include liver resection,chemoembolisation and Liver transplantation. Compared with pancreatic canc-er, patients with completely resected tumors have good prognosis. Key words: Multiple endocrine neoplasm type Ⅰ ; Pancreatic neoplasms; Diagnosis; Therapy; Prog-nosis

Surgical management of pancreatic endocrine tumors

Pancreatic endocrine tumors (PETs) are uncommon but clinically challenging and fascinating tumors with an annual incidence of 1 per 100,000 people. PETs present as either functional pancreatic tumors or as nonfunctional pancreatic tumors. Functional tumors are commonly associated with a specific hormonal syndrome directly related to a hormone secreted by the tumor, such as Zollinger-Ellison syndrome or organic hyperinsulinism. Nonfunctional tumors do not secrete a hormone resulting in a pathologic syndrome of clinical symptoms. The natural history of PETs is highly variable. Small, benign neoplasms, such as 90% of all insulinomas, are readily curable by surgical resection; however, most other functional and all nonfunctional pancreatic tumors have a much less favorable prognosis. Patients with completely resected tumors generally have a good prognosis, and an aggressive surgical approach in patients with advanced disease may also prolong survival. Many features of the management of pancreatic endocrine tumors, such as timing and extent of resection, and the use of laparoscopic procedures, are currently under debate. This Review describes the current status of surgical treatment for pancreatic endocrine tumors, and discusses the new developments in this field.

A Case of Multiple Endocrine Neoplasia Type I with Atypical Clinical Course

Multiple endocrine neoplasia type 1 (MEN 1) is characterized by the combined occurrence of primary hyperparathyroidism, enteropancreatic tumors and anterior pituitary adenoma. Yet carcinoid tumors, adrenal adenoma and lipoma might exist simultaneously. Thymic carcinoid tumors, which are recognized as one of the causes of death for patients with MEN 1, are uncommon and their natural history has barely been investigated. A 51-year-old man, who had undergone surgery and radiation therapy for thymic carcinoid tumor 15 years ago, was admitted to our hospital due to hypercalcemia that was detected on a routine health checkup. We found that he had hyperparathyroidism, a nonfunctioning pancreatic tumor and thyroid papillary microcarcinoma. We had studied the genetic alteration of the MEN 1 gene (menin) by using leukocyte genomic DNA. This genetic analysis clearly showed the missense mutation of the MEN 1 gene (V215M on exon 3). The thymic carcinoid tumor in this patient showed a relatively indolent course, in contrast to that for the ordinary MEN 1 patient, and this patient's thyroid papillary microcarcinoma was discovered incidentally. We present here this atypical case along with a review of the relevant literature. (J Korean Endocr Soc 23:266~271, 2008)

Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype–phenotype correlation

The existence of genotype-phenotype correlation in multiple endocrine neoplasia type 1 (MEN1) is controversial. Two founder mutations of the MEN1 gene in Northern Finland gave us an opportunity to compare clinical features among heterozygotes of different mutations. Study cohort included 82 MEN1 heterozygotes who were tested for MEN1 during the years 1982-2001. Medical records were reviewed for manifestations of MEN1, other tumours and cause of death by the end of August 2003. Logistic regression analysis was used in evaluating the impact of age, gender and mutational status of affected heterozygotes on the likelihood of developing manifestations of MEN1. Founder mutations 1466del12 and 1657insC were found in 39 and 29 individuals, and D418N, G156R and R527X mutations in 9, 3 and 2 individuals respectively. Except for pituitary adenoma and nonfunctional pancreatic tumour (NFPT), age was a risk factor for all the disease manifestations. For NFPT, frameshift/nonsense mutations (1657insC, R527X) gave an odds ratio (OR) of 3.26 (95% confidence intervals (CI), 1.27-8.33; P = 0.014) compared with in-frame/missense mutations (1466del12, D418N, G156R); including the founder mutation carriers (n = 68) only, the 1657insC mutation gave an OR of 3.56 (CI, 1.29-9.83; P = 0.015). For gastrinoma, in-frame/missense mutations predicted the risk with an OR of 6.77 (CI, 1.31-35.0; P = 0.022), and in the founder mutations group the 1466del12 mutation gave an OR of 15.09 (CI, 1.73-131.9, P = 0.014). In this study population, NFPT was more common in the frameshift/nonsense or 1657insC mutation carriers, whereas gastrinoma was more common in the in-frame/missense or 1466del12 mutation carriers.

Patterns of care and outcome of patients with gastroenteropancreatic neuroendocrine tumors (GNETs): Results from a Spanish multi-center hospital-based tumor registry

15110 Background: GNET represent an unusual family of neoplasms with a wide and complex spectrum of clinical behavior. To provide information regarding demographic characteristics, diagnostic procedures, tumor features, therapeutic interventions and outcomes of pts with GNETs, a national tumor registry was launched. We present here the results of this broad-based multi-institutional observational registry that comprises 41 sites representing all regions of Spain. Methods: Data was provided at www.retegep.net by participating centers and assessed for internal consistency by external independent reviewers. Results: The study cohort comprised 474 pts (57% male, median age: 59 years). 52% were carcinoids (C), 18% non-functioning pancreatic tumors (PT), 9% insulinomas and 6% gastrinomas. Most common primary tumor sites included midgut (19%), and head (14%) and body of the pancreas (9%). 10% were of unknown primary. Functioning symptoms led to diagnosis in 29% of cases. 5% were associated to MEN1. Diagnostic workup included serum hormone tests (60%), ultrasound (40%), CT scan (75%) and octreoscan (37%). 32% of pts presented with localized tumors (L), 14% with local-regional spread (LR) and 44% with advanced disease (Adv). Treatment for Adv disease consisted of somatostatin analogues (SA) (48%), surgery (45%), chemotherapy (CT) (42%), interferon (IFN) (23%), chemoembolization (9%) and radiofrequency ablation (5%). 5-year survival (5yS) for the whole group was 61.1% (SD:3.4%), 78.2% (SD:5.4%) for pts with L/LR disease and 47.1% (SD:5.6%) for Adv stages. 5yS rates were similar for C and PT tumors, also when stratifying according to tumor stage (84 and 76% for local-regional disease, and 51 and 49% for Adv disease). 1st line systemic therapy (n=175) consisted of CT (42%), SA (39%), IFN (5%), SA+IFN (10%) and SA+CT (3%), with 5yS rates of 59%, 36%, 42%, 46% and 38%, respectively (p=0.7). As second line systemic treatment (n=65) 45% received CT, 26% SA, 15% immunotherapy and 14% different drug combinations. Conclusions: This national database reveals relevant information regarding current Spanish practices and will provide valuable insights into the epidemiology and management of this heterogeneous uncommon disease No significant financial relationships to disclose.

Surgery on neuroendocrine tumours

Neuroendocrine tumours of the gastrointestinal tract and pancreas present a major challenge to physicians in their recognition and treatment requirements, and surgical treatment of these tumours has become increasingly important for symptom palliation and survival. For some carcinoid tumours the extent of surgery may depend on tumour size. Midgut carcinoid is the most common cause of the carcinoid syndrome, requiring surgery for primary and mesenteric tumours to minimize the risk for abdominal complications but also for removal of liver metastases to palliate hormonal symptoms. Among endocrine pancreatic tumours, insulinoma and gastrinoma often cause severe symptoms of hormone excess despite their inconspicuous size, but they can be successfully removed with improved pre- and intraoperative localization. Other tumours--glucagonoma, VIPoma, and non-functioning endocrine pancreatic tumours--are often large or metastasizing, but generally require surgical debulking to alleviate hormonal symptoms and have favourable survival.