Primary leiomyosarcoma of the pancreas: report of a case treated by local excision and review of the literature

First described by Ross in 1951, primary pancreatic leiomyosarcoma is a rare mesenchymal tumour of the pancreas, with nonspecific clinical and radiological features and a poor prognosis, if unresectable

Serotonin-Secreting Neuroendocrine Tumours of the Pancreas

Background: Serotonin-secreting pancreatic neuroendocrine tumours (5-HT-secreting pNETs) are very rare, and characterised by high urinary 5-hydroxyindole-acetic acid (5-HIAA) levels (or high serum 5-HT levels). Methods: Patients with 5-HT-secreting pancreatic neoplasms observed in our unit (1986-2015) were included. Diagnosis was based on urinary 5-HIAA or serum 5-HT levels. Results: Seven patients were enrolled (4 M/3 F), with a median age of 64 (range 38-69) years. Two patients had a carcinoid syndrome. Serum 5-HT was elevated in four patients. Urinary 5-HIAA levels were positive in six patients. The median tumour size was 4.0 (range 2.5-10) cm. All patients showed liver metastases at diagnosis. None underwent resective surgery; lymph node/liver biopsies were taken. Six lesions were well-differentiated tumours and one a poorly differentiated carcinoma (Ki67 range 3.4-70%). All but one patient received chemotherapy. Four patients received somatostatin analogues; three patients underwent ablation of liver metastases. One patient is alive with disease 117 months after observation. All the others died from disease progression after a follow-up within 158 months. Conclusions: Primary 5-HT-secreting pNETs are mostly metastatic to the liver; patients are not amenable to resective surgery. Despite high 5-HIAA urinary levels, few patients present with carcinoid syndrome. A five-year survival rate of 42.9% may be achieved with multimodal treatment

Etude de modèles de dimères et partitions quantiques sur réseaux hexagonaux

The quantum dimer models (QDM's) have a series of interesting behaviors, such as topological order and spin liquid phases. In this thesis, we study these models for an honeycomb lattice, and also their equivalence with the partition problems, a subject of the domain of combinatorics. Firstly, we study the RK model, for which the question on whenever one of its phases is gapped or not was still open. We describe an Monte-Carlo algorithm that allows to, among other results, access this gap directly. Secondly, we propose a generalization of this model. We find a more complex phase diagram, with phase transitions between the different topological sectors, and compatible with the Cantor deconfinement. Thirdly, we study the application of the RK model to honeycomb lattices associated to the planar partition problems. This imposes new boundary conditions, and we find a new model behavior. We also propose a méthod that uses the properties of the partition problem's configuration space to reduce the complexity of the QDM. Finally, we modelize the problems of classical crystal corner growth and melting with the formalism of the partition problems, finding a smooth transition between the limit interfaces of type "amoebae" and the arctic circle.Les modèles de dimères quantiques (QDM's) ont une série de comportements intéressants, comme de l'ordre topologique et des phases de liquides de spin. Dans cette thèse, nous explorons ces modèles pour un réseaux hexagonal, ainsi que leur équivalence aux problèmes de partitions, un sujet qui fait partie du domaine de la combinatoire. Premièrement, nous étudions le modèle RK, pour lequel la question sur la présence d'une phase avec un gap non-nul restait encore ouverte. Nous décrivons un algorithme Monte-Carlo qui nous permet, entre autres résultats, d'accéder directement au gap du système. Deuxièmement, nous proposons une généralisation de ce modèle. Nous trouvons un diagramme de phase beaucoup plus complexe, avec des transitions de phase entre différents secteurs topologiques, et compatible avec le déconfinement de Cantor. Troisièmement, nous étudions l'application du modèle RK à des réseaux hexagonales associés à des problèmes de partitions planaires. Cela impose des nouvelles conditions de bord, et nous trouvons un nouveau comportement du modèle. Nous proposons aussi une méthode que utilise les propriétés de l'espace de configurations des problèmes de partitions pour réduire la complexité du QDM.Finalement, nous modélisons les problèmes de croissance et effondrement de coin de cristaux classiques dans le cadre des problèmes de partition, trouvant une transition souple entre des interfaces limites du type "amibe" et le cercle arctique

Pancreatic solid pseudopapillary neoplasm in male patients: systematic review with three new cases

Pancreatic solid pseudopapillary neoplasm (pSPN) is a rare exocrine neoplasm, which generally occurs in young women. This study analyses the clinical characteristics of pSPN in male patients through a systematic review of the literature, adding three new cases from our institution. We reviewed our experience in Pspns, and we performed a systematic review of pSPN of all articles published in English in PubMed and SCOPUS from 1980. Using the final included articles, we evaluated clinic-pathological features, surgical treatment and prognosis of male patients affected by pSPN. From the literature review and our cases, we collected 246 male patients with a proven pSPN. Mean age was 34.3 (range 4\u201378) years, with 26.2% patients younger than 18\ua0years. Patients were asymptomatic in 35.9% of cases, despite a mean tumour size of 6.3\ua0cm. In 63.7% of cases, the pSPN was located in the body\u2013tail region. Distant metastases were reported at diagnosis in only 10 (4.1%) patients. A correct pre-operative diagnosis (including cytopathology) was provided in 53.6% of patients, with only 40 fine-needle aspiration/biopsy performed. Standard pancreatic resections represented 90.4% of surgical procedures. Beta-catenin and progesterone receptors were positive at immunostaining in 100% and 77.8% of cases, respectively. Fourteen (7.2%) patients relapsed after a mean disease-free survival of 43.1\ua0months. After a mean follow-up of 47 (range 4\u2013180) months, 89.5% of patients were alive and disease-free. Although rare, when dealing with a solid-cystic pancreatic mass, even in asymptomatic male patients, a pSPN should be considered as a possible diagnosis

Intraductal papillary mucinous neoplasms of the pancreas: Uncommon imaging presentation, evolution and comparison of guidelines

Pancreatic cystic lesions are often asymptomatic, incidentally detected and include a range of entities with varying degrees of concern for malignancy. Among these, intraductal papillary mucinous neoplasms (IPMN) are considered premalignant pancreatic lesions, with a broad pathological spectrum ranging from lesions without dysplasia, which can be managed conservatively, to malignant lesions that require surgical resection. The increasing use of CT and MRI has led to increased recognition of this entity incidentally, with branch-duct IPMN representing the most common subtype and the most challenging lesions in terms of patient management. The main imaging modality involved in diagnosis and surveillance of IPMN is MRI. Radiologists play an important role in the management of patients with IPMN, including lesion detection, characterization, follow-up and prognostication, allowing early MRI identification of features that are concerning for malignancy. The main aim of this pictorial review is to illustrate MRI features of IPMN and to discuss risk stratification scores based on different guidelines, with a main focus on branch-duct IPMN. The secondary aims include the presentation of common and uncommon imaging evolution of BD-IPMN as well as the discussion on current controversies on the appropriate management of IPMN

A Plea for Surgery in Pancreatic Metastases from Renal Cell Carcinoma: Indications and Outcome from a Multicenter Surgical Experience

Pancreatic metastases from renal-cell carcinoma (RCC-PMs) are rare. Surgery may play a role in improving overall (OS) and disease-free survival (DFS)

Multifunzionalità dell'azienda agricola

Quaderni di ricerca Ires ; n.111- Indice #3- Introduzione #5- Declinazioni e definizioni della multifunzionalità #7- Dinamica della multifunzionalità e sostenibilità dello sviluppo rurale #13- Le relazioni tra normativa e multifunzionalità #17- Le esternalità dell'agricoltura #23- Gli strumenti dello sviluppo rurale e le esternalità #35- Tipologie territoriali in Piemonte in funzione della multifunzionalità dell'azienda agricola #43- Multifunzionalità da diversificazione: i casi di studio #51- Considerazioni finali #57- Bibliografia #6

SLC22A3 polymorphisms do not modify pancreatic cancer risk, but may influence overall patient survival

Expression of the solute carrier (SLC) transporter SLC22A3 gene is associated with overall survival of pancreatic cancer patients. This study tested whether genetic variability in SLC22A3 associates with pancreatic cancer risk and prognosis. Twenty four single nucleotide polymorphisms (SNPs) tagging the SLC22A3 gene sequence and regulatory elements were selected for analysis. Of these, 22 were successfully evaluated in the discovery phase while six significant or suggestive variants entered the validation phase, comprising a total study number of 1,518 cases and 3,908 controls. In the discovery phase, rs2504938, rs9364554, and rs2457571 SNPs were significantly associated with pancreatic cancer risk. Moreover, rs7758229 associated with the presence of distant metastases, while rs512077 and rs2504956 correlated with overall survival of patients. Although replicated, the association for rs9364554 did not pass multiple testing corrections in the validation phase. Contrary to the discovery stage, rs2504938 associated with survival in the validation cohort, which was more pronounced in stage IV patients. In conclusion, common variation in the SLC22A3 gene is unlikely to significantly contribute to pancreatic cancer risk. The rs2504938 SNP in SLC22A3 significantly associates with an unfavorable prognosis of pancreatic cancer patients. Further investigation of this SNP effect on the molecular and clinical phenotype is warranted

Genome-wide association study identifies an early onset pancreatic cancer risk locus

Early onset pancreatic cancer (EOPC) is a rare disease with a very high mortality rate. Almost nothing is known on the genetic susceptibility of EOPC, therefore we performed a genome-wide association study (GWAS) to identify novel genetic variants specific for patients diagnosed with pancreatic ductal adenocarcinoma (PDAC) at younger ages. In the first phase, conducted on 821 cases with age of onset ≤60 years, of whom 198 with age of onset ≤50, and 3227 controls from PanScan I-II, we observed four SNPs (rs7155613, rs2328991, rs4891017 and rs12610094) showing an association with EOPC risk (P < 1x10-4 ). We replicated these SNPs in the PANcreatic Disease ReseArch (PANDoRA) consortium and used additional in silico data from PanScan III and PanC4. Among these four variants rs2328991 was significant in an independent set of 855 cases with age of onset ≤60 years, of whom 265 with age of onset≤50, and 4142 controls from the PANDoRA consortium while in the in silico data we observed no statistically significant association. However, the resulting meta-analysis supported the association (P = 1.15x10-4 ). In conclusion we propose a novel variant rs2328991 to be involved in EOPC risk. Even though it was not possible to find a mechanistic link between the variant and the function, the association is supported by a solid statistical significance obtained in the largest study on EOPC genetics present so far in the literature. This article is protected by copyright. All rights reserved

Genetic and non-genetic risk factors for early-onset pancreatic cancer

Early-onset pancreatic cancer (EOPC) represents 5-10% of all pancreatic ductal adenocarcinoma (PDAC) cases, and the etiology of this form is poorly understood. It is not clear if established PDAC risk factors have the same relevance for younger patients. This study aims to identify genetic and non-genetic risk factors specific to EOPC.A genome-wide association study was performed, analysing 912 EOPC cases and 10 222 controls, divided into discovery and replication phases. Furthermore, the associations between a polygenic risk score (PRS), smoking, alcohol consumption, type 2 diabetes and PDAC risk were also assessed.Six novel SNPs were associated with EOPC risk in the discovery phase, but not in the replication phase. The PRS, smoking, and diabetes affected EOPC risk. The OR comparing current smokers to never-smokers was 2.92 (95% CI 1.69-5.04, P = 1.44 × 10-4). For diabetes, the corresponding OR was 14.95 (95% CI 3.41-65.50, P = 3.58 × 10-4).In conclusion, we did not identify novel genetic variants associated specifically with EOPC, and we found that established PDAC risk variants do not have a strong age-dependent effect. Furthermore, we add to the evidence pointing to the role of smoking and diabetes in EOPC