Duodenal Origin Research Articles

Léiomyosarcome portal : une localisation extrêmement rare !

Leiomyosarcomas of large vessels are rare. It is a malignant tumour and the vast majority of these tumours arose from the inferior vena cava. We report a rare case of portal vein leiomyosarcoma, in a 56-years-old female patient admitted for chronic abdominal pain with abdominal mass in the right hypochondrium all evolving in a context of deterioration in general condition. We performed an abdominopelvic CT scan and then a MRI with contrast agent which objectified a large tissue mass containing areas of necrosis at the level of the duodeno-pancreatic compartment communicating at a large angle with the portal trunk over its entire length from the hepatic hilum to the spleno-mesenteric confluence responsible for a portal cavernoma downstream. This is associated with multiple secondary nodular tissue hepatic lesions. We also noted a respect for the fatty border separating the mass of the duodenal tract and the head of the pancreas, and also the absence of dilation of the pancreatic ducts making a pancreatic origin unlikely. To eliminate a duodenal origin of the mass we performed an upper digestive endoscopy which came back without any abnormality. An ultrasound-guided trans parietal biopsy of a secondary hepatic lesion was done and the pathological result of which speaks of a secondary hepatic lesion of a leiomyosarcoma.

Gastric perforation biopsy: is it obsolete?

PurposeThe aim of the study was to test the established hypothesis that biopsies of spontaneous gastric perforations should be taken to rule out cancer.MethodsA prospective observational study was performed. Consecutive patients with spontaneous gastric perforation were included. Biopsies of the edges of the perforation were submitted for histological evaluation. The epithelial type as well as the nature of the pathology were evaluated.ResultsSixty-eight patients were included. Eight (12%) biopsies revealed duodenal origin. Sixty (88%) biopsies revealed gastric mucosa of which 33 (48%) could be specifically typed. All biopsies revealed benign ulceration. No malignancies were detected in these biopsies or on subsequent gastroscopic follow up.ConclusionThis study suggests that routine intraoperative biopsy of gastric perforation may be questioned. Biopsy is probably better performed endoscopically after recovery.

NTRK2 expression in gastrointestinal stromal tumors with a special emphasis on the clinicopathological and prognostic impacts

Gastrointestinal stromal tumors (GISTs) are typically characterized by activating mutations of the KIT proto-oncogene receptor tyrosine kinase (KIT) or platelet-derived growth factor receptor alpha (PDGFRA). Recently, the neurotrophic tyrosine receptor kinase (NTRK) fusion was reported in a small subset of wild-type GIST. We examined trk IHC and NTRK gene expressions in GIST. Pan-trk immunohistochemistry (IHC) was positive in 25 (all 16 duodenal and 9 out of 16 small intestinal GISTs) of 139 cases, and all pan-trk positive cases showed diffuse and strong expression of c-kit. Interestingly, all of these cases showed only trkB but not trkA/trkC expression. Cap analysis of gene expression (CAGE) analysis identified increased number of genes whose promoters were activated in pan-trk/trkB positive GISTs. Imbalanced expression of NTRK2, which suggests the presence of NTRK2 fusion, was not observed in any of trkB positive GISTs, despite higher mRNA expression. TrkB expression was found in duodenal GISTs and more than half of small intestinal GISTs, and this subset of cases showed poor prognosis. However, there was not clear difference in clinical outcomes according to the trkB expression status in small intestinal GISTs. These findings may provide a possible hypothesis for trkB overexpression contributing to the tumorigenesis and aggressive clinical outcome in GISTs of duodenal origin.

Lymph node recurrence and re-excision after primary tumor resection of a histiocytic sarcoma of duodenal origin: a case report

BackgroundHistiocytic sarcoma is a rare malignant tumor that is similar in characteristics to a mature histiocyte/macrophage and is a relatively new disease entity. In approximately one-third of cases, the site of origin is a lymph node; development from the gastrointestinal tract, spleen, soft tissue, and skin has further been reported. The tumor characteristics are not well-understood as reports on its clinical presentation and treatment are limited. We report a case of duodenal primary histiocytic sarcoma.Case presentationAn elevated lesion in the second part of the duodenum was detected in a 70-year-old woman during routine examination using upper gastrointestinal tract endoscopy. Blood biochemistry findings were normal for tumor markers. No abnormal findings were observed in the blood count and biochemical examination. Upper gastrointestinal endoscopy revealed a 20-mm elevated lesion with a slight depression in the center, opposite to the papilla of the descending duodenum. The biopsy showed erosions of the mucosal epithelium and inflammatory cell infiltration, but no evidence of malignancy. Ultrasound-guided endoscopy revealed an ischemic tumor of submucosal origin, and bowel biopsy suggested a histiocytic sarcoma. Distant metastasis and lymph node enlargement were absent on abdominal sonography, computed tomography, and magnetic resonance imaging. Duodenal segmental resection was performed. Immunostaining of the excised lesion was positive for CD68, CD163, CD4, CD5, CD15, and CD45 and negative for CD1a, CD21, CD34, MPO, and S-100 protein. Ki-67 positivity was approximately 20%. Based on these findings, the diagnosis of histiocytic sarcoma was confirmed. Ten months after the surgery, a lymph node recurrence in the dorsum of the pancreatic uncus was observed. No evidence of recurrence was found in any other part; hence, we performed pancreaticoduodenectomy. Pathological findings of the excised lymph node confirmed the recurrence of histiocytic sarcoma in the lymph node.ConclusionsThis is the first reported case of a duodenal primary histiocytic sarcoma with recurrence in the lymph node after the primary resection. The patient was treated for recurrence by lymph node excision and pancreaticoduodenectomy.

Extended surgical resection for nonfunctioning duodenal neuroendocrine tumor

Duodenal neuroendocrine tumors (NETs) account for <3% of all gastrointestinal NET. Most lesions are small-sized and are located in the first or second duodenal part. Tumoral grading, evaluated by Ki67 index, strongly influences patient’s outcome. Endoscopic resection is recommended for lesions measuring <2 cm, while pancreaticoduodenectomy should be the treatment of choice for large duodenal NET; Whipple procedure should be preferred in case of duodenal origin and contiguity with gastric antrum. Involvement of surrounding structures, as well as the presence of resectable liver metastases, does not contraindicate surgical resection. Herein we report a case of a 68-year-old male, presenting with an extensive mass of the descending pre-ampullary duodenal part, with involvement of the right colon and the presence of a pericholecystic single liver metastasis. In spite of such advanced disease, surgery on the patient was successful, with an uneventful postoperative outcome.

Metastatic Patterns of Duodenopancreatic Neuroendocrine Tumors in Patients With Multiple Endocrine Neoplasia Type 1.

Patients with multiple endocrine neoplasia 1 syndrome (MEN1) often develop multifocal duodenopancreatic neuroendocrine tumors (dpNETs). Nonfunctional pancreatic neuroendocrine tumors (PanNETs) and duodenal gastrinomas are the most frequent origins of metastasis. Current guidelines recommend surgery based on tumor functionality, size ≥2 cm, grade or presence of lymph node metastases. However, in case of multiple primary tumors it is often unknown which specific tumor metastasized. This study aims to unravel the relationship between primary dpNETs and metastases in patients with MEN1 by studying endocrine differentiation. First, it was shown that expression of the endocrine differentiation markers ARX and PDX1 was concordant in 18 unifocal sporadic neuroendocrine tumors (NETs) and matched metastases. Thereafter, ARX, PDX1, Ki67 and gastrin expression, and the presence of alternative lengthening of telomeres were determined in 137 microscopic and macroscopic dpNETs and 36 matched metastases in 10 patients with MEN1. ARX and PDX1 H-score clustering was performed to infer relatedness. For patients with multiple metastases, similar intrametastases transcription factor expression suggests that most metastases (29/32) originated from a single NET of origin, while few patients may have multiple metastatic primary NETs. In 6 patients with MEN1 and hypergastrinemia, periduodenopancreatic lymph node metastases expressed gastrin, and clustered with minute duodenal gastrinomas, not with larger PanNETs. PanNET metastases often clustered with high grade or alternative lengthening of telomeres-positive primary tumors. In conclusion, for patients with MEN1-related hypergastrinemia and PanNETs, a duodenal origin of periduodenopancreatic lymph node metastases should be considered, even when current conventional and functional imaging studies do not reveal duodenal tumors preoperatively.

Endoscopic Removal of Gastric Lipoma with Hemorrhage Using Endoscopic Submucosal Dissection

Most upper gastrointestinal lipomas occur in the duodenal second portion, and gastric lipomas are rare. Most lipomas are usually asymptomatic, but symptoms such as abdominal pain, intussusception, ulceration, and intestinal obstruction may occur depending on the size and location and, rarely, can cause bleeding. Endoscopic polypectomy, endoscopic mucosal resection, and surgical resection are the treatments of choice for lipomas with intestinal obstruction or bleeding. Upper gastrointestinal bleeding from lipoma is mostly of duodenal origin and very rarely from the stomach. Here, we report a case of successful treatment of gastric lipoma with massive bleeding by endoscopic resection.

Duodenopancreatectomia: análise histopatológica de tumores periampulares

Introdução: A Gastroduodenopancreatectomia Cefálica (GDPC) é a cirurgia de escolha para o tratamento de tumores periampulares. Os pacientes portadores dessa doença possuem sobrevida de 5 anos estimada em 20% a 50%, estando relacionado com o status de ressecção, estágio da doença e a localização do tumor. Conhecer o comportamento biológicos dos tumores periampulares possibilita um melhor planejamento no tratamento e no seguimento desses doentes. Método: O estudo analisou 105 pacientes através do resultado histopatológico oriundo de peças cirúrgicas de GDPC. Dados sobre sítio de origem, tamanho do tumor, status linfonodal, invasão perineural, invasão angiolinfática, margens de ressecção cirúrgicas e grau de diferenciação do tumor foram coletados e comparados com a literatura vigente. Resultados: Os pacientes portadores de neoplasia maligna totalizaram 94 (89,5%) das GDPCs. Desses, 40% (42) eram tumores pancreáticos, 37% (39) eram tumores de papila duodenal (papila de Vater), 4% (4) eram de origem duodenal e 2% (2) eram de colédoco distal. O tamanho médio dos tumores foi de 3,43cm (p = 0,049), com 85% dos tumores maiores de 2 cm e 46 (52,9%) dos adenocarcinomas eram estágio T3. Os linfonodos foram positivos em 27,6% dos adenocarcinomas e a margem foi R0 em 87% dos pacientes. Conclusão: O comportamento biológico dos tumores periampulares é de grande importância para pacientes que foram submetidos a cirurgia de GDPC. Um melhor planejamento no tratamento e no seguimento dos doentes pode ser oferecido quando se conhece o tipo histológico desses tumores. A experiência dos centros na realização dessa cirurgia tem importante relevância nos resultados obtidos.

A large intrahepatic duodenal duplication cyst in a 3 year-old girl

Abstract Duodenal duplication cyst consists 6% of alimentary tract duplications and the prevalence is estimated to be less than 1 per 100,000 live births. This report is the first case report in a pediatric patient. A 19-month-old female patient was detected with a 5.2 cm sized intrahepatic cystic lesion in her follow-up ultrasonography examination for hydronephrosis. In her regular check-up, findings suggestive of food materials were detected inside the cyst. Further evaluation showed a 7 cm sized cyst located inside segment IV of the liver, connected to the duodenal bulb with a possibility of biliary communication. On exploration, duodenal duplication cyst with a 1.5 cm long stalk starting from the duodenal bulb to the hilum of the liver was identified. Due to its sophisticated location and possible communication with the biliary tree, Roux-en-Y cystojejunostomy was performed after resecting the stalk. The histopathologic finding of the stalk showed serosa, muscle, submucosa and duodenal mucosa which suggested its duodenal origin. The patient has been followed up without any complications for 7 months.

Abstract B32: Special AT-rich sequence-binding protein 1 (SATB1) is an independent prognostic marker of poor prognosis in operated pancreatic and pancreatobiliary type adenocarcinomas

Abstract Background: Special AT-rich sequence-binding protein 1 (SATB1) is a global gene regulator that has been demonstrated to confer a more aggressive tumor phenotype and a shorter patient survival in several major cancer forms. The expression and prognostic significance of SATB1 in pancreatic, distal bile duct, ampullary or duodenal adenocarcinomas has however not yet been reported. Material and methods: A consecutive retrospective cohort of 175 patients with pancreatic and periampullary adenocarcinomas, operated with pancreaticoduodenectomy from January 1 2001 – December 31 2011, in the University hospitals of Lund and Malmö, Sweden, were followed up until December 31 2013. Microscopic slides were revised in a blinded manner and histopathological parameters relevant for classification and prognosis were recorded. Tissue microarrays were constructed from all primary tumors and 103 matched lymph node metastases. The expression of SATB1 was assessed by immunohistochemistry, and any intensity in more than 1% of cancer cells was considered a positive staining. Five-year overall survival was calculated and visualized using the Kaplan-Meier method and log rank analysis, stratified for tumor morphology and expression of SATB1. Cox regression models were used to estimate the impact of SATB1-expression on 5-year overall survival in multivariable analysis, adjusted for clinicopathological parameters. The Chi-square test and Fischer’s Exact test were used for calculations of correlations between SATB1-expression and other parameters. Results: SATB1-expression could be assessed in 107 pancreatobiliary type primary tumors (pancreatic, distal bile duct and ampullary origin) and in 61 intestinal type tumors (ampullary and duodenal origin). Median 5-year overall survival was 26.4 months in pancreatobiliary type adenocarcinomas and 53.3 months in intestinal type adenocarcinomas. There were 15% (16/107) SATB1-positive pancreatobiliary type adenocarcinomas and 25% (15/61) SATB1-positive intestinal type adenocarcinomas. Pancreatobiliary type SATB1-positive cases had a shorter median 5-year overall survival; median 14.6 months (interquartile range 8.9 – 24.7) vs. 27.5 months (interquartile range 15.8 – 46.3) compared to negative cases (p=0.014). The difference in 5-year overall survival remained significant in multivariable analysis (p=0.014) and the hazard ratio was 2.2 (95% CI 1.2 – 4.0) adjusted for T-stage, N-stage, tumor size, tumor grade, perineural growth, invasion in lymphatic vessels, invasion in blood vessels, growth in peripancreatic fat, involved margins, age and sex. Other independent parameters in pancreatobiliary type tumors were tumor grade, tumor size and invasion in blood vessels. SATB1-expression was correlated to invasion in blood vessels (p=0.009), but not to any other clinicopathological parameter. In intestinal type adenocarcinomas there was no correlation between primary tumor SATB1-positivity and overall survival. SATB1-expression could be assessed in metastases of 65 pancreatobiliary type adenocarcinomas and 24 intestinal type adenocarcinomas. There was a significant correlation between SATB1-positivity in primary tumors and metastases, but SATB1-expression in metastases was neither associated with clinicopathological parameters nor with survival. Conclusions: Our results show that SATB1-expression is an independent marker of poor prognosis in primary periampullary cancer of pancreatic and pancreatobiliary type and that SATB1-positivity is correlated to tumor invasion in blood vessels. This is the first report on the expression and prognostic significance of SATB1 in pancreatic cancer, distal bile duct cancer, ampullary cancer and duodenal cancer. Citation Format: Jacob Elebro, Karin Jirstrom. Special AT-rich sequence-binding protein 1 (SATB1) is an independent prognostic marker of poor prognosis in operated pancreatic and pancreatobiliary type adenocarcinomas. [abstract]. In: Proceedings of the AACR Special Conference on Pancreatic Cancer: Innovations in Research and Treatment; May 18-21, 2014; New Orleans, LA. Philadelphia (PA): AACR; Cancer Res 2015;75(13 Suppl):Abstract nr B32.

Primary supratentorial intracerebral malignant paraganglioma

Paragangliomas are extra-adrenal neuroendocrine tumours that derive from neural crest. They are benign tumours in general but few cases had shown their tendency to metastasize. Malignant forms have been reported previously with intracranial metastasis from duodenal origin but primary intracranial origin represent a rare and unusual location for such tumours. We report a rare case for a 48 year old lady who presented with symptomatic right sided insular mass with negative metastatic work up. A complete surgical resection had been done with an unexpected diagnosis of primary gangliocytic paraganglioma with malignant features.

Primary supratentorial intracerebral malignant paraganglioma

Paragangliomas are extra-adrenal neuroendocrine tumors that derive from neural crest. In general, they are benign tumors but few cases had shown a tendency to metastasize. Malignant forms have been reported previously with intracranial metastasis from duodenal origin, but primary intracranial origin represents a rare and unusual location for such tumors. Here, we report a rare case of a 48-year-old lady who presented with symptomatic right-sided insular mass with negative metastatic work up. A complete surgical resection had been done with an unexpected diagnosis of primary gangliocytic paraganglioma with malignant features.

Intestinal-type of differentiation predicts favourable overall survival: confirmatory clinicopathological analysis of 198 periampullary adenocarcinomas of pancreatic, biliary, ampullary and duodenal origin

BackgroundPeriampullary adenocarcinomas comprise pancreatic, distal bile duct, ampullary and duodenal adenocarcinoma. The epithelia of these anatomical structures share a common embryologic origin from the foregut. With steadily increasing numbers of pancreatoduodenectomies over the last decades, pathologists, surgeons and oncologists are more often confronted with the diagnosis of “other than pancreatic” periampullary cancers. The intestinal subtype of ampullary cancer has been shown to correlate with better prognosis.MethodsHistological subtype and immunohistochemical staining pattern for CK7, CK20 and CDX2 were assessed for n = 198 cases of pancreatic ductal, distal bile duct, ampullary and duodenal adenocarcinoma with clinical follow-up. Routine pathological parameters were included in survival analysis performed with SPSS 20.ResultsIn univariate analysis, intestinal subtype was associated with better survival in ampullary, pancreatic ductal and duodenal adenocarcinoma. The intestinal type of pancreatic ductal adenocarcinoma was not associated with intraductal papillary mucinous neoplasm and could not be reliably diagnosed by immunohistochemical staining pattern alone. Intestinal differentiation and lymph node ratio, but not tumor location were independent predictors of survival when all significant predictor variables from univariate analysis (grade, TNM stage, presence of precursor lesions, surgical margin status, perineural, vascular and lymphatic vessel invasion, CK7 and CDX2 staining pattern) were included in a Cox proportional hazards model.ConclusionsIntestinal type differentiation and lymph node ratio but not tumor location are independent prognostic factors in pooled analysis of periampullary adenocarcinomas. We conclude that differentiation is more important than tumor location for prognostic stratification in periampullary adenocarcinomas.

Duodenal and ampullary carcinoid tumors: Using size to predict necessity for lymphadenectomy.

316 Background: The metastatic potential and choice of therapy of duodenal and ampullary carcinoid tumors are poorly understood. We evaluated the local management and outcomes in patients with these uncommon tumors and determined factors predicting risk of nodal involvement. Methods: 117 patients were identified with duodenal or ampullary carcinoid tumors between 1996 and 2012, who were treated in a single high-volume center. Clinicopathologic data and overall survival by local treatment modality were analyzed. Results: Among all patients, 64 (55%) were treated surgically, including 34 (29%) who underwent pancreaticoduodenectomy (PD), 14 (12%) who underwent local resection (partial duodenectomy), and 16 (13.6%) where a carcinoid tumor was found incidentally after PD for another indication. The remaining 53 patients (45%) underwent endoscopic excision. The average tumor size was 1.8 cm (0.1-8.5) and the majority were of duodenal origin (n=93, 80%). Surgical management was more commonly performed for ampullary tumors compared to tumors of duodenal origin (83% vs. 47%, p=0.002), and endoscopic excision was more common with smaller tumors (p=0.001). Most carcinoids were well-differentiated (94%) and 55% were T1/T2. Yet, among the 55 patients in whom lymph nodes were histologically assessed (PD or lymph node sampling), 51% had positive nodes (N+). In addition, on multivariate analysis nodal involvement was strongly associated with tumor size (OR: 9.9, p=0.001). Specifically, tumors larger than 1-cm had positive nodes in more than 70% of cases whereas those ≤1cm had a 5% risk of nodal involvement (1 of 21 patients) (Table). Similar rates of N+ were observed for both duodenal and ampullary carcinoids. With long-term follow-up, only one recurrence was observed (1%). The overall survival was similar among all treatment groups (median=139 months). Conclusions: Lymph node involvement is common in patients with duodenal and ampullary carcinoid tumor, particularly among tumors &gt;1 cm in size. When possible, surgical resection with lymphadenectomy is recommended for such tumors. [Table: see text]

The migrating motor complex: control mechanisms and its role in health and disease

The migrating motor complex (MMC) is a cyclic, recurring motility pattern that occurs in the stomach and small bowel during fasting; it is interrupted by feeding. The MMC is present in the gastrointestinal tract of many species, including humans. The complex can be subdivided into four phases, of which phase III is the most active, with a burst of contractions originating from the antrum or duodenum and migrating distally. Control of the MMC is complex. Phase III of the MMC with an antral origin can be induced in humans through intravenous administration of motilin, erythromycin or ghrelin, whereas administration of serotonin or somatostatin induces phase III activity with duodenal origin. The role of the vagus nerve in control of the MMC seems to be restricted to the stomach, as vagotomy abolishes the motor activity in the stomach, but leaves the periodic activity in the small bowel intact. The physiological role of the MMC is incompletely understood, but its absence has been associated with gastroparesis, intestinal pseudo-obstruction and small intestinal bacterial overgrowth. Measuring the motility of the gastrointestinal tract can be important for the diagnosis of gastrointestinal disorders. In this Review we summarize current knowledge of the MMC, especially its role in health and disease.

Intra-operative haemodynamic volatility in a patient undergoing retroperitoneal cyst excision

Excision of a suspected retroperitoneal, duodenal duplication cyst was performed in a pre-operatively normotensive patient under combined epidural and general anaesthesia. Intraoperatively, the cystic tumour was discovered to be a retroperitoneal mass, free from duodenal or adrenal origin. Development of severe arrhythmias, ST segment changes and hypertensive spikes during cyst handling and dissection suggested the possibility of a catecholamine-secreting tumour. These were managed effectively with pharmacological agents. Subsequently, histopathology of the specimen revealed a paraganglioma. Vasoactive tumour has to be suspected in every patient undergoing anaesthesia for retroperitoneal cystic lesion.

Negative Control of Human Pancreatic Secretion

The negative control of pancreatic exocrine secretion in man occurs during the interdigestive and postprandial periods of the digestive cycle. The physiological mechanisms involved include negative feedback mechanisms, well described and accepted in animals, and controlled by the cholecystokinin- and secretin-releasing factors of pancreatic and duodenal origin, along with the active pancreatic proteases present in the upper gut. The presence of these factors and their efficacy in humans, however, have their supporters and detractors, with a possibility for reconciliation among opponents. Besides these releasing factors, hormones, mostly from the intestine, are also involved in this inhibitory process of pancreatic secretion. Somatostatin, peptide YY, pancreatic polypeptide, glucagon, ghrelin, and leptin were described as potentially involved from studies mostly performed on animals. Finally, bile and bile salts have mixed responses on this inhibition, and their effects seem to be at the intestine level with gastrointestinal hormones involved. Future studies will have to be performed in humans to determine the presence of cholecystokinin- and secretin-releasing factors and their role. Finally, the demonstrated modulatory action of hormones and bile acids in other species needs to be confirmed in humans.

Prognostic relevance of the novel TNM-classification system for neuroendocrine tumors of foregut origin

15147 Background: Until recently no TNM-classification existed for neuroendocrine tumors (NET) of the gastroenteropancreatic system. In 2006 Rindi et al. proposed a new TNM-classification system for foregut-NET of gastric, duodenal and pancreatic origin. The present study validates prognostic significance of the new TNM-classification in a cohort from a German referral center. Methods: The case files of 193 patients with histologically proven foregut NET were analyzed retrospectively. Patients were classified according to Capella (1995) and the WHO (2000) and to the new TNM-classification. Uni- and multivariate analysis was performed using log-rank and Cox regression methods. Results: Primary tumors localization was gastric (n=48), duodenal (23) and pancreatic (122); 35 patients (18%) died during the observation period. Overall 5- and 10-year survival rates (YSR) were 76% and 65% resp., while 5- and 10-YSR according to NET-related deaths (74% of all deaths, 26/35) were 83% and 74% resp.. The classifications by Capella and the WHO significantly discriminated between low and high grade malignant NET (p&lt;0,001 and 0,002 resp.), but did not allow further prognostic differentiation. In contrast, the newly proposed TNM-classification system was able to significantly differentiate stage IV NET from all other stages (I vs. IV p=0,001; II vs. IV p=0,001; III vs. IV p=0,008) as well as stage III from stage I NET (p=0,038). Furthermore, the new classification system which includes a grading system according to the mitotic index (Ki67-index; G1 &lt;3%, G2 3–20%, G3 &gt;20%) was also able to significantly distinguish all three groups from each other (G1 vs. G3 p&lt;0,001; G2 vs. G3 p&lt;0,001). On multivariate analysis stage I and II as well as stage III-NET had a decreased hazard ratio (HR) when compared to stage IV-NET (I/II: HR 0,094, p=0,003; III: HR 0,38, p=0,096). In the same model G1 and G2-NET were associated with an decreased HR as compared to G3-NET (G1: HR 0,05, p=0,01; G2: HR 0,20, p=0,002). Conclusions: Here we demonstrate prognostic relevance of the newly proposed TNM-classification system for foregut-NET with statistical significance which therefore provides a new and powerful tool for future classification of NET. No significant financial relationships to disclose.

Gastrointestinal stromal tumors: CT and MRI findings

The objective of this study was to report the CT and MRI appearances of primary and metastatic gastrointestinal stromal tumor (GIST). The clinical and imaging findings of 31 patients with histological and immunohistochemical diagnosis of GIST were reviewed. The CT and MRI findings were assessed independently for size, location, enhancement characteristics, and pattern of metastatic disease. The tumors were of enteric (n=13), gastric (n=12), duodenal (n=2), and rectal (n=3) origin. In one case the primary site was the mesentery, without involvement of bowel. Primary tumors were typically exophytic (79%), larger than 5 cm (84%), and inhomogeneously enhancing (84%). Central necrosis of all tumors (37%) and aneurysmal dilation of enteric tumors (33%) were less common. Metastases were most commonly to mesentery (26%) or liver (32%). Less common findings were ascites (7%) and omental caking (3%). Liver metastases were hypervascular in 92% of patients and rapidly became cystic following therapy with imatinib mesylate (Gleevec; Novartis, East Hanover, NJ, USA). Lung metastases, bowel obstruction, vascular invasion, and significant lymphadenopathy were not seen in any patient. GISTs have some specific CT findings which could help differentiate them from other gastrointestinal tumors. Liver metastases became cystic following therapy, mimicking simple cysts. MRI was better than single-phase CT for assessing liver metastases, while CT was more sensitive for mesenteric metastases.

Neuroendocrine Tumours of the Pancreas: Predictors of Survival after Surgical Treatment

Aims: Neuroendocrine tumours of pancreatic and duodenal origin (NETP) are rare and we present a significant experience from a single centre. Methods: Data was collected on 44 patients who underwent surgery between 1988 and 2002. Since 1997, data have been recorded prospectively on a dedicated database. Results: Twenty-four patients had functioning tumours (16 insulinomas, 3 gastrinomas, 2 somatostatinomas, 1 vipoma, 1 glucagonoma and 1 carcinoid tumour). Nine functioning tumours and 13 non-functioning had a malignant phenotype. Twenty pancreaticoduodenectomies, 9 local excisions, 7 distal and 2 total pancreatectomies, 5 bypasses and 1 exploratory laparotomy were performed. Fourteen patients (31.8%) had surgical complications, 1 died peri-operatively (2.3%). The overall actuarial survival for resected cases was 74.4 and 42.5% at 5 and 10 years, respectively. Lymph node invasion and metastases were significant predictors of survival by univariate analysis and only the presence of metastases retained significance on multivariate analysis. Conclusion: Surgical resection is the only curative treatment for NETP. Resection can be safely carried out in a specialist centre and is associated with good long-term survival. The presence of metastases was a significant predictive factor for survival in patients with NEPT in this series.