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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/28903419/development-of-zebrafish-medulloblastoma-like-pnet-model-by-talen-mediated-somatic-gene-inactivation
#1
Jaegal Shim, Jung-Hwa Choi, Moon-Hak Park, Hyena Kim, Jong Hwan Kim, Seon-Young Kim, Dongwan Hong, Sunshin Kim, Ji Eun Lee, Cheol-Hee Kim, Jeong-Soo Lee, Young-Ki Bae
Genetically engineered animal tumor models have traditionally been generated by the gain of single or multiple oncogenes or the loss of tumor suppressor genes; however, the development of live animal models has been difficult given that cancer phenotypes are generally induced by somatic mutation rather than by germline genetic inactivation. In this study, we developed somatically mutated tumor models using TALEN-mediated somatic gene inactivation of cdkn2a/b or rb1 tumor suppressor genes in zebrafish. One-cell stage injection of cdkn2a/b-TALEN mRNA resulted in malignant peripheral nerve sheath tumors with high frequency (about 39%) and early onset (about 35 weeks of age) in F0 tp53(e7/e7) mutant zebrafish...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28898190/renal-tumors-of-childhood-radiologic-pathologic-correlation-part-2-the-2nd-decade-from-the-radiologic-pathology-archives
#2
Ellen M Chung, Grant E Lattin, Kimberly E Fagen, Andrew M Kim, Michael A Pavio, Adam J Fehringer, Richard M Conran
Malignant renal tumors account for 7% of childhood cancers, and Wilms tumors are by far the most common-but not in older children and adolescents. Among individuals in the latter half of their 2nd decade of life, renal cell carcinoma (RCC) is more common than Wilms tumor. The histopathologic spectrum of RCCs in children differs from that in adults. The most common subtype of RCC in children and adolescents is Xp11.2 translocation RCC, which is distinguished by hyperattenuation at nonenhanced computed tomography, a defined capsule, and associated retroperitoneal lymphadenopathy...
September 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28880421/diffuse-midline-gliomas-with-histone-h3-k27m-mutation-a-rare-case-with-pnet-like-appearance-and-neuropil-like-islands
#3
Yue Gao, Yang-Yang Feng, Juan-Han Yu, Qing-Chang Li, Xue-Shan Qiu, En-Hua Wang
Diffuse midline glioma with histone H3-K27M mutation is a new tumor entity defined by the 2016 WHO Classification of Tumors of the Central Nervous System. A 51-year-old Chinese woman presented with neck pain for a month. Subsequent MRI revealed an intramedullary neoplasm extending from C5 to C7. Histologically, the cellular area of the tumor was composed of primitive, poorly differentiated, small cells with scant cytoplasm, nuclear molding, and brisk mitotic activity, exhibiting PNET-like appearance, while in the hypocellular area, oligodendroglioma-like cells were observed...
September 6, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28877739/estrogen-and-soy-isoflavonoids-decrease-sensitivity-of-medulloblastoma-and-central-nervous-system-primitive-neuroectodermal-tumor-cells-to-chemotherapeutic-cytotoxicity
#4
Scott M Belcher, Caleb C Burton, Clifford J Cookman, Michelle Kirby, Gabriel L Miranda, Fatima O Saeed, Kathleen E Wray
BACKGROUND: Our previous studies demonstrated that growth and migration of medulloblastoma (MB), the most common malignant brain tumor in children, are stimulated by 17β-estradiol. The growth stimulating effects of estrogens are mediated through ERβ and insulin-like growth factor 1 signaling to inhibit caspase 3 activity and reduce tumor cell apoptosis. The objective of this study was to determine whether estrogens decreased sensitivity of MB cells to cytotoxic actions of chemotherapeutic drugs...
September 6, 2017: BMC Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28859038/refractory-lch-with-secondary-intracranial-pnet-a-case-report-and-review-of-literature
#5
Varsha Chayapathi, Amita Mahajan, Manas Kalra
The occurrence of second malignant neoplasms in patients with Langerhans cell histiocytosis is infrequent but has been reported. Here we report the case of a child with refractory Langerhans cell histiocytosis who was treated with cladribine and later developed a secondary intracranial primitive neuroectodermal tumor. The possible association of cladribine with second neoplasm is further discussed.
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28835865/primitive-neuroectodermal-tumor-and-wegener-s-granulomatosis-of-the-kidney-a-curious-combination-of-two-rare-entities
#6
Rugvedita Parakh, Satyajeet Parakh, Maria Tretiakova
Wegener's granulomatosis (WG) is characterized by necrotizing polyangiitis involving the respiratory tract and kidneys. It causes segmental necrotizing glomerulonephritis in the kidneys. In rare cases, a renal pseudotumor may be seen because of the granulomatous process. Association of WG with renal malignancy, however, is very uncommon. We report a case of a patient who presented several years after being treated for WG with malignant hypertension and an infiltrating mass in the right kidney. The histopathology of radical nephrectomy specimen showed presence of primitive neuroectodermal tumor (PNET)...
2017: Case Reports in Urology
https://www.readbyqxmd.com/read/28810315/-adult-peripheral-primary-neuroectodermal-tumor-a-case-report-and-literature-review
#7
M H Hu, F Long, S J Jiang
Objective: To analyze the clinical features, diagnosis and differential diagnosis of peripheral primary neuroectodermal tumor(pPNET). Methods: The clinical data and diagnosis of a patient with rapid progressive pPNET in Shandong Provincial Hospital affiliated to Shandong University in January 2016 was reported and the related literatures were reviewed.The literature reviews were carried out respectively in CNKI, Wanfang and PubMed by July 2016 with "primitive neurotodermal tumour" and "PNET" being the search term from March 1994 to July 2016, including 13 articles...
August 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28806254/rare-ileal-ewing-sarcoma-primitive-neuroectodermal-tumor-on-18f-fdg-pet-ct
#8
Pan Yu, Fei Xiaochun, Lv Jing, Zhang Yifan
A 24-year-old woman who had melena, dizziness, and fatigue and looked pale for 2 months was confirmed to have a microcytic hypochromic anemia in the local hospital. It was further revealed that the patient had splenomegaly and a small intestinal lump, and she was then referred to our hospital. The enhanced CT showed an ileal lesion with enhancement, which showed an increased F-FDG activity in further PET/CT scanning. Lymphoma was therefore suspected, and an uncomplicated surgical resection of the ileal lesion was then performed...
October 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28801375/primary-pure-endometrial-primitive-neuroectodermal-tumors-with-ewsr1-gene-rearrangement-report-of-2-cases
#9
Binara Assylbekova, Amal Kanbour-Shakir, Kavita Varma, Rohit Bhargava, Esther Elishaev
Primary Primitive Neuroectodermal Tumor (PNET) of the female genital tract is very rare. The majority has been described in ovaries, and the endometrium is an even rarer site. Herein we describe 2 cases of primary uterine corpus PNET cases with EWSR1 FISH confirmation. To the best of our knowledge, there are four cases reported in the English literature. The knowledge of these tumors' incidence in the endometrium is important to keep in mind, to differentiate it from poorly differentiated and undifferentiated adenocarcinomas, should such pattern of growth be encountered...
August 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28761532/skull-base-bony-lesions-management-nuances-a-retrospective-analysis-from-a-tertiary-care-centre
#10
Amit Kumar Singh, Arun Kumar Srivastava, Jayesh Sardhara, Kamlesh Singh Bhaisora, Kuntal Kanti Das, Anant Mehrotra, Rabi Narayan Sahu, Awadhesh Kumar Jaiswal, Sanjay Behari
BACKGROUND: Skull base lesions are not uncommon, but their management has been challenging for surgeons. There is large no of bony tumors at the skull base which has not been studied in detail as a group. These tumors are difficult not only because of their location but also due to their variability in the involvement of important local structure. Through this retrospective analysis from a Tertiary Care Centre, we are summarizing the details of skull base bony lesions and its management nuances...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28761507/primary-intracranial-dural-based-ewing-sarcoma-peripheral-primitive-neuroectodermal-tumor-mimicking-a-meningioma-a-rare-tumor-with-review-of-literature
#11
REVIEW
Vikul Kumar, Anshu Singh, Vivek Sharma, Mohan Kumar
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a malignant small, round cell tumor arising from bone and soft tissue in children and young adults. It can occur at osseous and extraosseous sites. Its usual locations are diaphysis of long bones followed by pelvis, ribs, vertebrae, and rarely skull. We reviewed the literature and PubMed advanced search on ES/pPNET occurring at extraosseous sites, mainly involving the central nervous system (CNS). We reported a case of a 22-year-old male presenting with seizure finally diagnosed as a case of ES/pPNET...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28753586/development-of-zebrafish-medulloblastoma-like-pnet-model-by-talen-mediated-somatic-gene-inactivation
#12
Jaegal Shim, Jung-Hwa Choi, Moon-Hak Park, Hyena Kim, Jong Hwan Kim, Seon-Young Kim, Dongwan Hong, Sunshin Kim, Ji Eun Lee, Cheol-Hee Kim, Jeong-Soo Lee, Young-Ki Bae
Genetically engineered animal tumor models have traditionally been generated by the gain of single or multiple oncogenes or the loss of tumor suppressor genes; however, the development of live animal models has been difficult given that cancer phenotypes are generally induced by somatic mutation rather than by germline genetic inactivation. In this study, we developed somatically mutated tumor models using TALEN-mediated somatic gene inactivation of cdkn2a/b or rb1 tumor suppressor genes in zebrafish. One-cell stage injection of cdkn2a/b-TALEN mRNA resulted in malignant peripheral nerve sheath tumors with high frequency (about 39%) and early onset (about 35 weeks of age) in F0 tp53e7/e7 mutant zebrafish...
July 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28728220/-diagnostic-utility-of-tyrosine-hydroxylase-in-peripheral-neuroblastic-tumors
#13
N Zhang, N Liu, L J He
Objective: To investigation the diagnostic utility of tyrosine hydroxylase (TH) immunohistochemically as a marker of peripheral neuroblastic tumors(pNT). Methods: The study included 1 024 cases, 643 primary and metastatic pNT cases, 381 non-pNT cases, including small round cell tumors such as primitive neuroectodermal tumor (PNET), rhabdomyosarcoma, lymphoma, nephroblastoma, as well as other more common tumors (medulloblastoma, hepatoblastoma, pleuropulmonary blastoma, renal clear cell sarcoma, Langerhans cell histiocytosis, lipoblatoma etc)...
July 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28718313/massive-embryonal-rhabdomyosarcoma-of-the-hand-in-an-infant-with-metastasis-at-birth-management-dilemma
#14
Jayakrishnan K Narayana Kurup, Vinay C Kamble, Ashwath M Acharya, Anil K Bhat
BACKGROUND: Rhabdomyosarcomas are malignant tumors arising from striated muscle but can be often confused with primitive neuroectodermal tumors and Ewing sarcoma. They are often classified based on age of presentation and histological features. Three major types of rhabdomyosarcomas are embryonal, alveolar, and pleomorphic with characteristic presentations. METHOD: Here, we present a case of embryonal rhabdomyosarcoma in the hand of a 5-month-old child with lymphatic metastasis, the age, site, and metastasis being unusual feature for this type of rhabdomyosarcoma...
September 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28670073/ewing-s-sarcoma-of-the-trachea-in-an-adolescent-girl
#15
Jaisankar Puthusseri, Geetha Narayanan, T R Preethi, G Jayapriya
Primitive neuroectodermal tumors (PNET) are aggressive neoplasms of neuroectodermal origin. Although they are known to arise in a host of locations, involvement of the trachea has rarely been reported. We describe an adolescent girl who presented with stridor and was diagnosed with PNET of the trachea. She is in remission following treatment with combination chemotherapy and local radiotherapy.
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28647700/molecular-proteomic-characterization-of-a-pediatric-medulloblastoma-xenograft
#16
George T Tsangaris, Konstantinos Dimas, Angeliki Malamou, Angeliki Katsafadou, Chrissa Papathanasiou, Dimitrios J Stravopodis, Constantinos E Vorgias, Maria Gazouli, Athanasios K Anagnostopoulos
BACKGROUND/AIM: Medulloblastoma (MBL), an archetypal primitive neuroectodermal tumor of the cerebellum, is the most common pediatric central nervous system malignancy representing approximately 20% of all childhood brain tumors. Herein, we report on a new xenotransplantable tumor cell line, derived from a 6-year-old female patient with cerebellar medulloblastoma, and the completele proteome molecular characterization of subsequent tumors from MBL xenotrasplanted mice. MATERIALS AND METHODS: Tumors were grown in nude mice as subcutaneous xenografts (MBLX) composed of small round cells with hyperchromatic nuclei and scant cytoplasm...
July 2017: Cancer Genomics & Proteomics
https://www.readbyqxmd.com/read/28631646/primitive-neuroectodermal-tumor-presenting-as-a-presacral-mass-a-rare-case-report-with-review-of-literature
#17
Pradnya S Bhadarge, Sonali S Datar, Pradeep S Umap, Alok C Shrivastava
Primitive neuroectodermal tumors (PNETs) are a group of highly malignant small round cell tumor (SRCT) of neuroectodermal origin. They exhibit a great diversity in their clinical manifestations and pathologic similarities with other SRCTs. PNET commonly occurs in the central nervous system, head and neck region, paravertebral region, pelvis, and lower extremities. PNET presenting as a presacral mass is very rare. We present a case of 65-year-old female patient presented with a mass in the abdomen. Exploratory laparotomy with excision of mass was carried out...
April 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28631645/primary-leptomeningeal-primitive-neuroectodermal-tumor-a-difficult-entity-to-diagnose
#18
Jyothy Sehgal, Murthy Murali Krishna Jagarlapudi, Murthy V R K Tenneti, Syed Ameer Basha, Sundaram Challa
A 25-year-old male presented with difficulty in walking, loss of vision, and seizures. A clinical possibility of craniospinal meningeal pathology was considered. On computed tomography (CT) scan and magnetic resonance imaging, there was an enhancement of leptomeninges with few ring-enhancing lesions in both frontal lobes and right frontoparietal region. He was evaluated for low backache and occipital headache 2½ years earlier and was found to have communicating hydrocephalus on CT scan. He underwent ventriculoperitoneal shunt and was followed up with CT scans...
April 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28616622/a-case-of-primary-mediastinal-ewing-s-sarcoma-primitive-neuroectodermal-tumor-presenting-with-chest-pain
#19
Ural Koc, Erkan Duman
No abstract text is available yet for this article.
June 2017: Turkish Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28605142/aberrant-expression-of-fli-1-in-melanoma
#20
Nisha Ramani, Phyu P Aung, Wen-Jen Hwu, Priyadharsini Nagarajan, Michael T Tetzlaff, Johnathan L Curry, Doina Ivan, Victor G Prieto, Carlos A Torres-Cabala
Friend leukemia integration site 1 (FLI-1) nuclear transcription factor has been proposed as a suitable tool in the differential diagnosis of small round cell sarcomas. It has also been described as nuclear marker of endothelial differentiation. Expression of FLI-1 has been demonstrated in Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) and vascular neoplasms. In the present study, we describe 2 cases of metastatic melanoma with small round blue cell morphology that showed strong nuclear expression of FLI-1...
June 12, 2017: Journal of Cutaneous Pathology
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