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https://www.readbyqxmd.com/read/29676474/a-rare-case-of-ewing-s-sarcoma-primitive-neuroectodermal-tumor-metastasizing-to-left-atrium-through-left-inferior-pulmonary-vein
#1
Suraj K Kulkarni, Nagaraj Moorthy, Shankar Somanna, Manjunath Cholenahally Nanjappa
Cardiac metastasis of Ewing's sarcoma is exceedingly rare. A 40-year-old male was admitted with complaints of nonproductive cough, exertional dyspnea, and fatigue since 4 months with a history of abdominal wall swelling which was excised and proven to be Ewing's sarcoma/primitive neuroectodermal tumor (PNET). A transthoracic echocardiography demonstrated inhomogeneous mass located posterior to the left ventricle and a solitary mass protruding into the left atrium through the left inferior pulmonary vein. Due to accompanying pulmonary metastasis and possible poor outcome of the surgery, surgical resection was not considered...
April 20, 2018: Echocardiography
https://www.readbyqxmd.com/read/29676370/primary-primitive-neuroectodermal-tumor-of-the-kidney
#2
Sunil Vitthalrao Jagtap, Pradnya Pandurang Kale, Anil Huddedar, Atul Bhanudas Hulwan, Swati S Jagtap
Renal primitive neuroectodermal tumor (PNET) is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient. We report a 45-year-old male having complaints of pain in the abdomen, hematuria, and mass in the abdomen for 6 months. Abdominal and pelvic sonography finding showed cystic-solid, right renal mass suggestive of malignancy. On histopathology diagnosed as malignant small round blue cell tumor suggestive of primitive neuroectodermal tumor...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29676369/a-mesenteric-primary-peripheral-ewing-s-sarcoma-primitive-neuroectodermal-tumor-with-molecular-cytogenetic-analysis-report-of-a-rare-case-and-review-of-literature
#3
Yi-Shu Liao, I-Han Chiang, Hong-Wei Gao
Rare cases of Ewing's sarcoma/primitive neuroectodermal tumors (EWS/PNETs) arising from mesenteric tissue have been reported. This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm × 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum. Surgical resection was completed during exploratory laparotomy. Immunohistochemically, the tumor cells revealed CD99, friend leukemia virus integration-1 and NKX2...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29655834/metformin-use-associates-with-longer-progression-free-survival-of-patients-with-diabetes-and-pancreatic-neuroendocrine-tumors-receiving-everolimus-and-or-somatostatin-analogues
#4
Sara Pusceddu, Claudio Vernieri, Massimo Di Maio, Riccardo Marconcini, Francesca Spada, Sara Massironi, Toni Ibrahim, Maria Pia Brizzi, Davide Campana, Antongiulio Faggiano, Dario Giuffrida, Maria Rinzivillo, Sara Cingarlini, Francesca Aroldi, Lorenzo Antonuzzo, Rossana Berardi, Laura Catena, Chiara De Divitiis, Paola Ermacora, Vittorio Perfetti, Annalisa Fontana, Paola Razzore, Carlo Carnaghi, Maria Vittoria Davì, Carolina Cauchi, Marilina Duro, Sergio Ricci, Nicola Fazio, Federica Cavalcoli, Alberto Bongiovanni, Anna La Salvia, Nicole Brighi, Annamaria Colao, Ivana Puliafito, Francesco Panzuto, Silvia Ortolani, Alberto Zaniboni, Francesco Di Costanzo, Mariangela Torniai, Emilio Bajetta, Salvatore Tafuto, Silvio Ken Garattini, Daniela Femia, Natalie Prinzi, Laura Concas, Giuseppe Lo Russo, Massimo Milione, Luca Giacomelli, Roberto Buzzoni, Gianfranco Delle Fave, Vincenzo Mazzaferro, Filippo de Braud
BACKGROUND & AIMS: Metformin seems to have anti-cancer effects. However, it is not clear whether use of glycemia and metformin affect outcomes of patients with advanced pancreatic neuroendocrine tumors (pNETs). We investigated the association between glycemia and progression-free survival (PFS) of patients with NETs treated with everolimus and/or somatostatin analogues, as well as the association between metformin use and PFS time. METHODS: We performed a retrospective analysis of 445 patients with advanced pNET treated at 24 medical centers in Italy, from 1999 through 2015...
April 12, 2018: Gastroenterology
https://www.readbyqxmd.com/read/29643685/unilateral-brown-fat-suppression-on-fdg-pet-ct-detecting-sympathetic-denervation
#5
Saurabh Arora, Nishikant Avinash Damle, K Sreenivasa Reddy, Girish Kumar Parida, Abhinav Singhal, Sreedharan Thankarajan Arunraj, Chandrasekhar Bal, Roma Singh, Shobhana Raju, Dhritiman Chakraborty
We present here a case of primitive neuroectodermal tumor (PNET) who initially presented with involvement of the right 3rd rib and underwent neoadjuvant chemotherapy, rib excision, and adjuvant chemoradiotherapy and later underwent posterolateral thoracotomy, pleural nodule excision, and the right 11th rib metastatic lesion excision. Follow-up 18 F-FDG PET/CT/computed tomography revealed unilateral brown fat suppression in the form of decreased metabolic uptake in the ipsilateral cervical, axillary, and paravertebral brown fat as compared to metabolically active contralateral brown fat, likely due to paravertebral sympathetic chain damage...
April 2018: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/29629846/molecular-profiling-of-pancreatic-neuroendocrine-tumors-pnets-and-the-clinical-potential
#6
Massimiliano Camilli, Konstantinos Papadimitrio, Amanda Nogueira, Lorena Incorvaia, Antonio Galvano, Federica D'Antonio, Jose Ferri, Daniele Santini, Nicola Silvestris, Antonio Russo, Marc Peeters, Christian Rolfo
Pancreatic neuroendocrine tumors (pNETs) represent a small part of pancreatic neoplasms, and the knowledge about their indolent clinical course remains a subject of investigation. They occur sporadically or as part of familial cancer syndromes and are classified by WHO in 3 categories. There is ongoing research to understand their molecular profiling and leading mutations. Areas covered: The aim of this review is to clarify the overall aspects of tumorigenesis, to expose the latest developments in understanding the course of the disease and the possible therapeutic implications of these...
April 9, 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29629321/laparoscopic-distal-pancreatectomy-for-neuroendocrine-tumors-of-the-pancreas
#7
In Gun Hyun, Yoo-Seok Yoon, Ho-Seong Han, Jai Young Cho, YoungRok Choi, Sungho Kim, Kil Hwan Kim
The incidence of incidentally discovered nonfunctioning pancreatic neuroendocrine tumors (PNETs) is increasing because of the widespread use of radiologic imaging studies. Due to their mostly small size, PNETs in the body and tail of the pancreas are suited for laparoscopic surgery. This video described our technique of laparoscopic distal pancreatectomy with and without preservation of spleen and splenic vessels for PNET. The decision on preservation of spleen and splenic vessels was made according to the relative location of tumors to the splenic vessels...
February 2018: Gland Surgery
https://www.readbyqxmd.com/read/29629320/combined-associating-liver-partition-and-portal-vein-ligation-for-staged-hepatectomy-alpps-followed-by-left-trisectionectomy-and-whipple-operation-for-pnet
#8
Ren Ji, Shi Zuo, Siyuan Qiu, Ping Li, Albert Chan, William Sharr, Chung Mau Lo
Pancreatic neuroendocrine tumor (PNET) is slow-growing, and account only for 2% of all pancreatic primary tumors. Surgical resection is still the only curative treatment for PNET patients. Unfortunately, most of PNETs was found with unresectable multiple liver metastases and extrahepatic metastasis as their characteristics of non-functional and asymptomatic. With advances in liver surgery in these years, especially combined associating liver partition and portal vein ligation for staged hepatectomy (ALPPS), provide a new curative surgical treatment for PNET with liver metastases patient...
February 2018: Gland Surgery
https://www.readbyqxmd.com/read/29629319/liver-transplantation-for-unresectable-pancreatic-neuroendocrine-tumors-with-liver-metastases-in-an-era-of-transplant-oncology
#9
REVIEW
Keita Shimata, Yasuhiko Sugawara, Taizo Hibi
Patients with pancreatic neuroendocrine tumors (pNETs) very often present with a metastatic disease at the first diagnosis. Liver transplantation (LT) for unresectable pNET with liver metastases (pNETLM) has been described to prolong survival in highly selected patients, although outcomes were worse than those of patients who underwent LT for gastrointestinal NETLM (GI-NETLM). In this review, several proposed criteria are described with their rationale and controversies. Most of the data used to establish these criteria do not reflect the recent improvements of non-surgical treatments that has changed the landscape of treatment for pNETs, including the development of peptide receptor radionuclide therapy and molecular-targeted agents (sunitinib and everolimus)...
February 2018: Gland Surgery
https://www.readbyqxmd.com/read/29629318/role-of-surgery-in-pancreatic-neuroendocrine-tumor
#10
REVIEW
Kai Pun Wong, Julian Shun Tsang, Brian Hung-Hin Lang
Pancreatic neuroendocrine tumours (PNETs) are rare. They are generally accepted to be slow-growing and have an indolent course. These tumours can be non-functioning or functioning, consisting of a biochemically heterogeneous group of tumours including insulinomas, gastrinomas, carcinoids and glucagonomas. Although surgery remains the mainstay of treatment, controversy still exists especially for non-functioning tumours <2 cm in size. Whether these should be resected or undergo intensive surveillance remains unclear...
February 2018: Gland Surgery
https://www.readbyqxmd.com/read/29629316/management-of-functional-neuroendocrine-tumors-of-the-pancreas
#11
REVIEW
Kjell Öberg
Pancreatic neuroendocrine tumors (pNETs) constitute a heterogenous group of malignancies with varying clinical presentation, tumor biology and prognosis. The incidence of pNETs has steadily increased during the last decades with an estimated incidence 2012 of 4.8/100,000. Recent whole genome sequencing of pNETs has demonstrated mutations in the DNA repair genes MUTYH and point mutations and gene fusions in four main pathways from chromatin remodeling, DNA damage repair, activation of mechanistic target of rapamycin (mTOR) signaling and the telomere maintenance...
February 2018: Gland Surgery
https://www.readbyqxmd.com/read/29629315/surgical-resection-of-neuroendocrine-tumors-of-the-pancreas-pnets-by-minimally-invasive-surgery-the-laparoscopic-approach
#12
REVIEW
Tomoki Shirota, Yuichi Nagakawa, Yatsuka Sahara, Chie Takishita, Yosuke Hijikata, Yuichi Hosokawa, Tetsushi Nakajima, Hiroaki Osakabe, Kenji Katsumata, Akihiko Tsuchida
Neuroendocrine tumors of the pancreas (pNETs) are a rare group of neoplasms that originate from the endocrine portion of the pancreas. Tumors that either secrete or do not secrete compounds, resulting in symptoms, can be classified as functioning and non-functioning pNETs, respectively. The prevalence of such tumors has recently increased due to the use of more sensitive imaging techniques, such as multidetector computed tomography, magnetic resonance imaging and endoscopic ultrasound. The biological behavior of pNETs varies widely from indolent, well-differentiated tumors to those that are far more aggressive...
February 2018: Gland Surgery
https://www.readbyqxmd.com/read/29629229/ambiguity-in-the-dural-tail-sign-on-mri
#13
Ramesh S Doddamani, Rajesh K Meena, Dattaraj Sawarkar
Background: Meningiomas give rise to the dural tail sign (DTS) on contrast-enhanced magnetic resonance imaging (CEMRI). The presence of DTS does not always qualify for a meningioma, as it is seen in only 60-72% of cases. This sign has been described in various other lesions like lymphomas, metastasis, hemangiopericytomas, schwannomas and very rarely glioblastoma multiforme (GBM). The characteristics of dural-based GBMs are discussed here, as only eleven such cases are reported in the literature till date...
2018: Surgical Neurology International
https://www.readbyqxmd.com/read/29619528/grade-2-pancreatic-neuroendocrine-tumors-overbroad-scope-of-ki-67-index-according-to-mri-features
#14
Yabin Hu, Shengxiang Rao, Xiaolin Xu, Yibo Tang, Mengsu Zeng
PURPOSE: To evaluate the value of MR imaging features in stratifying Grade 2 (G2) pancreatic neuroendocrine tumors (PNETs) using the 5% cut-off value of the Ki-67 index as reference standards. MATERIALS AND METHODS: Between January 2010 and October 2016, 41 G2 PNET patients (One patient had 3 tumors) with preoperative MR imaging were included. Tumor grading was based on the revised 2016 World Health Organization classification of PNETs. MR imaging features included size, shape, consistency, T1-w and T2-w signal intensities, enhancement pattern, apparent diffusion coefficient (ADC) ratios (tumor/normal pancreatic parenchyma)...
April 4, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29618015/high-fear-of-disease-occurrence-is-associated-with-low-quality-of-life-in-patients-with-multiple-endocrine-neoplasia-type-1-men1-results-from-the-dutch-men1-study-group
#15
Rachel S van Leeuwaarde, Carolina R C Pieterman, Eveline M A Bleiker, Olaf M Dekkers, Anouk N van der Horst-Schrivers, Ad R Hermus, Wouter W de Herder, Madeleine L Drent, Peter H Bisschop, Bas Havekes, Menno R Vriens, Gerlof D Valk
Objective: Multiple Endocrine Neoplasia type 1 (MEN1) is a hereditary disease characterized by a high risk of developing primary hyperparathyroidism, duodenopancreatic neuroendocrine tumors (dpNETs) and pituitary tumors (PIT). Up to now, it is unclear if having MEN1 leads to psychological distress because of fear of disease occurrence (FDO), thereby potentially affecting quality of life. Design: A cross-sectional study was performed using the Dutch MEN1 cohort. All patients received the Cancer Worry Scale (score ≥ 14 reflecting high FDO), the SF-36 Health Related Quality of Life questionnaire (SF-36) and questions on sociodemographic and medical history...
March 30, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29616127/therapeutic-approach-in-glioblastoma-multiforme-with-primitive-neuroectodermal-tumor-components-case-report-and-review-of-the-literature
#16
Arsela Prelaj, Sara Elena Rebuzzi, Giovanni Caffarena, Julio Rodrigo Giròn Berrìos, Silvia Pecorari, Carmela Fusto, Alessandro Caporlingua, Federico Caporlingua, Annamaria Di Palma, Fabio Massimo Magliocca, Maurizio Salvati, Silverio Tomao, Vincenzo Bianco
Glioblastoma multiforme (GBM) is the most common and aggressive malignant glioma that is treated with first-line therapy, using surgical resection followed by local radiotherapy and concomitant/adjuvant temozolomide (TMZ) treatment. GBM is characterised by a high local recurrence rate and a low response to therapy. Primitive neuroectodermal tumour (PNET) of the brain revealed a low local recurrence rate; however, it also exhibited a high risk of cerebrospinal fluid (CSF) dissemination. PNET is treated with surgery followed by craniospinal irradiation (CSI) and platinum-based chemotherapy in order to prevent CSF dissemination...
May 2018: Oncology Letters
https://www.readbyqxmd.com/read/29615284/external-beam-radiotherapy-in-the-treatment-of-gastroenteropancreatic-neuroendocrine-tumours-a-systematic-review
#17
D L Chan, R Thompson, M Lam, N Pavlakis, J Hallet, C Law, S Singh, S Myrehaug
AIMS: External beam radiotherapy (EBRT) is infrequently used to treat gastroenteropancreatic neuroendocrine tumours (GEPNETS), with little published data to date. We carried out a systematic review to assess the activity of EBRT for GEPNETS. MATERIALS AND METHODS: Major databases were searched for papers including at least five patients treated with contemporary EBRT techniques. Eligible studies underwent dual independent review. The primary end points were response rate for lesions treated with definitive intent and recurrence-free survival for primary lesions treated with neoadjuvant or adjuvant intent...
March 31, 2018: Clinical Oncology: a Journal of the Royal College of Radiologists
https://www.readbyqxmd.com/read/29599907/epidrug-induced-upregulation-of-functional-somatostatin-type-2-receptors-in-human-pancreatic-neuroendocrine-tumor-cells
#18
Marije J Veenstra, Peter M van Koetsveld, Fadime Dogan, William E Farrell, Richard A Feelders, Steven W J Lamberts, Wouter W de Herder, Giovanni Vitale, Leo J Hofland
Somatostatin receptors are a pivotal target for treatment of pancreatic neuroendocrine tumors (pNET), either with somatostatin analogues (SSA) or radiolabeled SSA. The highest affinity target for the most commonly used SSA is the somatostatin receptor type 2 ( sst 2 ). An important factor that may complicate treatment efficacy, is the variable number of receptors expressed on pNETs. Gene expression is subject to complex regulation, in which epigenetics has a central role. In this study we explored the possible role of epigenetic modifications in the variations in sst 2 expression levels in two human pNET cell lines, BON-1 and QGP-1...
March 13, 2018: Oncotarget
https://www.readbyqxmd.com/read/29594173/acinar-cell-cystadenoma-a-rarity-in-advanced-von-hippel-lindau-disease-a-case-report
#19
Tim Fahlbusch, Andrea Tannapfel, Waldemar Uhl, Chris Braumann
Background: Von Hippel-Lindau (VHL) disease may occur at various localisations which can be both intra- and extrapancreatic as well as challenging to diagnose by medical imaging. Case Report: A positron emission tomography/magnetic resonance imaging in a 40-year-old woman was performed to monitor a haemangioblastoma. Additionally, it showed findings which were considered to be a pancreatic neuroendocrine tumour (pNET) and retroumbilical metastasis. The suspected metastasis was laparoscopically resected; however, pathological evaluation did not lead to a clear categorisation...
February 2018: Visceral Medicine
https://www.readbyqxmd.com/read/29576279/prognostic-factors-and-survival-after-surgical-resection-of-pancreatic-neuroendocrine-tumor-with-validation-of-established-and-modified-staging-systems
#20
Nikolaos Benetatos, James Hodson, Ravi Marudanayagam, Robert P Sutcliffe, John R Isaac, John Ayuk, Tahir Shah, Keith J Roberts
BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) display wide heterogeneity with highly variable prognosis. This study aimed to identify variables related to survival after surgical resection of PNET. METHODS: A total of 143 patients were identified from a prospectively maintained database. Patient characteristics were analyzed and prognostic factors for overall survival and progression-free survival were evaluated. The WHO, ENETS and AJCC scoring systems were applied to the cohort, and their ability to predict patient outcomes were compared...
March 6, 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
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