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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/28810315/-adult-peripheral-primary-neuroectodermal-tumor-a-case-report-and-literature-review
#1
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
#2
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...
August 12, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28801375/primary-pure-endometrial-primitive-neuroectodermal-tumors-with-ewsr1-gene-rearrangement-report-of-2-cases
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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...
January 1, 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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/28601416/statistics-of-soft-tissue-sarcoma-in-japan-report-from-the-bone-and-soft-tissue-tumor-registry-in-japan
#15
Koichi Ogura, Takahiro Higashi, Akira Kawai
BACKGROUND: No previous reports to date have characterized the national profiles of soft-tissue sarcomas (STSs). In the present study, we reviewed current practice for STSs in Japan using data from a nationwide organ-specific cancer registry for bone and soft-tissue tumors in Japan, the Bone and Soft Tissue Tumor (BSTT) Registry. METHODS: In the registry, we identified 8228 patients with STSs during the period 2006-2012, and extracted data on patient demographics, treatment, and outcome at the last follow-up for each patient...
July 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28592714/the-2016-who-classification-of-tumours-of-the-central-nervous-system-the-major-points-of-revision
#16
Takashi Komori
The updated 2016 edition of the World Health Organization (WHO) Classification of Tumours of the Central Nervous System (CNS) uses molecular parameters and the histology to define the main tumor categories for the first time. This represents a shift from the traditional principle of using neuropathological diagnoses, which are primarily based on the microscopic features, to using molecularly-oriented diagnoses. Major restructuring was made with regard to diffuse gliomas, medulloblastomas and other embryonal tumors...
June 8, 2017: Neurologia Medico-chirurgica
https://www.readbyqxmd.com/read/28589056/vaginal-ewing-sarcoma-an-uncommon-clinical-entity-in-pediatric-patients
#17
Nathan M Cross, A Luana Stanescu, Erin R Rudzinski, Douglas S Hawkins, Marguerite T Parisi
Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28554176/fine-needle-aspiration-of-metastatic-central-type-primitive-neuroectodermal-tumors-in-patients-with-a-germ-cell-tumor
#18
Shaoxiong Chen, Muhammad Idrees, Jingmei Lin, Howard H Wu
OBJECTIVE: Central type primitive neuroectodermal tumors (PNET) are some of the most frequent somatic type tumors derived from germ cell tumors and can metastasize. We studied the cytomorphological features of metastatic central type PNET by fine-needle aspiration (FNA). MATERIALS AND METHODS: A computerized search of our laboratory information system was performed for the 9-year period from 2005 through 2014 to identify all cytology cases in which a diagnosis of metastatic central type PNET had been rendered...
May 30, 2017: Acta Cytologica
https://www.readbyqxmd.com/read/28534008/a-comparison-of-pediatric-vs-adult-patients-with-the-ewing-sarcoma-family-of-tumors
#19
Vivek Verma, Kyle A Denniston, Christopher J Lin, Chi Lin
PURPOSE: This study sought to identify differences in clinical characteristics, outcomes, and treatments between adult and pediatric patients with the Ewing sarcoma family of tumors (ESFT). METHODS: By using the Surveillance, Epidemiology, and End Results database from 1983 to 2013, 1,870 patients were analyzed (n = 976 pediatric, n = 894 adult). Between the two groups, demographic, tumor, and treatment characteristics were collated and compared. The chi-square test determined differences in proportions of the variables between groups...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28515922/primary-primitive-neuroectodermal-tumor-of-the-cervix-a-report-of-two-cases-and-review-of-the-literature
#20
Xiaofeng Wang, Yan Gao, Yanning Xu, Yixin Liu, Pengpeng Qu
Peripheral primitive neuroectodermal tumor (PNET) of the cervix uteri is extremely rare. Two cases of PNET of the cervix are presented herein. Two female patients, aged 48 and 43 years, presented with irregular uterine bleeding over the course of 1 year, and increased urinary frequency for 1 month, respectively. On gynecological examination, a mass in the cervix was palpated and a biopsy performed. The findings of the initial biopsy revealed small-cell carcinoma in both patients. Following neoadjuvant chemotherapy and radiotherapy, radical hysterectomy was performed in both patients...
May 2017: Molecular and Clinical Oncology
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