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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/28631646/primitive-neuroectodermal-tumor-presenting-as-a-presacral-mass-a-rare-case-report-with-review-of-literature
#1
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
#2
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
#3
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
#4
Nisha Ramani, Phyu P Aung, Wen-Jen Hwu, Priyadharsini Nagarajan, Michael T Tetzlaff, Jonathan L Curry, Doina Ivan, Victor G Prieto, Carlos A Torres-Cabala
FLI-1 (Friend leukemia integration site 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 two 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
#5
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...
June 7, 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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28510278/tfg-met-fusion-in-an-infantile-spindle-cell-sarcoma-with-neural-features
#11
Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu
An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression...
May 16, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28509141/primary-ewing-sarcoma-of-the-kidney-a-case-report-and-treatment-review
#12
Muhammad Sadiq, Iftikhar Ahmad, Jamila Shuja, Khushnaseeb Ahmad
Ewing sarcomas/primitive neuroectodermal tumors (ES/PNET) of the kidney are rarely found high-grade malignant tumors, offering poor prognosis. Although established treatment guidelines for ES of kidney are scarce, a multi-modality treatment approached is typically implemented. Herein, we report a 14-year-old female patient with ES of right kidney. Post-nephrectomy disease recurrence was treated with chemotherapy (i.e., vincristine, doxorubicin and cyclophosphamide); marked reduction in tumor size (i.e., from 18...
April 21, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508928/embryonal-tumors-in-canadian-children-less-than-36-months-of-age-results-from-the-canadian-pediatric-brain-tumor-consortium-cpbtc
#13
E Story, D L Johnston, U Bartels, A S Carret, B Crooks, D D Eisenstat, C Fryer, L Lafay-Cousin, V Larouche, B Wilson, S Zelcer, M Silva, J Brossard, E Bouffet, D L Keene
Embryonal tumors are a heterogeneous group of central nervous system (CNS) tumors whose subgroups have varying incidence and outcome. Despite these differences, they are often grouped as a single entity for study purposes. To date, there are no Canadian multi-institutional studies examining the incidence and outcome of all embryonal subtypes. The current study is an observational study reviewing embryonal tumors in all patients less than 36 months of age diagnosed with a CNS tumor in Canada from 1990 to 2005...
May 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28472972/primary-ewing-s-sarcoma-primitive-neuroectodermal-tumor-of-the-ileum-case-report-of-a-16-year-old-chinese-female-and-literature-review
#14
Teng Li, Fang Zhang, Yarui Cao, Shoubin Ning, Yongmin Bi, Weicheng Xue, Li Ren
BACKGROUND: Ewing's sarcoma (ES) and primitive neuroectodermal tumors (PNET) are closely related tumors. Although soft tissue ES/PNET are common in clinical practice, they are rare in the small intestine. Because of the absence of characteristic clinical symptoms, they are easily misdiagnosed as other benign or malignant diseases. CASE PRESENTATION: Here, we present the case of a 16-year-old female who complained of anemia and interval hematochezia. Her serum test results showed only a slight elevation of CA-125 and a low level of hemoglobin...
May 4, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28469346/primitive-neuroectodermal-tumor-of-lung-in-adult-with-hemorrhagic-brain-metastasis-an-extremely-rare-case-scenario
#15
Abhishek Purkayastha, Neelam Sharma, Amul Kapur, Kavita Sahai
Primitive neuroectodermal tumors (PNETs) are highly malignant neoplasms of embryonal origin manifesting in children and adolescents, rarely seen in adults. Carcinoma lung with hemorrhagic metastasis to the brain is very common, but primary lung PNET with hemorrhagic brain metastasis is extremely uncommon. We hereby report a 29-year-old female diagnosed as PNET lung was treated with vincristine, adriamycin, and cyclophosphamide alternating with ifosfamide plus etoposide followed by radiotherapy (RT). After 9 months, she developed hemorrhagic brain metastasis from PNET lung confirmed from tissue immunohistology postcraniotomy...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28469330/profile-of-primary-pediatric-brain-and-spinal-cord-tumors-from-north-india
#16
Nadia Shirazi, Meenu Gupta, Nowneet Kumar Bhat, Braham Prakash Kalra, Ranjit Kumar, Manju Saini
BACKGROUND AND OBJECTIVE: The study was carried out to find the profile of pediatric brain and spinal cord tumors during 2006-2015 in a tertiary referral center of North India. MATERIALS AND METHODS: It was a retrospective medical record-based observational study. All children <18 years of age with confirmed histopathological diagnosis of cancer were included in the study. RESULTS: Central nervous system (CNS) tumors constituted 5.6% of all pediatric solid malignancies in our hospital...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28468165/orbital-reconstruction-in-neuroectodermal-tumor-of-the-orbit-multimodal-treatment-approach
#17
Jorge Ernesto Cantini A, Diana M Díaz López, Esteban Francisco Hernandez Florez
INTRODUCTION: Primitive neuroectodermal tumors of peripheral origin are very rare, and orbital neuroectodermal tumors are even more uncommon. Only 25 patients with primary orbital involvement in the pediatric age group have been reported. METHODS: In this article, the authors describe their experience in the multimodality treatment approach to treat neuroectodermal tumor of the orbit. The authors also present a male patient 3-year old presenting with a neuroectodermal tumor of the right orbit causing rapidly progressive proptosis...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28462848/outcome-of-resectable-pediatric-ewing-sarcoma-of-the-ribs
#18
Gehad Ahmed, Manal Zamzam, Mohamed S Zaghloul, Ahmed Kamel, Ranin Soliman, Iman Zaky, Asmaa Salama, Nehal Kamal, Maged ElShafiey
PURPOSE: Was to evaluate the outcome of multimodality treatment in resectable primary Ewing sarcoma/primitive neuroectodermal tumor ES/PNET of the ribs and role of thoracoscopy in facilitating resection of these tumors. PATIENTS AND METHODS: This was a retrospective study including 22 patients with primary ES/PNET of the ribs surgically treated at Children's Cancer Hospital Egypt (CCHE) between January 2008 until the end of December 2014. RESULTS: Median age was 8...
April 24, 2017: Journal of the Egyptian National Cancer Institute
https://www.readbyqxmd.com/read/28459463/mycn-induces-neuroblastoma-in-primary-neural-crest-cells
#19
R R Olsen, J H Otero, J García-López, K Wallace, D Finkelstein, J E Rehg, Z Yin, Y-D Wang, K W Freeman
Neuroblastoma (NBL) is an embryonal cancer of the sympathetic nervous system (SNS), which causes 15% of pediatric cancer deaths. High-risk NBL is characterized by N-Myc amplification and segmental chromosomal gains and losses. Owing to limited disease models, the etiology of NBL is largely unknown, including both the cell of origin and the majority of oncogenic drivers. We have established a novel system for studying NBL based on the transformation of neural crest cells (NCCs), the progenitor cells of the SNS, isolated from mouse embryonic day 9...
May 1, 2017: Oncogene
https://www.readbyqxmd.com/read/28444219/dna-methylation-of-a-novel-pak4-locus-influences-ototoxicity-susceptibility-following-cisplatin-and-radiation-therapy-for-pediatric-embryonal-tumors
#20
Austin L Brown, Kayla L Foster, Philip J Lupo, Erin C Peckham-Gregory, Jeffrey C Murray, M Fatih Okcu, Ching C Lau, Surya P Rednam, Murali Chintagumpala, Michael E Scheurer
Background: Ototoxicity is a common adverse side effect of platinum chemotherapy and cranial radiation therapy; however, individual susceptibility is highly variable. Therefore, the objective of this study was to conduct an epigenome-wide association study to identify differentially methylated CpG sites associated with ototoxicity susceptibility among cisplatin-treated pediatric patients with embryonal tumors. Methods: Samples were collected for a discovery (n=62) and a replication cohort (n=18) of medulloblastoma and primitive neuroectodermal tumor patients...
April 24, 2017: Neuro-oncology
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