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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/29714464/insulin-like-growth-factor-1-receptor-expression-in-pediatric-tumors-a-comparative-immunohistochemical-study
#1
Resul Karakuş, Esra Karakuş, Suna Emir, Ayper Kaçar, Derya Özyörük
Background/aim: Insulin-like growth factor-1 receptor (IGF-1R) is a pivotal receptor tyrosine kinase involved in the cell cycle and malignant tumor transformation. It is differentially expressed in various types of tumors. We aimed to determine the expression of IGF- 1R in different pediatric tumors and to shed light on possible new indications of anti-IGF-1R treatment approaches. Materials and methods: A total of 147 specimens were analyzed according to their expression of IGF-1R. Specimens included those from rhabdomyosarcomas, Wilms tumors, Ewing sarcoma/primitive neuroectodermal tumors, peripheral neuroblastic tumors, acute lymphoblastic lymphoma, Hodgkin lymphoma, Burkitt lymphoma, retinoblastoma, pleuropulmonary blastoma, Langerhans cell histiocytosis, endodermal sinus tumors (ESTs), and myeloid sarcoma...
April 30, 2018: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29682023/extraosseous-primary-intracranial-ewing-sarcoma-peripheral-primitive-neuroectodermal-tumor-series-of-seven-cases-and-review-of-literature
#2
Amit Kumar Singh, Arun Kumar Srivastava, Lily Pal, Jayesh Sardhara, Rajan Yadav, Shalini Singh, Kamlesh Singh Bhaisora, Kuntal Kanti Das, Anant Mehrotra, Rabi Narayan Sahu, Awadhesh Kumar Jaiswal, Sanjay Behari
Background: The Ewing sarcoma peripheral PNET (ES-pPNET) is very rare small round cell tumour that involves the CNS as either a primary dural neoplasm or by direct extension from contiguous bone or soft tissue. Materials and Methods: Biopsy proven cases of intracranial ES/pPNET with orbital involvement operated during Jan 2010-Jan 2014 were retrospectively included and their clinical data, operative and histological findings were reviewed from institutional oncology register...
April 2018: Asian Journal of Neurosurgery
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
#3
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
#4
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
#5
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/29670360/integrated-bioinformatic-analysis-unveils-significant-genes-and-pathways-in-the-pathogenesis-of-supratentorial-primitive-neuroectodermal-tumor
#6
Guang-Yu Wang, Ling Li, Bo Liu, Xiao Han, Chun-Hua Wang, Ji-Wen Wang
Purpose: This study aimed to explore significant genes and pathways involved in the pathogenesis of supratentorial primitive neuroectodermal tumor (sPNET). Materials and methods: Gene expression profile of GSE14295 was downloaded from publicly available Gene Expression Omnibus (GEO) database. Differentially expressed genes (DEGs) were screened out in primary sPNET samples compared with normal fetal and adult brain reference samples (sPNET vs fetal brain and sPNET vs adult brain)...
2018: OncoTargets and Therapy
https://www.readbyqxmd.com/read/29643685/unilateral-brown-fat-suppression-on-fdg-pet-ct-detecting-sympathetic-denervation
#7
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/29624145/surgical-decision-making-in-the-management-of-childhood-tumors-of-the-cns-disseminated-at-presentation
#8
Matthew A Kirkman, Richard Hayward, Kim Phipps, Kristian Aquilina
OBJECTIVE It is relatively unusual for pediatric CNS tumors to be disseminated at presentation, and the literature on the clinical features, management, and outcomes of this specific group is scarce. Surgical management in this population is often challenging, particularly in the presence of hydrocephalus. The authors present their recent experience of treating pediatric CNS tumors that were disseminated at presentation, and they compare these lesions with focal tumors. METHODS The authors performed a retrospective review of prospectively collected data on children presenting to a tertiary center between 2003 and 2016 inclusive...
April 6, 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29617945/intracranial-neoplasms-in-the-first-year-of-life-results-of-a-third-cohort-of-patients-from-a-single-institution
#9
Sebastian M Toescu, Gregory James, Kim Phipps, Owase Jeelani, Dominic Thompson, Richard Hayward, Kristian Aquilina
BACKGROUND: Brain tumors in the first year of life are rare and their management remains challenging. OBJECTIVE: To report on the contemporary management of brain tumors in infants with reference to previous series from our institution. METHODS: Retrospective cohort study design. Electronic/paper case note review of all brain tumors diagnosed at our institution in children aged <1 yr since the publication of our previous series. RESULTS: Ninety-eight patients were seen...
April 3, 2018: Neurosurgery
https://www.readbyqxmd.com/read/29616903/sinonasal-ewing-sarcoma-a-case-report-and-literature-review
#10
Jonathan K Lin, Jonathan Liang
INTRODUCTION: Ewing sarcoma (ES) of the sinonasal tract and associated primitive neuroectodermal tumors are rare. To our knowledge, only 10 case reports of sinonasal ES of the nose or paranasal sinuses have been reported. Furthermore, there has been only 1 case of sinonasal ES arising from the middle turbinate. Recommended management of sinonasal ES stems from the management of its osseous counterpart, ES, but treatment with surgery, radiotherapy, and chemotherapy is varied. Five-year survival rates vary from 21% to 70%, with the lower rates representing patients presenting with metastatic disease...
March 7, 2018: Permanente Journal
https://www.readbyqxmd.com/read/29616127/therapeutic-approach-in-glioblastoma-multiforme-with-primitive-neuroectodermal-tumor-components-case-report-and-review-of-the-literature
#11
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/29611053/extra-osseous-ewing-sarcoma-of-the-pancreas-case-report-with-radiologic-pathologic-and-molecular-correlation-and-brief-review-of-the-literature
#12
Miglena K Komforti, Evgeniya Sokolovskaya, Catherine A D'Agostino, Ryma Benayed, Rebecca M Thomas
In 2002, due to extensive histomorphologic, immunohistochemical, and cytogenetic similarities, the World Health Organization unified undifferentiated small round blue cell neoplasms of soft tissue and bone (previously segregated as Ewing sarcoma or Primitive Neuroectodermal tumor) into one category: Ewing family of tumors (EFT). Osseous EFT are more common, and while extra-osseous EFT can occur anywhere in the body, those of the pancreas are rare and likely to be seen in the second decade of life in the head of the pancreas...
April 2, 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29572122/hpv-infection-and-p16-ink4a-and-tp53-expression-in-rare-cancers-of-the-uterine-cervix
#13
Nabiha Missaoui, Sarra Mestiri, Ahlem Bdioui, Thouraya Zahmoul, Hajer Hamchi, Moncef Mokni, Sihem Hmissa
Cervix cancer remains among most commonly diagnosed cancer in developing countries. Except squamous cell carcinoma and adenocarcinoma, the etiopathology and oncogenic mechanisms of rare cancers remain largely unknown. The study was performed to investigate the value of HPV infection and the expression of p16INK4A and TP53 in rare primitive cancers of the cervix. We conducted a retrospective study of rare primitive cancers of the cervix. Main clinicopathological features were reported. HPV infection was detected by in situ hybridization...
March 6, 2018: Pathology, Research and Practice
https://www.readbyqxmd.com/read/29568390/characterization-of-brain-tumor-initiating-cells-isolated-from-an-animal-model-of-cns-primitive-neuroectodermal-tumors
#14
Sergey Malchenko, Simone T Sredni, Jerusha Boyineni, Yingtao Bi, Naira V Margaryan, Maheedhara R Guda, Yulia Kostenko, Tadanori Tomita, Ramana V Davuluri, Kiran Velpula, Mary J C Hendrix, Marcelo B Soares
CNS Primitive Neuroectodermal tumors (CNS-PNETs) are members of the embryonal family of malignant childhood brain tumors, which remain refractory to current therapeutic treatments. Current paradigm of brain tumorigenesis implicates brain tumor-initiating cells (BTIC) in the onset of tumorigenesis and tumor maintenance. However, despite their significance, there is currently no comprehensive characterization of CNS-PNETs BTICs. Recently, we described an animal model of CNS-PNET generated by orthotopic transplantation of human Radial Glial (RG) cells - the progenitor cells for adult neural stem cells (NSC) - into NOD-SCID mice brain and proposed that BTICs may play a role in the maintenance of these tumors...
March 2, 2018: Oncotarget
https://www.readbyqxmd.com/read/29544549/ewing-sarcoma-of-the-adrenal-gland-a-case-report-and-review-of-the-literature
#15
Hanane Eddaoualline, Khadija Mazouz, Bouchra Rafiq, Ghizlane El Mghari Tabib, Nawal El Ansari, Rhizlane Belbaraka, Abdelhamid El Omrani, Mouna Khouchani
BACKGROUND: Ewing sarcoma/primitive neuroectodermal tumor is a family of highly malignant proliferation of neuroectodermal origin, most often skeletal, adrenal localization is extremely rare. Only few cases have been reported in the literature. Classical management includes radical surgery with adjuvant chemotherapy or radiotherapy or both. This case report is the only one where recurrence was surgically removed, and it confirms the importance of adjuvant treatment, and the efficacy of neoadjuvant chemotherapy...
March 16, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29537138/-pre-emptive-strike-the-case-for-early-treatment-of-hepatic-sinusoidal-obstruction-syndrome-with-defibrotide
#16
Revathi Rajagopal, Marianne B Phillips, Nicholas G Gottardo
The initial signs of hepatic sinusoidal obstruction syndrome (HSOS) can be challenging to recognize in children, especially outside the hematopoietic stem cell transplantation setting. To assist clinicians to promptly identify HSOS, the European Society for Blood and Marrow Transplantation has proposed pediatric HSOS diagnostic criteria which emphasize unexplained consumptive and transfusion-refractory thrombocytopenia. To highlight the importance of these "bellwether" early signs of HSOS and the efficacy of pre-emptive treatment with defibrotide, we describe the case of a child with a right 11th rib primitive neuroectodermal tumor who developed HSOS following focal radiotherapy and actinomycin-D treatment...
March 14, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29528832/embryonal-tumors-of-the-central-nervous-system-from-the-radiologic-pathology-archives
#17
Robert Y Shih, Kelly K Koeller
Embryonal tumors of the central nervous system (CNS) are highly malignant undifferentiated or poorly differentiated tumors of neuroepithelial origin and have been defined as a category in the World Health Organization (WHO) classification since the first edition of the "Blue Book" in 1979. This category has evolved over time to reflect our ever-improving understanding of tumor biology and behavior. With the most recent update in 2016, many previous histologic diagnoses incorporate molecular parameters for the first time (genetically defined entities)...
March 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29514411/embryonal-tumor-with-multilayered-rosettes-diagnosis-on-intra-operative-squash-smear
#18
Kirti Gupta, Vikram Singh, Ashish Aggarwal, Pravin Salunke
We describe a rare example of infratentorial primitive neuroectodermal tumor categorized as embryonal tumor with multilayered rosettes diagnosed on intra-operative squash smear and frozen section and discuss its key diagnostic features, pitfalls and differentials. Correct interpretation at the time of frozen section is crucial as it helps in deciding the further course of surgery. To the best of our knowledge, this is the first such report in the literature.
March 7, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29492943/spinal-peripheral-primitive-neuroectodermal-tumors-a-radiological-study-of-ten-cases
#19
Xiaoping Yi, Moling Zhou, Shenghui Liao, Guanghui Gong, Hongling Yin, Yongheng Chen, Weihua Liao
AIM: To summarize the imaging features of spinal peripheral primitive neuroectodermal tumor (spPNET) patients. MATERIAL AND METHODS: The computed tomography (CT) and magnetic resonance imaging (MRI) features of ten spPNET patients were retrospectively analyzed, and the clinicopathological data were reviewed. RESULTS: Four men and six women with a mean age of 24.7 years (range, 3-44 years) were included. A total of ten spPNET lesions were found in the ten patients, including six extradural and four intradural extramedullary lesions...
January 9, 2018: Turkish Neurosurgery
https://www.readbyqxmd.com/read/29485447/68ga-dotanoc-pet-ct-in-medulloblastoma
#20
Sreedharan Thankarajan Arunraj, Girish Kumar Parida, Nishikant Avinash Damle, Saurabh Arora, Sreenivasa Reddy, Dhritiman Chakraborty, Meghna Prabhu, Madhavi Tripathi, Chandrasekhar Bal
Medulloblastoma, also known as cerebellar primitive neuroectodermal tumor, is the most common brain tumor in children and arises in the posterior cranial fossa. We present the case of a patient with desmoplastic type of medulloblastoma, which showed recurrence more than once. When Ga-DOTANOC PET-CT was done, the lesions showed somatostatin receptor expression, opening another potential therapeutic option for this patient.
May 2018: Clinical Nuclear Medicine
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