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Bolzano, Italy

Rossi S.,Treviso General Hospital | Gasparotto D.,Italian National Cancer Institute | Toffolatti L.,Treviso General Hospital | Pastrello C.,Italian National Cancer Institute | And 14 more authors.
American Journal of Surgical Pathology | Year: 2010

Although Gastrointestinal stromal tumors (GISTs) affect about 0.0014% of the population, GISTs smaller than 1cm (microGISTs) are detectable in about 20% to 30% of elderly individuals. This suggests that microGISTs likely represent premalignant precursors that evolve only in a minute fraction of cases toward overt GISTs. We sought histopathologic and molecular explanations for the infrequent clinical progression in small GISTs. To investigate the mechanisms of GIST progression and identify subsets with differential malignant potential, we carried out a thorough characterization of 170 GISTs <2cm and compared their KIT/PDGFRA status with overt GISTs. The proliferation was lower in microGISTs compared with GISTs from 1 to 2cm (milliGISTs). In addition, microGISTs were more frequently incidental, gastric, spindle, showed an infiltrative growth pattern, a lower degree of cellularity, and abundant sclerosis. The progression was limited to 1 ileal and 1 rectal milliGISTs. KIT/PDGFRA mutations were detected in 74% of the cases. The overall frequency of KIT/PDGFRA mutation and, particularly, the frequency of KIT exon 11 mutations was significantly lower in small GISTs compared with overt GISTs. Five novel mutations, 3 in KIT (p.Phe506Leu, p.Ser692Leu, p.Glu695Lys) 2 in PDGFRA (p.Ser847X, p.Ser667Pro), plus 4 double mutations were identified. Small GISTs share with overt GIST KIT/PDGFRA mutation. Nevertheless, microGISTs display an overall lower frequency of mutations, particularly canonical KIT mutations, and also carry rare and novel mutations. These molecular features, together with the peculiar pathologic characteristics, suggest that the proliferation of these lesions is likely sustained by weakly pathogenic molecular events, supporting the epidemiologic evidence that microGISTs are self-limiting lesions. © 2010 by Lippincott Williams & Wilkins. Source

Nuzzo G.,Catholic University of the Sacred Heart | Giuliante F.,Catholic University of the Sacred Heart | Ardito F.,Catholic University of the Sacred Heart | Giovannini I.,Catholic University of the Sacred Heart | And 26 more authors.
Archives of Surgery | Year: 2012

Objective: To evaluate improvements in operative and long-term results following surgery for hilar cholangiocarcinoma. Design: Retrospective multicenter study including 17 Italian hepatobiliary surgery units. Patients: A total of 440 patients who underwent resection for hilar cholangiocarcinoma from January 1, 1992, through December 31, 2007. Main Outcome Measures: Postoperative mortality, morbidity, overall survival, and disease-free survival. Results: Postoperative mortality and morbidity after liver resection were 10.1% and 47.6%, respectively. At multivariate logistic regression, extent of resection (rightor right extended hepatectomy) and intraoperative blood transfusion were independent predictors of postoperative mortality (P=.03 and P=.006, respectively); in patients with jaundice, mortality was also higher without preoperative biliary drainage than with biliary drainage (14.3% vs 10.7%). During the study period, there was an increasingly aggressive approach, with more frequent caudate lobectomies, vascular resections, and resections for advanced tumors (T stage of 3 or greater and tumors with poor differentiation). Despite the aggressive approach, the blood transfusion rate decreased from 81.0% to 53.2%, and mortality slightly decreased from 13.6% to 10.8%. Median overall survival significantly increased from 16 to 30 months (P=.05). At multivariate analysis, R1 resection, lymph node metastases, and T stage of 3 or greater independently predicted overall and disease-free survival. Conclusions: Surgery for hilar cholangiocarcinoma has improved with decreased operative risk despite a more aggressive surgical policy. Long-term survival after liver resection has also increased, despite the inclusion of cases with more advanced hilar cholangiocarcinoma. Preoperative biliary drainage was a safe strategy before right or right extended hepatectomy in patients with jaundice. Pathologic factors independently predicted overall and disease-free survival at multivariate analysis. ©2012 American Medical Association. All rights reserved. Source

Mautone A.,Bolzano General Hospital | Brown J.R.,Dartmouth Hitchcock Medical Center
Journal of Interventional Cardiology | Year: 2010

Contrast-induced nephropathy (CIN) is an acute and severe complication after contrast media administration. The most important step in preventing CIN is identifying high-risk patients. In this review, we evaluate and summarize the evidence regarding the CIN prophylaxis, including the withdrawal of the potentially nephrotoxic drugs, hydration by isotonic solution or NaHCO 3, pharmaceutical treatment with N-acetylcysteine (N-AC), adenosine antagonists, ascorbic acid, renal procedures including hemofiltration or dialysis, and to the optimal use of the contrast. We suggest it is possible to reduce the burden of CIN by carefully incorporating these recommendations. After review of published literature in this field, we conclude that the cornerstone of the CIN prevention should be combination of hydration (normal saline or NaHCO3) and the use of N-AC. © 2010, Wiley Periodicals, Inc. Source

Scarpa M.,University of Padua | Buffone E.,Central Hospital | La Marca P.L.,Hospital of Malcesine | Campello M.,Bolzano General Hospital | Rampazzo A.,University of Padua
Journal of Pediatric Rehabilitation Medicine | Year: 2010

We describe the cases of two adult sisters recently diagnosed with the attenuated form of mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome). MPS VI is a rare, clinically heterogeneous lysosomal storage disorder that is characterized by a deficiency in the glycosaminoglycan-degrading enzyme arylsulfatase B. Both cases had been misdiagnosed for over 30 years despite the presence of several characteristics of the disease, including short stature (mild), coarse facial features, skeletal dysmorphisms, carpal tunnel syndrome, heart valve disease, and spinal cord compression, which together are suggestive of a lysosomal storage disease. Awareness about the clinical features of MPS VI should be communicated amongst treating neurologists, rheumatologists and other specialists who are involved in the healthcare decisions of these patients with presenting symptoms, so they can refer them to specialized centers for proper diagnosis and treatment. © 2010 - IOS Press and the authors. All rights reserved. Source

Simone G.,Regina Elena Cancer Institute | De Nunzio C.,University of Rome La Sapienza | Ferriero M.,Regina Elena Cancer Institute | Cindolo L.,Padre Pio da Pietrelcina Hospital | And 29 more authors.
European Journal of Surgical Oncology | Year: 2016

Aim: Although extensively addressed in US registries, the utilization rate of Partial Nephrectomy has been poorly addressed in European settings. Our aim is to evaluate the impact of hospital volume on the use of PN for cT1 renal tumors. Methods: 2526 patients with cT1N0M0 renal tumors treated with either PN or radical nephrectomy at 10 European centres in the last decade were included in the analysis. Joinpoint regression analysis was used to identify significant changes over time in linear slope of the trend for each center. The correlation between yearly caseload and the slopes was assessed with the non-parametric Spearman test. Coincident pairwise tests and regression analyses were used to generate and compare the trends of high-volume (HV), mid-volume (MV) and low-volume (LV) groups. Results: Yearly caseload was significantly associated with increased use of PN (R = 0.69, p = 0.028). The utilization rate of PN was stable at LV centres (p = 0.67, p = 0.7, p = 0.76, for cT1, cT1a, and cT1b tumors, respectively), while increased significantly at MV (p = 0.002, 0.0005 and 0.007, respectively) and HV centers (all p < 0.0001). Regression analysis confirmed the trends for HV and MV as significantly different from those observed in LV centres (all p ≤ 0.002) and highlighted significant differences also between MV and HV centres (all p ≤ 0.03). Conclusions: We confirmed the association between caseload and the use of PN for cT1 tumors. Our findings suggest that a minimum caseload might turn the tide also in LV centres while a selective referral to HV centers for cT1b tumors should be considered. © 2016 Elsevier Ltd. Source

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