Postoperative Outcomes of Enucleation and Standard Resections in Patients with a Pancreatic Neuroendocrine Tumor.

BACKGROUND: Either enucleation or more extended resection is performed to treat patients with pancreatic neuroendocrine tumor (pNET). Aim was to analyze the postoperative complications for each operation separately. Furthermore, independent risk factors for complications and incidence of pancreatic insufficiency were analyzed. METHODS: Retrospective all resected patients from two academic hospitals in The Netherlands between 1992 and 2013 were included. Postoperative complications were scored by both ISGPS and Clavien-Dindo criteria. Based on tumor location, operations were compared. Independent risk factors for overall complications were identified. During long-term follow-up, pancreatic insufficiency and recurrent disease were analyzed. RESULTS: Tumor enucleation was performed in 60/205 patients (29%), pancreatoduodenectomy in 65/205 (31%), distal pancreatectomy in 72/205 (35%) and central pancreatectomy in 8/205 (4%) patients. Overall complications after tumor enucleation of the pancreatic head and pancreatoduodenectomy were comparable, 24/35 (69%) versus 52/65 (80%). The same was found after tumor enucleation and resection of the pancreatic tail (36 vs.58%). Number of re-interventions and readmissions were comparable between all operations. After pancreatoduodenectomy, 33/65 patients had lymph node metastasis and in patients with tumor size ≤2 cm, 55% had lymph node metastasis. Tumor in the head and BMI ≥25 kg/m(2) were independent risk factors for complications after enucleation. During follow-up, incidence of exocrine and endocrine insufficiency was significant higher after pancreatoduodenectomy (resp. 55 and 19%) compared to the tumor enucleation and distal pancreatectomy (resp. 5 and 7% vs. 8 and 13%). After tumor enucleation 19% developed recurrent disease. CONCLUSION: Since the complication rate, need for re-interventions and readmissions were comparable for all resections, tumor enucleation may be regarded as high risk. Appropriate operation should be based on tumor size, location, and functional status of the pNET.

Postoperative Complications, In-Hospital Mortality and 5-Year Survival After Surgical Resection for Patients with a Pancreatic Neuroendocrine Tumor: A Systematic Review.

Studies on postoperative complications and survival in patients with pancreatic neuroendocrine tumors (pNET) are sparse and randomized controlled trials are not available. We reviewed all studies on postoperative complications and survival after resection of pNET. A systematic search was performed in the Cochrane Central Register of Controlled Trials, MEDLINE and EMBASE from 2000-2013. Inclusion criteria were studies of resected pNET, which described postoperative complications separately for each surgical procedure and/or 5-year survival after resection. Prospective and retrospective studies were pooled separately and overall pooled if heterogeneity was below 75%. The random-effect model was used. Overall, 2643 studies were identified and after full-text analysis 62 studies were included. Pancreatic fistula (PF) rate of the prospective studies after tumor enucleation was 45%; PF-rates after distal pancreatectomy, pancreatoduodenectomy, or central pancreatectomy were, respectively, 14-14-58%. Delayed gastric emptying rates were, respectively, 5-5-18-16%. Postoperative hemorrhage rates were, respectively, 6-1-7-4%. In-hospital mortality rates were, respectively, 3-4-6-4%. The 5-year overall survival (OS) and disease-specific survival (DSS) of resected pNET without synchronous resected liver metastases were, respectively, 85-93%. Heterogeneity between included studies on 5-year OS in patients with synchronous resected liver metastases was too high to pool all studies. The 5-year DSS in patients with liver metastases was 80%. Morbidity after pancreatic resection for pNET was mainly caused by PF. Liver resection in patients with liver metastases seems to have a positive effect on DSS. To reduce heterogeneity, ISGPS criteria and uniform patient groups should be used in the analysis of postoperative outcome and survival.

Management of Islet Cell Tumours: A Single Hospital Experience.

BACKGROUND/AIMS: Islet cell tumours (ICTs) are uncommon tumours in clinical practice. Surgical resection is the treatment of choice for ICTs, but localisation of these lesions can be challenging. The aim of this study was to analyse the clinical diagnosis and treatment for ICTs. METHODOLOGY: Thirty-one patients with ICTs who were diagnosed and who underwent surgical treatment in the affiliate hospital of Luzhou Medical College from 1 January 2000 to 31 July 2013 were enrolled. The clinical data of these patients were retrospectively reviewed. RESULTS: Among 31 patients (6 males, 25 females), 15 cases (48.39%) had non-functional ICTs and 16 (51.61%) cases were insulinoma: The mean age of patients with non-functional ICTs was 42.73 ± 12.34 years and of those with insulinoma was 48.88 ± 13 years. Non-functional ICTs had a non-specific presentation. Insulinoma makes different clinical presentations mostly with symptoms of hypoglycaemia. CONCLUSIONS: Preoperative and/or intra-operative localisation is needed for ICTs; CT scan or MRI is used routinely as the first choice. If the lesion is very small, DSA is also good for localisation before operation. IOUS is a reliable technique in exactly localising insulinoma. ICTs are considered to be cured with successful surgical removal.

Management of Islet Cell Tumours: A Single Hospital Experience.

BACKGROUND/AIMS: Islet cell tumours (ICTs) are uncommon tumours in clinical practice. Surgical resection is the treatment of choice for ICTs, but localisation of these lesions can be challenging. The aim of this study was to analyse the clinical diagnosis and treatment for ICTs. METHODS: Thirty-one patients with ICTs who were diagnosed and who underwent surgical treatment in the affiliate hospital of Luzhou Medical College from 1 January 2000 to 31 July 2013 were enrolled. The clinical data of these patients were retrospectively reviewed. RESULTS: Among 31 patients (6 males, 25 females), 15 cases (48.39%) had non-functional ICTs and 16 (51.61%) cases were insulinoma. The mean age of patients with non-functional ICTs was 42.73 ± 12.34 years and of those with insulinoma was 48.88 ± 13 years. Non-functional ICTs had a non-specific presentation. Insulinoma makes different clinical presentations mostly with symptoms of hypoglycaemia. CONCLUSIONS: Preoperative and/or intra-operative localisation is needed for ICTs; CT scan or MRI is used routinely as the first choice. If the lesion is very small, DSA is also good for localisation before operation. IOUS is a reliable technique in exactly localising insulinoma. ICTs are considered to be cured with successful surgical removal.

[Diagnosis and treatment of 51 patients with pancreatic islet cell tumors].

OBJECTIVE: To investigate the diagnosis and treatment of pancreatic islet cell tumors. METHODS: Fifty-one patients with islet cell tumors treated in our department from January 1991 to April 2011 were included in this study. The data of clinical features, diagnosis and treatment were retrospectively analyzed. RESULTS: Among the 51 cases, 38 cases showed typical Whipple's triad, and the other 13 cases were non-functional islet cell tumors. In these 13 cases, 5 patients had no specific clinical symptoms, and 8 patients had abdominal distending pain. The positive rates of imaging were: B-ultrasound 43.1%, multi-slice spiral CT 69.8%; MRI 62.5%, endoscopic ultrasonography (EUS) 64.7% (11/17), and intraoperative ultrasound (IOUS) 96.3%, the differences among them were statistically significant (P<0.05). All patients underwent surgical treatment. Postoperative pancreatic leakage happened in 6 cases. Finally all the patients recovered after effective external drainage, anti-infection treatment and nutritional support. CONCLUSIONS: Intraoperative ultrasonography (IOUS) has a higher accuracy in the diagnosis of pancreatic islet cell tumors, compared with preoperative B-ultrasonography, CT, MRI, and endoscopic ultrasound (EUS). The most effective treatment of this disease is surgery.

Pancreatic neuroendocrine tumors: radiographic calcifications correlate with grade and metastasis.

BACKGROUND: Studies to identify preoperative prognostic variables for pancreatic neuroendocrine tumor (PNET) have been inconclusive. Specifically, the prevalence and prognostic significance of radiographic calcifications in these tumors remains unclear. METHODS: From 1998 to 2009, a total of 110 patients with well-differentiated PNET underwent surgical resection at our institution. Synchronous liver metastases present in 31 patients (28%) were addressed surgically with curative intent. Patients with high-grade PNET were excluded. The presence of calcifications in the primary tumor on preoperative computed tomography was recorded and correlated with clinicopathologic variables and overall survival. RESULTS: Calcifications were present in 16% of patients and were more common in gastrinomas and glucagonomas (50%), but never encountered in insulinomas. Calcified tumors were larger (median size 4.5 vs. 2.3 cm, P=0.04) and more commonly associated with lymph node metastasis (75 vs. 35%, P=0.01), synchronous liver metastasis (62 vs. 21%, P<0.01), and intermediate tumor grade (80 vs. 31%, P<0.01). On multivariate analysis of factors available preoperatively, calcifications (P=0.01) and size (P<0.01) remained independent predictors of lymph node metastasis. Overall survival after resection was significantly worse in the presence of synchronous liver metastasis (5-year, 64 vs. 86%, P=0.04), but not in the presence of radiographic calcifications. CONCLUSIONS: Calcifications on preoperative computed tomography correlate with intermediate grade and lymph node metastasis in well-differentiated PNET. This information is available preoperatively and supports the routine dissection of regional lymph nodes through formal pancreatectomy rather than enucleation in calcified PNET.

Early experience for the robotic duodenum-preserving pancreatic head resection.

BACKGROUND: The duodenum-preserving pancreatic head resection (DPPHR) has been accepted as a valid surgical alternative to more extensive standard resections for the treatment of benign and low malignant tumors at the head of the pancreas. In this article, a new minimally invasive operation, the robot-assisted laparoscopic technique, is introduced for this procedure. METHODS: From March 2010 to Dec 2010, four patients (three women and one man), with a mean age of 42.3 years (range: 21-62 years), underwent robot-assisted laparoscopic DPPHR at the Hepato-Bilio-Pancreatic Surgical Department of Rui Jin Hospital in Shanghai, China. The preoperative symptoms include two cases of repeated upper abdominal pain, one case with no obvious preoperative symptoms, and one case of repeated hypoglycemia. The da Vinci Surgical System was used to perform the main steps of the operation. All patients underwent a pancreaticogastrostomy for pancreaticoenteric reconstruction to the distal stump. RESULTS: All four surgeries were successfully performed. There were no deaths. The mean operative time was 298.8 (270-335) min, average blood loss was 425 ml (range: 100-600 ml). The mean postoperative hospital stay was 26.8 days (range: 20-30 days). The one patient with an islet cell tumor has had normal blood glucose levels since the operation, and the other three patients have had no hyperglycemia. Three of the patients developed a pancreatic fistula that was cured by conservative treatment. CONCLUSIONS: The robotic surgical system is technically fully capable of performing the complex DPPHR procedure with an acceptable range of surgical complications. It breaks through the bottleneck of the traditional laparoscopic technology and expands the range of its applications. However, this new technology is still at an exploratory stage, and the long-term effect remains to be validated by additional clinical data.

[Importance of surgery in distant metastatic pancreatic neuroendocrine neoplasms]. / Stellenwert der Chirurgie bei fernmetastasierten neuroendokrinen Neoplasien des Pankreas.

The majority of patients with pancreatic neuroendocrine neoplasms (pNEN) already present with distant metastases at diagnosis. The heterogeneity of pNEN and the broad spectrum of treatment options make adequate patient selection and an evidence-based strategy essential. In metastatic pNEN both primary resection and resection of liver metastases have been shown to improve overall survival. Surgical treatment of liver metastases can also be carried out with palliative intent, especially for symptomatic pNEN and can have a positive effect on disease-free survival and overall survival. Classical hepatectomy techniques and innovative techniques (two-stage resections, liver transplantation) are available to the surgeon. In complex growth types of liver metastases, there is increasing evidence for a combination of surgery and ablative methods. Due to a relevant risk of recurrence following liver resection, pNEN patients need to be included in multimodal treatment concepts. Current areas of interest in the treatment of metastatic pNEN are the use of adjuvant/neoadjuvant chemotherapy and surgery in G3-NEN and G3-NEC patients. The aim of this review is to give an overview on the impact of surgery in the situation of distant metastatic NEN of the pancreas.

[Clinical applications of laparoscopic pancreatectomy].

OBJECTIVE: To summarize the experiences of laparoscopic pancreatectomy and to discuss its clinical indications and operative techniques. METHODS: From March 2003 to February 2010, 49 patients with pancreatic diseases, including 18 males and 31 females with a mean age of (42 ± 14) years old (range: 21 - 77), underwent laparoscopy at our department. The preoperative diagnoses included insulinoma (n = 38), cystadenoma (n = 4), cystadenocarcinoma (n = 1), nonfunctional islet cell tumor (n = 5) and solid-pseudopapillary tumor (n = 1). RESULTS: Thirty-nine cases underwent total laparoscopic surgery and 8 cases converted to open surgery. The operative duration was (245 ± 146) minutes (range: 90 - 960). The mean intraoperative blood loss was (191 ± 306) ml (range: 20 - 1500). Secondary operations were needed in 3 cases and pancreatic fistula occurred in 7 cases. The postoperative hospital stay was (10 ± 7) days (range: 4 - 31). CONCLUSION: Laparoscopic pancreatectomy is safe and feasible in the treatment of most benign pancreatic diseases and some malignant tumors.

Central pancreatectomy for benign tumors of the neck and body of the pancreas: report of eight cases.

BACKGROUND: To discuss the advantage of central pancreatectomy in the patients with benign tumors of the neck and body of the pancreas. METHODS: Eight patients from January 1990 to December 2007 were retrospectively analyzed in our hospital. The operation is carried out by exposition of the pancreatic neck and body involved by the lesion. Thereafter, the lesion is dissected from the spleen artery and porto-mesenteric vein. The cephalic stump is sutured, and the distal stump is anastomosed by an end-to-end invaginated pancreaticojejunostomy with double-layer suture. RESULTS: Central pancreatectomy was done in eight patients including five mucinous cystadenomas, one serious cystadenoma, one insulinoma and one nonfunctional islet cell tumor. No mortality rate developed and morbidity rate was 37.5%. Pancreatic fistula occurred in three cases and was treated conservatively. CONCLUSION: Central pancreatectomy is a safe technique for benign tumors of the pancreatic neck and body, especially when the enucleation is very difficult.

Central pancreatectomy: a new technique for resection of selected pancreatic tumors.

BACKGROUND: Pancreatic tumors located in the neck region usually require pancreaticoduodenectomy or splenopancreatectomy. For small benign tumors enucleation is not usually feasible due to their size and localization; then pancreatectomy is often needed. Central pancreatectomy consists of a limited resection of the midportion of the pancreas and can be offered in benign and low-grade malignant tumors of the neck of the pancreas. The study aimed to evaluate whether central pancreatectomy has a place in pancreatic surgery. METHODS: In this study, which covered a period of 14 months, we performed central pancreatectomy in four selected patients. Preoperative evaluation and operative frozen section biopsy in indicated cases allowed proper selection for the procedure. Operative details, complications and follow-up were recorded. RESULTS: Four patients, two with serous cystadenoma, and one with an islet cell tumor, and one with a hydatid cyst, were identified for the procedure. The mean tumor size was 3 cm, the mean operative time was 217.5 minutes, and the mean blood loss was 382.5 ml. There was no morbidity or mortality in this series. No endocrine or exocrine deficiency was observed in any patient during a mean follow-up of 22.7 months. CONCLUSIONS: Central pancreatectomy is a procedure that offers excellent results in benign and low-grade malignant tumors. It preserves functional elements (endocrine and exocrine) of the pancreas and also eliminates the infective and hematological effects of splenectomy. Thus, central pancreatectomy should be included in the armamentarium of pancreatic surgery, and in order to obtain good results, proper indications and adequate experience are recommended.

[Diagnosis and surgical treatment for non-functional islet cell tumor: a retrospective analysis of 44 cases].

OBJECTIVE: To evaluate the methods of diagnosis and surgical treatment for nonfunctional islet cell tumor (NICT). METHODS: Forty-four patients with non-functional islet cell tumor treated at the First Affiliated Hospital of Nanjing Medical University during January 1968 to June 2008 were analyzed retrospectively. There were 9 males and 35 females, aged from 7- to 70-years-old. Clinical manifestation: 15 cases (34.1%) of abdominal masses, 17 patients (38.6%) with epigastric or back pain, 5 cases of jaundice, 5 cases (11.4%) for upper abdominal fullness or vomiting, 10 cases (22.7%) of pancreatic tumor noticed by routine health checkups or imaging examinations. Imaging examination: CT scan, sonography, ERCP, MRI, upper GI series were performed in 33 (75.0%), 16 (36.4%), 6 (13.6%), 2 (4.5%), and 10 cases (22.7%) respectively. Operation methods: 39 patients (88.6%) underwent surgical resection and the other 5 patients did not. COMPLICATIONS: pancreatic fistula in 7 patients (15.9%), intra-abdominal bleeding in 4 (9.1%), gastrojejunal anastomosis outlet obstruction in 1 (2.3%), biliary fistula in 2 (4.5%) and incisional infection in 3 (6.8%). Surgery related mortality happened in 2 patients (4.5%), both treated before 1999. Twenty-five patients underwent operation between January 1999 and June 2008 were followed up for 6 to 108 months. All survive except one died 75 months after the surgery for unknown reason. CONCLUSIONS: No specific clinical manifestation is recognized for non-functional islet cell tumor. Spiral CT is an optimal diagnostic method, while surgery is the first choice for treatment. Middle segmental pancreatectomy has become an alternative surgical protocol for NICT.

Role of medial pancreatectomy in the management of intraductal papillary mucinous neoplasms and islet cell tumors of the pancreatic neck and body.

BACKGROUND/AIM: Medial pancreatectomy has been applied as a safe and effective alternative in benign diseases located in the pancreatic neck or body. However, the role of this procedure remains controversial. We investigate outcomes using this method in 14 patients. METHODS: Fourteen consecutive patients underwent medial pancreatectomy. The surgical indications, postoperative outcomes, and pathologic characteristics were retrospectively analyzed. RESULTS: Among 10 patients with intraductal papillary mucinous neoplasms, 3 patients had minimally invasive adenocarcinoma, and 3 had adenocarcinoma in situ. Four patients required an additional resection of the pancreatic remnant because of a positive surgical margin. A medial pancreatectomy was converted to a distal pancreatectomy in 1 patient with adenocarcinoma in situ. Three patients with islet cell tumor and 1 patient with solid pseudopapillary tumor had no malignant disease. Postoperative complications occurred in 6 patients (43%): 5 had pancreatic fistulas and 1 had a gastric ulcer. All of the patients are doing well without recurrence. CONCLUSIONS: A medial pancreatectomy is a safe and effective alternative for the treatment of intraductal papillary mucinous neoplasm, islet cell tumor, or solid pseudopapillary tumor located in the neck or body of the pancreas. However, a precise preoperative and intraoperative assessment including an examination of frozen sections is mandatory to select the appropriate surgical procedure.

Laparoscopic vs open resection of pancreatic endocrine neoplasms: single institution's experience over 14 years.

BACKGROUND: Laparoscopic resection of benign pancreatic endocrine neoplasms (PENs) has become the standard of care for tumors in the pancreatic tail. Over a 14-year period, we have resected both benign and malignant tumors of the entire pancreas laparoscopically and compared our survival and complication rates with open controls. MATERIALS AND METHODS: We collected our data retrospectively and reviewed our outcomes with an actuarial 5-year survival according to Kaplan-Meier. Patients who underwent minimally invasive techniques were compared to patients who were approached with open techniques. RESULTS: From April 1992 to September 2006, we operated on 31 patients for PENs: 13 (42%) were operated on using open techniques and 18 (58%) laparoscopically, and conversion occurred in one patient (6%). In the laparoscopic group, eight (47%) tumors were malignant compared to six (43%) in the open group. Operative times averaged 188 min for the minimally invasive approach and 305 min for the open approach (p = 0.02). Length of stay was 25 days (range 8-82) for the laparoscopic group compared to 20 days (range 6-63; p > 0.05). Overall morbidity and fistula rates ranged from 67 to 24% in the laparoscopic group to 69 to 38% in the open group (p > 0.05). There were no postoperative mortalities. The average follow-up was 63 months for the open group and 33 months for the laparoscopic group. The overall actuarial survival rates were both 90% at 5 years. CONCLUSIONS: Laparoscopic resection of benign and malignant PENs has similar overall complication and 5-year survival rates as the open technique; however, the laparoscopic approach is associated with shorter operative times.

[Laparoscopic resection of pancreatic islet cell tumors].

OBJECTIVE: To summarize the surgical technique and clinical experience of total laparoscopic resection of the pancreatic islet cell tumors. METHODS: From July 2002 to December 2007, 30 cases including 12 males and 18 females were diagnosed as pancreatic islet cell tumor. There were at least one positive imaging examination of each patient preoperatively. The location of the tumors included 4 in proximal pancreas and 26 in distal pancreas. RESULTS: The tumors were successfully found and removed in 28 cases and unsuccessfully located in the other 2 cases. The procedures included local resections in 13 cases and distal pancreatectomies in 15 cases (spleen reserved in 7 cases). The mean operation time was 165 min (range, 65 - 465 min). The mean blood loss was 145 ml (range, 50 - 800 ml). Pancreatic leakage occurred in 3 cases, 2 of which were cured conservatively. And the other one were cured by endoscopic retrograde cannulation of the pancreatic duct. The mean postoperative hospital stay was 5.6 days (range, 2 - 17 d). There were no conversions and death. After follow up of (14.3 +/- 16.7) months (range, 4 - 62 months), there were no recurrences. CONCLUSION: Total laparoscopic resection is a safe and effective method for pancreatic islet cell tumors.

Somatotroph hyperplasia. Successful treatment of acromegaly by removal of a pancreatic islet tumor secreting a growth hormone-releasing factor.

A 21-yr-old woman with Turner's syndrome presented with signs and symptoms of acromegaly. The serum growth hormone (GH) (95+/-9.4 ng/ml; mean+/-SEM) and somatomedin C (11 U/ml) levels were elevated, and an increase in GH levels after glucose instead of normal suppression, increase after thyrotropin-releasing hormone (TRH) administration instead of no change, and decrease after dopamine administration instead of stimulation were observed. The pituitary fossa volume was greater than normal (1,440 mm(3)) and the presence of a pituitary tumor was assumed. After tissue removal at transsphenoidal surgery, histological study revealed somatotroph hyperplasia rather than a discrete adenoma. Postoperatively, she remained clinically acromegalic and continued to show increased GH and somatomedin levels. A search was made for ectopic source of a growth hormone-releasing factor (GRF). Computer tomographic scan revealed a 5-cm Diam tumor in the tail of the pancreas. Following removal of this tumor, serum GH fell from 70 to 3 ng/ml over 2 h, and remained low for the subsequent 5 mo. Serum somatomedin C levels fell from 7.2 to normal by 6 wk postoperatively. There were no longer paradoxical GH responses to glucose, TRH, and dopamine. Both the medium that held the tumor cells at surgery and extracts of the tumor contained a peptide with GRF activity. The GRF contained in the tumor extract coeluted on Sephadex G-50 chromatography with rat hypothalamic GH-releasing activity. Stimulation of GH from rat somatotrophs in vitro was achieved at the nanomolar range, using the tumor extract. The patient's course demonstrates the importance of careful interpretation of pituitary histology. Elevated serum GH and somatomedin C levels in a patient with an enlarged sella turcica and the characteristic responses seen in acromegaly to TRH, dopamine, and glucose do not occur exclusively in patients with discrete pituitary tumors and acromegaly. This condition can also occur with somatotroph hyperplasia and then revert to normal after removal of the GRF source. Thus, in patients with acromegaly a consideration of ectopic GRF secretion should be made, and therefore, careful pituitary histology is mandatory. Consideration for chest and abdominal computer tomographic scans before pituitary surgery, in spite of their low yield, may be justified.

Clinicopathologic features and treatment trends of pancreatic neuroendocrine tumors: analysis of 9,821 patients.

The natural history of pancreatic neuroendocrine tumors (PNET) remains poorly defined. Our objectives were to examine the clinicopathologic features of PNETs, to assess treatment trends over time, and to identify factors associated with undergoing resection. From the National Cancer Data Base (1985-2004), 9,821 patients were identified with PNETs. Clinicopathologic features and treatment trends were examined. Multivariable logistic regression was used to assess factors associated with undergoing resection. Of 9,821 patients with PNETs, 85% were nonfunctional, 7.1% were functional, and 7.9% were carcinoid tumors. Of the 3,851 (39.0%) patients who underwent pancreatectomy, 449 (11.7%) received adjuvant chemotherapy, and 254 (6.6%) received adjuvant radiation. From 1985 to 2004, utilization of pancreatectomy increased from 39.4 to 44.3% (P < 0.0001). Patients were less likely to undergo resection if they were > 55 years old, had tumors in the head of the pancreas, tumors > or = 4 cm, or had distant metastases (P < 0.0001). Patients treated at NCCN/NCI, academic, or high-volume hospitals were more likely to undergo resection. There are disparities in the utilization of pancreatectomy for PNETs. As PNETs have a better prognosis than adenocarcinoma, concerns regarding the morbidity and mortality of pancreatic surgery and neoplasms should not preclude resection.

[Insulinoma]. / Wyspiak wydzielajacy insuline.

Insulinoma is considered the most common endocrine tumour of the pancreas with an annual prevalence of 4 cases per million people. Contrary to the other endocrine tumours of this organ, over 90% of the insulinomas are benign in nature. The clinical presentation of this neoplasm depends on excessive production of insulin and pro-insulin and is characterised by the symptoms of neuroglycopenia and catecholamine response. Effective management requires directed biochemical testing, careful choice of preoperative imaging tests, and complete pancreatic exploration by an experienced endocrine surgeon utilising intraoperative ultrasound. The only curative treatment for insulinoma is complete resection of the tumour. The aim of this paper is to critically discuss contemporary diagnosis and treatment of this neoplasm on the basis of progress made in recent years.

Insulinoma / Insulinoma

RESUMEN El insulinoma es un tumor poco frecuente de las células de los islotes pancreáticos, caracterizado por la proliferación de las células betas. Son tumores neuroendocrinos pancreáticos, generalmente benignos y de pequeño tamaño, caracterizados por el síndrome de hipersecreción de insulina con el desarrollo de hipoglucemia. La mayoría de estos tumores son esporádicos, solo alrededor de 15-30 % es hereditario; está asociado a enfermedades como la neoplasia endocrina múltiple tipo 1, o el síndrome de Von Hippel-Lindau. La incidencia de dicho tumor es de 3-10 casos por cada millón de habitantes. La edad media de aparición es entre 40-50 años de edad y es más frecuente en mujeres. El reconocimiento temprano y el diagnóstico adecuado de este tumor pancreático raro, son aspectos importantes pues es potencialmente curable con un tratamiento quirúrgico oportuno.

ABSTRACT Insulinoma is a rare pancreatic islet cell tumor characterized by proliferation of beta cells. They are pancreatic neuroendocrine tumors, generally benign and small in size, characterized by insulin hypersecretion syndrome with the development of hypoglycemia. Most of these tumors are sporadic, only about 15-30% are hereditary; it is associated with diseases such as multiple endocrine neoplasia type 1, or Von Hippel-Lindau syndrome. The incidence of this tumor is 3-10 cases per million inhabitants. The average age of onset is between 40-50 years of age and it is more frequent in women. Early recognition and proper diagnosis of this rare pancreatic tumor are important aspects, as it is potentially curable with timely surgical treatment.

Renal Cell Carcinoma and a Pancreatic Neuroendocrine Tumor: A Coincidence or Instance of Von Hippel-Lindau Disease?

We herein report a rare case of a 79-year-old man who presented with the simultaneous occurrence of pancreatic neuroendocrine tumors (PNET) and renal cell carcinomas (RCC), without any other Von Hippel-Lindau (VHL)-associated lesions or any pertinent family history. Computed tomography showed vascular-rich solid lesions in the left kidney and the pancreatic tail, measuring 72 mm and 15 mm in size, respectively. Preoperatively, RCC with pancreatic metastasis was suspected and laparotomy was performed. However, the resected specimens revealed a different tumor histology, namely renal clear cell carcinoma (G2, pT3) and PNET (G1, pT3). The patient and his family refused genetic testing, however, so far, the patient has not developed any VHL-associated lesions for more than four years.