Uncinate Research Articles

Pancreaticoduodenectomy in a patient with intestinal malrotation and distal cholangiocarcinoma: a case report and review of the literature

BackgroundA combination of intestinal malrotation and distal cholangiocarcinoma is considered a rare condition and poses some difficulties in surgical management. We present a case of a patient with asymptomatic nonrotation of the midgut with a concomitant distal cholangiocarcinoma who underwent successful pancreaticoduodenectomy.Case presentationA 52-year-old Sudanese man presented to our hospital with progressive painless jaundice associated with dark urine, pale stool, and itching for the last 2 months. He had no other complaint or significant previous medical history apart from being an ex-smoker. His clinical examination revealed a palpable gallbladder and scratch mark. His other systems were unremarkable. His blood test results showed a normal complete blood count, elevated total bilirubin (mainly direct bilirubin), elevated alkaline phosphatase, and normal cancer antigen 19-9 and carcinoembryonic antigen. Ultrasound, computed tomography of the abdomen, and magnetic resonance cholangiopancreatography showed a dilated intrahepatic and extrahepatic biliary system down to the distal part, where the lumen was obstructed by a periampullary mass measuring 2.4 by 2.1 cm. The patient’s gallbladder was distended. He had no liver metastases or ascites and few lymph nodes. Inversion of the superior mesenteric artery and superior mesenteric vein but no invasion was seen, and malrotation of the bowel was observed with the large bowel on the left side and the small bowel to the right of the abdomen. Endoscopic retrograde cholangiopancreatography showed a fleshy ampulla that was stented. Laparotomy showed malrotation, with the duodenum straight on the right side of the midline, and Ladd’s band crossed the second portion of the duodenum. The vessels were approached from the lateral side meticulously after kocherization of the duodenum and pancreas, dissection along an extended portion of the superior mesenteric artery to assure preservation of the superior mesenteric artery and branches going to the jejunum, Ladd’s procedure, division of the jejunum 10 cm below the uncinate process of pancreas, and modified pancreaticoduodenectomy were performed, and anastomoses were performed in the standard fashion. The patient had an uneventful postoperative course, started oral feeding after 5 days, and discharged to home on day 10 for regular follow-up. Histopathology confirmed distal cholangiocarcinoma, and the patient was referred for further oncological management.ConclusionsPancreaticoduodenectomy can be safely performed in patients with intestinal malrotation with some modifications of the standard approach. Meticulous dissection after preoperative identification of vascular anomaly and a lateral approach are of great help to reduce morbidity.

Carcinoid of the uncinate process of the pancreas in a child: An unusual case

Carcinoid of the pancreas is rare among children. We treated a debilitating child who complained about post-prandial pain with a mass in upper abdomen, vomiting and weakness for a period of six months. Abdominal examination revealed an immobile, mildly tender, solid mass in the right lumbar region. Ultrasonography of abdomen demonstrated a tumour of head of the pancreas and ultrasonography-guided fine needle aspiration cytology of the mass suggested a malignant small round cell tumour. With Computed Tomogram an enhancing compact big mass was found at the head of the pancreas compressing the duodenum without invasion and metastasis. At exploratory laparotomy, we also detected a well-defined, rounded, big solid mass arising from uncinate process of the pancreas with no metastasis. After the excision of the mass along with a healthy portion of uncinate process, the patient recovered successfully. Histopathology evaluation showed a neoplasm composed of small nests and sheets of a dense cluster of malignant round cells with hyperchromatic nuclei, sometimes forming acini with stromal invasion. Diagnosis was malignant carcinoid of the pancreas, T2 N0 M0. At follow-up of three and a half years, the patient was found symptom-free and there was no evidence of recurrence.
 Mediscope Vol. 7, No. 1: Jan 2020, Page 58-62

MON-LB59 Radiofrequency Ablation as a Primary Therapy for Benign Functioning Insulinoma

Background Insulinomas are rare life-threatening pancreatic neuroendocrine tumors. Surgical removal continues to be the treatment of choice for such benign cases with a high cure rate. However, surgery is associated with a considerable risk of morbidity and mortality.Here we describe a case of benign solitary insulinoma successfully treated with RFA in a patient who strongly refused surgery. Case Description A 56-year- old non- diabetic, male is known to have hypertension and lymphoma diagnosed at age of 20 years, in remission. He presented with recurrent episodes of transient ischemic attack and stroke over the last three years. A change in his cognitive function, behavior, and memory was noticed. During his hospital stay for the second episode of stroke, he was found to have hypoglycemia which was asymptomatic. Insulinoma was confirmed based on the followings: low plasma glucose level of 2.04 mmol/l (4.0- 5.6), inappropriately elevated plasma f insulin and C-peptide 68.9 mU/l (3-13) and 4.08 ug/l (1.0-3.1); respectively. Sulfonylurea screening test was negative. MRI of the abdomen showed a 3.2x2.5 cm, well-circumscribed hypervascular lesion at the uncinate process of the pancreas, which is compatible with neuroendocrine tumor. Treatment modalities have been explained to the patient who was fully informed about the risk and benefit of each treatment option. However, he strongly refused surgery. Meanwhile, he was admitted with a third attack of stroke with concurrent hypoglycemia. In view of his refusal of the surgical treatment and due to his recent stroke and high-risk status for surgery, the option of radiofrequency ablation was decided. RFA of the pancreatic tumor using 40.75 GY fractions was carried out with a favorable outcome. The patient achieved biochemical normalization and remains euglycemic during his follow up. Reversal of his cognitive, behavioral, and memory changes was recognized. CT abdomen during a follow-up of 2 months after radiation showed a mild regression of the size of the tumor with no evidence of new lesions or distance metastasis. He remained under close follow up at the neuroendocrine clinic. ConclusionThis case shows a treatment challenge which required the use of an alternative treatment option other than the standard of care in managing a case of benign insulinoma. Successful treatment was achieved in our case with the use of RFA rather than surgery. This report highlights the evolving evidence of nonsurgical RFA being a potential safe, feasible therapeutic modality option or even an alternative to surgery in selected cases of benign insulinoma. Because of the rarity of this tumor and low rate of therapeutic application of RFA in similar cases, the role of RFA had not been extensively studied. Large studies are needed to evaluate the long-term outcomes of RFA in this setting.

MON-911 Debilitating Neuropsychiatry Symptoms in Pancreatic Insulinoma Co-Secreting Serotonin and IGF-1

Background:Insulinoma is the most common type of functioning pancreatic neuroendocrine tumor (NET). Polyhormonal secretions from the NET, giving rise to distinct clinical symptoms such as carcinoid symptoms are rare. Clinical Case: We report a 68-year-old woman who presented with four months history of recurrent diaphoresis, palpitations, tremors and chest tightness. These were associated with episodic paroxysms of flushing and diarrhoea. The physical examination was unremarkable. She was a well-nourished woman with BMI of 28 kg/m2. Initial laboratory tests ruled out any renal, liver abnormalities with normal cortisol and thyroid function test. Further evaluation confirms insulin mediated hypoglycaemia with low random blood sugar 2.5 mmol/l (4.4-7.8) and failure to suppress C-peptide, 1092 pmol/L (298-2350) and insulin levels, 12.7 mU/L (3-25). Urine 5-HIAA was markedly elevated 2430.37 µmol/day (3.66-42.89) with borderline elevation of serum chromogranin A level 122 ng/mL (27-94). IGF-1 was also raised at 416 ug/L (91-282). Two months later she presented with new onset of delirium, incoherence, agitation and restlessness independent of her hypoglycaemic events. These symptoms deteriorated and fluctuates throughout the day with period of normalcy in between. This has led to requirement of a full time caregiver for her. Cranial CT excluded any brain pathology. We are faced with a diagnostic challenge to localize the primary lesion as radiological imaging so far were normal. GALLIUM-68 PET CT showed physiological uptake in the uncinate process of the pancreas (SUVmax 14.4). Endoscopic ultrasound of the pancreas was normal. An intra-arterial calcium stimulation test with hepatic venous sampling (ASVS) confirms a lesion at the head of pancreas with two times increment of insulin from baseline at the gastroduodenal artery distribution. Despite elimination of hypoglycaemic events with Diazoxide 100mg twice daily, her neuropsychiatric symptoms persisted. We postulate that this might be from excessive peripheral production of serotonin by the pancreatic carcinoid tumour or a niacin deficiency state because of metabolic diversion of its precursor, tryptophan. Conclusion:This case highlights the occurrence of debilitating neuropsychiatry manifestations in a likely neuroendocrine tumour arising from the head of pancreas secreting insulin, serotonin and IGF-1.

MON-922 Multiple Endocrine Neoplasia Type 1- A Clarion Call for Clarity

A 35-year-old gentleman presented with epigastric pain and bilious emesis. He also endorsed urinary frequency, non-bloody diarrhea and diffuse bone pain. On physical examination he had epigastric tenderness and multiple hyperpigmented skin lesions.An abdominal computed tomography (CT) scan revealed multiple diverticula with peri-colonic fat stranding in the descending and sigmoid colon, concerning for diverticulitis. He was started on a course of metronidazole and ciprofloxacin. A 3.1 cm mass was incidentally noted in the uncinate process of the pancreas. Bilateral adrenal nodules were also appreciated.An endoscopic ultrasound (EUS) guided trans-gastric fine needle aspiration biopsy was performed, revealing a well differentiated pancreatic neuroendocrine tumor (pNET - pT3N1Mx, intermediate risk). Chromogranin A was elevated to 108 ng/ml (reference range <93 ng/ml). Serum and urine metanephrine, V-peptide, gastrin, glucagon and parathyroid hormone related peptide were all normal; indicating a nonfunctioning neuroendocrine tumor. He underwent a pancreaticoduodenectomy. Octreotide scan was unrevealing for residual uptake. Adrenal biopsy revealed adrenal adenomas.Three years later, he presented with severe abdominal pain and a new pancreatic mass was noted on CT. Chromogranin A was elevated to 227 ng/mL. EUS revealed a 0.35 cm mass in the bed of the pancreatic head, encasing the superior mesenteric artery. Pathology was positive for recurrence of the neuroendocrine tumor.He was hypercalcemic to 11.4 mg/dL and parathyroid hormone was elevated to 319 pg/mL. CT neck revealed a 0.1 cm nodule concerning for parathyroid adenoma. He underwent a subtotal parathyroidectomy.Genetic testing confirmed Multiple Endocrine Neoplasia Type 1 (MEN1) with a heterozygous mutation of the menin1 gene.MEN1 is a rare genetic syndrome with affected individuals at increased risk of developing pancreatic, pituitary, parathyroid gland and cutaneous tumors. With a kaleidoscope of presentations, clinicians must maintain a high index of suspicion for MEN1, particularly for cases with nonfunctioning pNETs which present insidiously and are the foremost cause of mortality in MEN1 patients.1Further clarity is needed on MEN1 associated pNET prognostic risk stratification, surveillance and targeted immunochemotherapy.2 Timely and algorithmic screening for MEN 1 syndrome in patients with pancreatic incidentalomas is essential to improving patient outcomes.1.Kamilaris CDC, Stratakis CA. Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis. Front Endocrinol. 2019;10:339. doi:10.3389/fendo.2019.003392.Yates CJ, Newey PJ, Thakker RV. Challenges and controversies in management of pancreatic neuroendocrine tumours in patients with MEN1. Lancet Diabetes Endocrinol. 2015;3(11):895-905. doi:10.1016/S2213-8587(15)00043-1

Morphogenesis of the ventral pancreas anlagen is influenced by the SMA branching pattern

Developmentally, the uncinate process of the pancreas is derived from the ventral pancreatic anlagen, supplied by the superior mesenteric artery (SMA), and contains pancreatic polypeptide (PP)-rich islets of Langerhans. In contrast, the other parts of the pancreas originate from the dorsal anlagen supplied by the celiac system and contain PP-poor islets. This study was performed to investigate whether morphogenesis of the ventral pancreas anlagen is associated with the pattern of SMA branching. SMA branches to the pancreatic body were dissected in 44 cadavers. The cadavers were divided into two groups: the SMA group in which the SMA gave off branches to the pancreatic body and the General group in which it did not. In the SMA group, the ratio of the diameter of the SMA branch supplying the pancreatic body (SMA branch) to that of the SMA itself was calculated. After dissection was completed, tissues were collected from all pancreatic specimens for HE staining and for immunohistochemistry with PP and insulin antibodies. There were 25 cadavers in the General group and 19 in the SMA group. In 10/19 cadavers from the SMA group, PP-rich islets were confirmed in the pancreatic body. The SMA branch diameter ratio was significantly smaller in the SMA group cadavers with PP-poor islets (n = 9) than in cadavers with PP-rich islets (n = 10) (P < 0.001). These findings suggest a relation between the SMA branching pattern and the distribution of PP cells.

An unusual case of steatorrhea

Partial agenesis of the pancreas is a rare developmental anomaly of the pancreas which can lead to exocrine and endocrine insufficiency. We report a 61-year-old male patient with steatorrhea who was found to have complete agenesis of the dorsal pancreas. He presented with a history of loose stools for 5 years, and the stools were at times oily in nature without mucus or blood. His clinical examination was unremarkable. Seventy-two hour stool fat was increased (21.4 g, normal 200), and computed tomography of the abdomen revealed a normal head and uncinate process of the pancreas, abscence of body and tail of pancreas associated with a nondilated duct system. He was put on pancreatic enzyme supplements and he responded to the treatment, and on follow-up after 6 months, his stool frequency had declined and his fecal elastase had improved.

Significance Of "Dominant" Uncinate Duct Dilatation In Ipmn: A New High-Risk Criterion

Presenter: Samer AlMasri MD | University of Pittsburgh Medical Center Background: The uncinate process of the pancreas has a dominant drainage ductal system that can either empty into the accessory or main pancreatic ducts. Although several autopsy series have outlined the anatomical footprints for this independent drainage system, current international consensus guidelines of intraductal papillary mucinous neoplasms (IPMNs) still consider it as a branch duct, even though it is the main drainage system for a once independent portion of the pancreas. We hypothesized that, in addition to the well-defined high-risk criteria of IPMNs, dilation of the “dominant” uncinate duct (UDD) is associated with advanced neoplasia (high grade dysplasia /invasive cancer = HGD/IC) on final resection pathology, and may therefore be classified as an independent high-risk criterion. Methods: A retrospective review of patients who underwent surgical resection of a pancreatic IPMNs between 2008-2019 at our institution was performed. Preoperative imaging studies (pancreas protocol CT and /or MRI) were reviewed by an abdominal radiologist who was blinded to the final pathological Results. In addition to the Fukuoka criteria, we recorded whether there was any UDD irrespective of the location of the primary lesion. Using multivariate logistic regression, the pathological significance of UDD was determined. Results: A total of 260 patients were identified. The mean age at diagnosis was 67.9 years and 49.2% were females. Of the entire cohort, 122 (47%) had HGD/IC and 138 (53%) had non-invasive (low/intermediate grade dysplasia) disease in the surgical specimen. UDD was noted in 59 (22.7%) patients, of which 36 (61%) had invasive disease on pathological evaluation (P < 0.003). On multivariate logistic regression for the entire cohort, adjusting for size, main duct dilation, presence of enhancing mural nodule, bilirubin level and demographics, UDD was an independent predictor of invasive disease (OR= 2.99, P < 0.04). Subgroup analysis was performed on patients with main/mixed duct IPMNs and branch duct IPMNs with high risk criteria confined to the dorsal pancreas (n=170, 66.4%) in order to determine the pathological significance of UDD in these remote lesions. Even in this subgroup of patients, UDD was a significant predictor of HGD/invasive cancer (OR= 7.6, P < 0.004), suggesting it may be associated with an aggressive field effect. Conclusion: This is the largest study to date evaluating the significance of uncinate “dominant” duct dilation in IPMNs, and shows it to be a high-risk feature associated with HGD/IC. This association persisted for IPMNs limited to the dorsal pancreas, suggesting UDD may be associated with an aggressive “field effect”. Although these findings warrant validation with larger prospective studies, we recommend this feature be considered an additional high-risk criterion in IPMN, irrespective of the location of the cystic lesion.

2137 The Game Changing Utility of EUS-FNA for Pancreatic Evaluation: Not All Pancreas Masses on CT Are Pancreas Tumors!

INTRODUCTION: A pancreatic mass seen on cross-sectional imaging with liver lesions or lymphadenopathy is concerning for a pancreatic malignancy. Previous reports have discussed the utility of EUS-FNA for evaluation of pancreatic masses; this case series aims to reinforce the game-changing utility of EUS-FNA for the clarification of suspected pancreatic neoplasia. CASE DESCRIPTION/METHODS: Case #1: A 68-year-old woman undergoing evaluation for hematuria was found to have two enhancing liver lesions, two pulmonary nodules, and a low attenuation zone in the uncinate process of the pancreas on CT (Image 1). Physical exam and laboratory tests were unremarkable. A pancreas protocol CT at our center confirmed the above findings and the patient was referred for EUS-FNA. On EUS, pancreatic anatomy was completely normal and no mass was identified. Subsequent evaluation with a triphasic MRI (Image 2) concluded that the liver lesions were atypical hemangiomas and the pancreas had focal fatty replacement that can mimic a pancreatic tumor. A repeat EUS at 6 months was unremarkable and the patient is doing well. Case #2: A 56-year-old woman with history of melanoma presented with RUQ pain and +30lbs weight loss over the previous 5 months. Physical examination was unremarkable and laboratory tests were notable for elevated LFTs. CT (Image 3) revealed a homogeneously enhancing retroperitoneal mass arising between the pancreatic uncinate and D3 with several enlarged mesenteric lymph nodes, suspected to be pancreatic neoplasm. Further evaluation with EUS revealed a 3.5 cm heterogenous periduodenal mass abutting D2 and enlarged peri-gastric lymph nodes. FNA was performed and histopathology identified the mass as gastrointestinal stromal tumor (GIST) with reactive lymph nodes. The patient was started on neoadjuvant Imatinib with approximately 25% shrinkage of the mass appreciated in the first 2 months. Afterward, the patient underwent pancreaticoduodenectomy and surgical pathology revealed a mixed subtype duodenal GIST with a low mitotic rate. DISCUSSION: We report 2 cases of suspected metastatic pancreatic carcinoma whose diagnoses were elucidated as focal fatty replacement and duodenal GIST, respectively, with the help of EUS +/- FNA. Pancreatic lesions found on cross-sectional imaging that are thought to be primary pancreatic neoplasms should be further evaluated by EUS. EUS with/without FNA can be a game-changing imaging modality in such cases, providing an accurate diagnosis and helping guide appropriate management.

Clinical efficacy of radical resection with individualized surgical approach for borderline resectable pancreatic head carcinoma

Objective To explore the clinical efficacy of radical resection with individualized surgical approach for borderline resectable pancreatic head carcinoma. Methods The retrospective descriptive study was conducted. The clinicopathological data of 54 patients with borderline resectable pancreatic head carcinoma who underwent radical resection with individualized surgical approach in the West China Hospital of Sichuan University from January 2015 to January 2018 were collected. There were 37 males and 17 females, aged from 37 to 73 years, with a median age of 59 years. For venous type borderline resectable pancreatic head carcinoma, surgery for pancreatic head carcinoma and (or) pancreatic head and neck carcinoma was performed via inferior mesenteric vein, and surgery for pancreatic uncinate process carcinoma was performed via inferior colon artery. For arterial type borderline resectable pancreatic head carcinoma, surgery for pancreatic head carcinoma and (or) pancreatic head and neck carcinoma was performed via medial uncinate artery, and surgery for pancreatic uncinate process carcinoma was performed via left posterior artery. Observation indicators: (1) surgical situations; (2) postoperative complications; (3) postoperative pathological examination; (4) follow-up. Patients were followed up by outpatient examination or telephone interview once every 3 months to detect survival up to March 2019. Measurement data with normal distribution were represented by Mean±SD. Measurement data with skewed distribution were represented by M (range), and count data were represented by absolute numbers or percentage. Kaplan-meier method was used to draw the survival curve and calculate the survival rate. Results (1) Surgical situations: all the 54 patients underwent expanded pancreatoduodenectomy combined with superior mesenteric vein/portal vein (SMV/PV) resection, including 15 via inferior mesenteric vein, 20 via inferior colon artery, 12 via medial uncinate artery, and 7 via left posterior artery. The operation time was (320±83)minutes, and the volume of intraoperative blood loss was (865±512)mL. (2) Postoperative complications: of 54 cases, 28 had postoperative complications, including 13 with grade 1 Clavien-Dindo complications, 12 with grade 2 Clavien-Dindo complications, 3 with grade 3 or above Clavien-Dindo complications. One of the 28 patients with postoperative complications died and 27 were improved after symptomatic and supportive treatment. (3) Postoperative pathological examination: of 54 patients, 31 had R0 resection and 23 had R1 resection. In the 23 patients with R1 resection, 5 underwent surgery via the inferior mesenteric vein (4 with involvement of pancreatic anterior surface, 1 with involvement of both pancreatic anterior and posterior surface), 9 underwent surgery via the inferior colon artery (2 with involvement of both pancreatic anterior and posterior surface, 2 with involvement of superior mesenteric artery margin, 2 with involvement of pancreatic posterior surface, 2 with involvement of pancreatic anterior surface, 1 with involvement of superior mesenteric artery margin and pancreatic posterior surface), 5 underwent surgery via the medial uncinate process artery (2 with involvement of superior mesenteric artery margin, 2 with involvement of both pancreatic anterior and posterior surface, 1 with involvement of pancreatic neck transected margin), and 4 underwent surgery via the left posterior artery (3 with involvement of superior mesenteric artery margin, 1 with involvement of both pancreatic anterior and posterior surface). Of 54 patients, 16 had no positive lymph nodes, 26 had 1-3 positive lymph nodes, and 12 had 4 or more positive lymph nodes. The tumor diameter was (3.20±0.14)cm. There were 48 of 54 patients with nerve infiltration, 41 with superior mesenteric vein and/or portal vein infiltration, and 11 with vascular thrombus. There were 17 of 54 patients with high differentiation and medium differentiation, and 37 with low differentiation and undifferentiation. (4) Follow-up: 54 patients were followed up for 1-42 months, with a median time of 19 months. The 1-, 3-year overall survival rate was 78.0%, 11.4%. Conclusion As for the borderline resectable pancreatic head cancer, individualized and customized surgical approach according to the location of tumor and the relationship with blood vessels is helpful to standardize the radical resection and avoid R2 resection. Key words: Pancreatic neoplasms; Pancreatic cancer; Borderline resectable; pancreaticoduodenectomy; Surgical approach; R2 resection; Efficacy

Progress of obsessive-compulsive disorder in diffusion tensor imaging

Diffusion tensor imaging (DTI), a noninvasive MRI technique for measuring the integrity and direction of white matter in the brain, is a promising method to explore the pathogenesis of obsessive compulsive disorder (OCD). At present, the etiological research of OCD suggests that, in addition to the classical cortical-striatum-thalamus-cortical (CSTC)circuit, the parietal lobe, temporal lobe, occipital lobe and other regions of the brain are also involved in the pathogenesis of OCD. White matter fibers are important structure of material information communication in various brain regions and affected by individual neurodevelopment. Therefore, it is very important to explore the characteristics of white matter fiber in the brain of patients with OCD by using diffusion tensor imaging technology clarifying its role in the occurrence of OCD. This paper reviews the studies on uncinate fasciculus, superior longitudinal fasciculus, corpus callosum in adult DTI studies and the progress of DTI studies on children and adolescents. Key words: Obsessive-compulsive disorder; Diffusion tensor imaging; Diffusion tractography

SUN-LB057 An Interesting Case of Multiple Pancreatic Neuroendocrine Tumour: Normoglycaemic Glucagonoma and Concurrent Gastrinoma

Background: Pancreatic neuro-endocrine tumours (pNETs) are rare tumours, constituting less than 5% of pancreatic tumours. Of these cases, only an estimated 6% present with functional secretion of more than one hormone. Clinical Case: A 72-year-old woman was admitted to the acute frailty ward with symptoms of generalized weakness, fatigue and reduced exercise tolerance with shortness of breath on exertion. She reported unintentional weight loss of 16 kg in the preceding 3 months and widespread migratory rash with epidermal breakdown in the lower legs that was resistant to antibiotic therapy. Initial investigations on admission revealed severe anaemia with a haemoglobin (Hb) of 51 g/L that was refractory to blood transfusion, prompting gastroscopy that showed erosive gastritis. CT/MRI scans revealed a 24 mm solid arterial-enhancing mass lesion in the uncinate process of the pancreas, highly suggestive of a neuroendocrine tumour (NET). Histopathological examination of a skin biopsy showed intraepithelial vacuolisation of the cells in the upper epidermis, neutrophilic infiltration with psoriasiform hyperplasia and confluent parakeratosis; typical features of necrolytic migratory erythema (NME). Fasting serum gut hormone profile prior to omeprazole therapy was consistent with multiple functioning NET with elevated serum glucagon level (>500 pmol/L) and hypergastrinaemia (274 pmol/L). Fasting serum glucose level was 4.5 mmol/L and serum albumin was 24 g/L. Elevated serum fasting serum glucagon and gastrin levels strongly supported diagnosis of neuroendocrine tumour resulting in normoglycaemic glucagonoma syndrome (NME, weight loss without diabetes mellitus) and Zollinger-Ellison syndrome (anaemia and hyperchlorhydria). The rash resolved rapidly less than a week after commencing medical management with octreotide, a somatostatin receptor analogue. She was also commenced on high dose PPI therapy. Somatostatin receptor scintigraphy was performed that confirmed a somatostatin-avid primary tumour in the pancreas without distant metastases. Surveillance will include blood glucose monitoring, interval rescanning with CT abdomen and pelvis with contrast and octreotide scan to assess for metastases. Surgical resection of the tumour will be considered as the patient's performance status improves. Conclusion: We report a rare case of a patient diagnosed with a multiple functioning pNET. The clinician must always consider unconventional rashes as dermatological manifestations of systemic disease. A normal fasting serum glucose level is not a reliable exclusion criterion of glucagonoma syndrome due to functioning insulin secretion from beta pancreatic islet cells. Isolated measurement of fasting serum gastrin level is not appropriate for diagnosis of Zollinger-Ellison syndrome in patients treated with proton pump inhibitors or histamine receptor antagonists. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

Research on mode of anisotropy and other diffusion tensor imaging derived indices of white matter with mild to moderate Alzheimer disease based on tract based spatial statistics

Objective We performed a diffusion tensor imaging (DTI) study to explore the value of mode of anisotropy (MO) and other DTI-derived indices in assessing white matter fiber damage with mild to moderate Alzheimer disease (AD) patients. Methods This study was a prospective study. From February 2015 to February 2018, 33 patients with mild to moderate AD according to criteria were prospectively recruited as AD group at the First Affiliated Hospital of Wenzhou Medical University. Twenty healthy age-, sex-matched volunteers were recruited as normoal conctrast (NC) group. All subjects were performed by conventional brain MRI and DTI scans. The MO, anisotropic fraction (FA), mean diffusivity (MD), radial diffusivity (RD) and axial diffusivity (AxD) of white matter fibers were obtained from DTI images after TBSS post-processing. The statistical analyses of DTI indices between AD group and NC group were carried out in Functional MRI Software Library software. Results As compared with NC group, we found 1 cluster with significant decrease FA and increase RD in AD group, the affected fibers involving bilateral anterior thalamic radiation, corticospinal tract, cingulate gyrus, hippocampus, inferior fronto-occipital fasciculus, inferior longitudinal fasciculus, uncinated fasciculus, superior longitudinal fasciculus (P<0.05). The MD value increase(P<0.1) was found to coincide with the decrease of FA and the increase of RD, while no significant increase or decrease in P<0.05. Three clusters of MO increases (P<0.05) accompanying with increases of AxD were found in left anterior limb of internal capsule, bilateral posterior limb of internal capsule, posterior forceps, left centrum semiovale and parietal lobe. Two clusters of MO decreases (P<0.05) accompanying with decreases of FA and increases of RD were found in fornix, forceps, external capsule, centrum semiovale and left frontal lobe. Conclusions The combination of MO with DTI routine tensor indices (FA, RD, MD, AxD) can further reveal the microstructural damage of white matter fibers in AD, especially to crossing-fibers. The abnormalities of MO combined with FA, RD and MD reveal the degeneration of both bundles of crossing-fibers, and the abnormalities of MO combined with AxD reveal that the white matter damage was mainly caused by axon damage of one bundle of cross fibers. Key words: Alzheimer disease; Diffusion tensor imaging; Mode of anisotropy; Comparative study

SOLID PSEUDOPAPILLARY TUMOR OF THE PANCREAS IN CHILDREN: 10 YEARS OF EXPERIENCE

Introduction: a solid pseudopapillary tumor (SPPT) of the pancreas is an extremely rare disease occurring in 0.13-2.7% of all pancreatic tumors. This tumor is characterized by a fairly favorable clinical course, with low metastasis potential. The clinical picture is highly non-specific, it is more common in girls of puberty and young women under 30 years of age. The main treatment method is surgical. The purpose of the study is to study the clinical and pathological characteristics of this disease and the possibilities of surgical treatment of patients with this pathology. Material and methods. The analysis of clinical data of patients diagnosed with SPPT in our Institute over the past 10 years (2007-2017). And also therу was reviewed the morphological data of SPPT, types of surgical treatment, results, and complications. Results. For 10 years, SPPT was detected in 24 patients. All patients were girls of puberty from 9 to 17 years. In most cases, the disease was asymptomatic and was diagnosed as an "accidental find" during a medical examination. Two patients had liver metastases. All patients underwent surgical treatment in a different volume: 8 patients - laparoscopic distal pancreatic resection, 3 - gastropancreatoduodenal resections, 3 - pancreatoduodenal resection, 6 - central pancreatic resections, 3 - distal subtotal pancreas resection and 1 - resection of uncinate process of the pancreas with resection of the duodenal wall. Complications occurred in 6 patients: in 4 - postoperative pancreatitis with the formation of pancreatic fistula and in one case - bleeding from the pancreatic branch of the splenic artery and bleeding from the wound surface. The follow-up period lasted from 3 months to 11 years. All patients are alive. Conclusion. SPPT is a rare tumor with a benign course potential. Clinical signs and symptoms are relatively nonspecific. Surgical treatment is the main method of treatment, although it is characterized by a high risk of postoperative complications. In general, the prognosis of this disease is favorable, even in the presence of distant metastases.

Pancreatic acinar cell carcinoma\u2014case report and literature review

BackgroundPancreatic acinar cell carcinoma (ACC) is a rare tumor that constitutes 1% of all pancreatic neoplasms. Pancreatic ACC has unique characteristics in terms of biological behavior, imaging and prognosis.Case presentationThe present study reported two cases of pancreatic ACC confirmed by postoperative pathology and both cases exhibited several different imaging features and laboratory test results. Both cases had approximately 4 cm mass located in uncinate process of pancreas. Dilated intra- and extra-hepatic bile ducts was observed in one case, along with calcification. Heterogeneous enhancement of the tumor was exhibited in both patients with different intensities. Obstructive jaundice, elevated α-fetoprotein and CA 19–9 was found in one case, while the other case had normal liver function and tumor markers.ConclusionsIt was difficult to accurately diagnose pancreatic ACC before the operation despite its unique characteristics. Radical resection was the best treatment modality for resectable pancreatic ACC.

Liver metastasis from pancreatic gastrointestinal stromal tumor presenting 5 years after resection: a rare case with management challenges

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors arising from the gastrointestinal tract. Stromal tumors arising outside the gastrointestinal tract are called extra-gastrointestinal stromal tumors, usually found in omentum, mesentery, retroperitoneum and gallbladder. We report a case of 31 years old male with 3-month history of painless jaundice and undocumented weight loss. Computerized tomography scan showed a well-defined, rounded enhancing lesion in the uncinate process of pancreas measuring 53x49mm compressing the distal common bile duct. Percutaneous biopsy confirmed gastrointestinal stromal tumor. He underwent Whipple’s procedure followed by adjuvant imatinib due to high-risk features. He remained well for a long time and developed oligo-metastasis in liver 5 years after resection while he was still on imatinib. The patient underwent hepatic resection and remains well one year after the second resection on adjuvant treatment. Pancreatic extragastrointestinal stromal tumor involving uncinate process is a rare disease entity. First line of management is negative margin resection followed by adjuvant therapy with imatinib for high-risk tumors. In case of resectable liver metastasis, resection of metastasis followed by multimodal therapy seems to prolong the disease free and overall survival.

Sarcomatoid Carcinoma of the Pancreas: An Unusual Cause of Abdominal Pain

Introduction: Sarcomatoid carcinoma of the pancreas (SCP) is a rare and highly malignant variant of pancreas carcinoma that usually has a very poor prognosis. Epithelial-mesenchymal transition (EMT), a transition between different phenotypes, is regarded as an essential process in tumor progression. When EMT occurs, epithelial tumor cells lose their restricted phenotypes, concomitant with the loss of epithelial characteristics and the acquisition of motile behavior. These changes, which contribute to the transition from an epithelial morphology towards a more mesenchymal fibroblastic phenotype, have been reported to occur in some special tumors, such as cholangiocarcinomas; this change is also defined as sarcomatous change. Case Report: A 37-year-old-female with a history of hypertension presented to the hospital with the chief complaint of epigastric abdominal pain. Her abdominal pain progressively worsened and she came back to the hospital 3 weeks later. She also complained of pale, foul-smelling, floating stools that began 3 days prior to presentation with a decrease in her appetite. Routine laboratory showed: hemoglobin, 12.8 g/dL; WBC, 5,100 cells/uL; platelet, 244,000/mm3; with elevation in AST, ALT, alkaline phosphatase; total bilirubin, 4.0 mg/dL; direct bilirubin, 3.1 mg/dL; and LDH 306 u/L and negative viral markers. Tumor marker assays showed elevated CA19-9. Results: CT abdominal scan showed a hypoechoic mass in the uncinate process of the pancreas (Figure 1) with intrahepatic biliary ductal dilatation and dilation of the proximal common bile duct (CBD). The patient underwent an esophagogastroduodenoscopy with brush biopsies of the CBD with stent placement, as well as, an endoscopic ultrasound fine needle aspiration of the pancreatic mass. The patient underwent a pancreaticoduodenectomy to help diagnosis and remove the tumor. The histopathological diagnosis was as follows: ductal adenocarcinoma with sarcomatoid features with the invasion of the tumor in the extra-pancreatic common bile duct, stage IIB (Figure 2A & 2 B).1372_A.tif Figure 1: CT abdomen with contrast showing the hypoechoic mass in the head of the pancreas with intrahepatic biliary ductal dilatation.1372_B.tif Figure 2: Pathology specimen showing adenosquamous carcinomaDiscussion: Adenosquamous carcinoma of the pancreas is associated with high rates of recurrence and metastasis and carries a poor prognosis, much worse than even that of pancreatic adenocarcinoma, which has a reported median overall survival of no more than 5 months. SCP is a rarely appreciated subset of pancreatic malignancy that does not necessarily portend to a uniformly dismal prognosis.1372_C.tif Figure 3: Sarcomatous changes.

Analysis of 50 cases of solid pseudopapillary tumor of pancreas: Aggressive surgical resection provides excellent outcomes

IntroductionThis study reports the clinicopathological characteristics and the perioperative and long-term treatment outcomes after aggressive surgical resection in solid pseudopapillary tumor (SPT) of the pancreas performed at a high volume center for pancreatic surgery in India. Materials and methodsWe analyzed a prospectively maintained database of the patients operated for SPT at Tata Memorial Hospital, India over a period of 11 years from February 2007 to February 2018. ResultsFifty consecutive patients operated for SPT, during the study period were included. The median age at presentation was 24 years. Majority of the patients (43/50) were female (86%). Disease was predominantly localized in the head and uncinate process of pancreas (66%). Median tumor size was 7.7 cm (Range 1.6–15 cm). Tumor extent was radiologically defined as borderline resectable or locally advanced in 48% (n = 24) patients. Forty-six major pancreatic resections were performed, which included 10 (21%) vascular resections, 2 synchronous liver metastasectomies, 1 multi visceral resection and 5 total pancreaticosplenectomies. Five of these resections were reoperations in patients deemed inoperable on exploration at other centers. R0 resection was achieved in 47 patients (98%). Postoperative major morbidity was 19% and there was no mortality. At a median follow-up of 29 months (Range, 1–121 months), all patients were alive without any recurrence. ConclusionAggressive complete surgical resection of SPT achieves excellent long-term survival. Surgery, especially for large and borderline resectable tumors, can be potentially complex and should be performed at high-volume centers to provide the best chance of cure.

A challenging diagnosis: renal vein leiomyosarcoma, iniatially resembling a retroperitoneal tumor

A 74-year-old male patient was referred to our hospital due to an incidental finding. CT scan and MRI revealed a retroperitoneal mass (4, 5 x 4,6 cm) in the hilum of right kidney. Which was in close contact with the second part of the duodenum, the uncinate process of pancreas, the right hepatic lobe and the infundibulum of gallbladder. CT scan also showed right hydronephrosis and no evidence of metastatic disease. An endoscopic-ultrasound-guided fine-needle-aspiration of the paraduodenal mass was performed, which was reported as a likely mesenchymal tumor. We carried out an open abdominal approach: there was a tumor arising from the right renal vein without involving adjacent structures. A right radical nephrectomy with en-bloc resection of the tumor was performed. Pathological exam revealed a well-differentiated leiomyosarcoma originated from the right renal vein with margins free from tumor. There was less than 50% of necrosis and 9 mitosis per 10 high-power fields. Cells stained positive for smooth muscle actin and desmin; and negative for S100 stain. Conclusion: Renal vein leiomyosarcomas are very difficult to diagnose preoperatively because USE, CT and MRI studies are nonspecific and do not allow an adequate differential diagnosis with other retroperitoneal tumors. The best treatment consists of complete surgical removal of the mass, including nephrectomy. The prognosis is bad and chemotherapy may be offered to cases with metastatic disease.

Application value of multiple imaging techniques in the MDCT combined with assessment of vascular invasion using a tumor-to-vessel contact computed tomography grading system in preoperative evaluation of pancreatic cancer

Application value of multiple imaging techniques in the MDCT combined with assessment of vascular invasion using a tumor-to-vessel contact computed tomography grading system in preoperative evaluation of pancreatic cancer