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neuroectodermic tumor treatment

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https://www.readbyqxmd.com/read/28994342/malignant-ectomesenchymoma-series-analysis-of-a-histologically-and-genetically-heterogeneous-tumor
#1
Brannan B Griffin, Pauline M Chou, David George, Lawrence J Jennings, Nicoleta C Arva
AIMS: Malignant ectomesenchymoma is a rare pediatric neoplasm with dual mesenchymal and neuroectodermal elements. Mesenchymal component is usually rhabdomyosarcoma, particularly embryonal subtype, whereas neuroectodermal derivatives are frequently a neuroblastic tumor. Ectomesenchymoma manifests in various sites given the wide migration of neural crest cells during development, though the pelvis/perineum is most often involved. Moreover, no unique unifying molecular abnormality has been determined...
October 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28968318/melanotic-neuroectodermal-tumor-of-infancy-in-the-maxilla
#2
Haluk Yener Ünsal, Mustafa Yalçin
Melanotic neuroectodermal tumor of infancy is uncommon and has rapid expansile growth. Melanotic neuroectodermal tumor of infancy is a very rare and benign but locally aggressive neoplasm that originates from neural crest and is composed of relatively primitive pigment-producing cells. The lesion commonly affects the maxilla of infants during the first year of life. Common treatment methods include surgical excision and resection of the tumor. The aim of this article is to present the diagnosis and treatment of a patient with melanotic neuroectodermal tumor that occurred in the anterior maxilla of a 6-month-old male baby...
September 29, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28835865/primitive-neuroectodermal-tumor-and-wegener-s-granulomatosis-of-the-kidney-a-curious-combination-of-two-rare-entities
#3
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
#4
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/28718313/massive-embryonal-rhabdomyosarcoma-of-the-hand-in-an-infant-with-metastasis-at-birth-management-dilemma
#5
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
#6
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/28607817/a-rare-case-report-of-mixed-olfactory-neuroblastoma-carcinoma-with-review-of-literature
#7
Charandeep S Gandhoke, Aditi Dewan, Divya Gupta, Simran K Syal, Anita Jagetia, Ravindra K Saran, Ravi Meher, Arvind K Srivastava, Daljit Singh
BACKGROUND: Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Mixed olfactory neuroblastoma which contains areas of divergent differentiation is even rare. Till date, only 4 cases of mixed olfactory neuroblastomas have been reported. CASE DESCRIPTION: We report the case of a 46-year-old male who presented with the chief complaints of nasal bleeding and nasal obstruction since 4 months. Radiological imaging was suggestive of a large heterogeneous mass in the left superior nasal cavity with extensions into bilateral maxillary, ethmoidal, and sphenoidal sinuses, as well as into the anterior cranial fossa...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28601416/statistics-of-soft-tissue-sarcoma-in-japan-report-from-the-bone-and-soft-tissue-tumor-registry-in-japan
#8
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/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/28509141/primary-ewing-sarcoma-of-the-kidney-a-case-report-and-treatment-review
#10
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/28472972/primary-ewing-s-sarcoma-primitive-neuroectodermal-tumor-of-the-ileum-case-report-of-a-16-year-old-chinese-female-and-literature-review
#11
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/28468165/orbital-reconstruction-in-neuroectodermal-tumor-of-the-orbit-multimodal-treatment-approach
#12
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
#13
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/28444219/dna-methylation-of-a-novel-pak4-locus-influences-ototoxicity-susceptibility-following-cisplatin-and-radiation-therapy-for-pediatric-embryonal-tumors
#14
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, our objective was to conduct an epigenome-wide association study to identify differentially methylated cytosine-phosphate-guanine (CpG) sites associated with ototoxicity susceptibility among cisplatin-treated pediatric patients with embryonal tumors. Methods: Samples were collected for a discovery cohort (n = 62) and a replication cohort (n = 18) of medulloblastoma and primitive neuroectodermal tumor patients...
October 1, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28438654/dysembryoplastic-neuroectodermal-tumor-an-analysis-from-the-surveillance-epidemiology-and-end-results-program-2004-2013
#15
Ha Son Nguyen, Ninh Doan, Michael Gelsomino, Saman Shabani
BACKGROUND: Dysembryoplastic neuroectodermal tumor (DNT) is a rare neoplasm. Though the pathology is commonly considered benign, there have been various reports documenting rapid growth, recurrence/progression, sudden death, and malignant transformation. Most studies have addressed outcomes regarding seizure control, but limited data exist regarding incidence and survival. Consequently, we explore the Surveillance, Epidemiology, and End Results (SEER) database to explore the epidemiology of DNT...
July 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28405084/primitive-neuroectodermal-tumors-of-the-kidney
#16
Geetha Narayanan, Varun Rajan, T R Preethi
Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) rarely occurs as a primary renal tumor. The disease affects young adults and children and has an aggressive course. The clinical presentation and imaging of these tumors are nonspecific, and they often present at an advanced stage. We present the clinical features, imaging, diagnosis, and treatment of 7 cases of renal PNET (4 men, 3 women; median age, 32 years). Common presenting symptoms were flank or abdominal pain and a mass in the abdomen. On imaging, a large heterogenous infiltrating renal mass with areas of calcification, hemorrhage, and necrosis and tumor thrombus can give a clue to the diagnosis of renal PNET...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28403132/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...
April 11, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28383090/lumbosacral-epidural-primitive-neuroectodermal-tumor-pnet-case-report-and-literature-review
#18
Keyvan Eghbal, Amir Reza Dehghanian, Fariborz Ghaffarpasand
BACKGROUND: The primitive neuroectodermal tumors (PNET) of the spine are rare and usually are intramedullary and being reported in children. We herein report an epidural PNET of lumbosacral area presenting with cauda equine syndrome in an adult. CASE REPORT: A 38-year-old woman presented to our emergency room with acute onset lower extremity weakness and urinary incontinency. Emergency MRI revealed an isoitense on T1-weighted and heterogeneously hyperintense in T2-weighted images in epidural lumbosacral area...
March 9, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/28378288/development-of-cystic-malacia-after-high-dose-cranial-irradiation-of-pediatric-cns-tumors-in-long-term-follow-up
#19
Fumiyuki Yamasaki, Takeshi Takayasu, Ryo Nosaka, Ikuno Nishibuchi, Hiroshi Kawaguchi, Manish Kolakshyapati, Shumpei Onishi, Taiichi Saito, Kazuhiko Sugiyama, Masao Kobayashi, Kaoru Kurisu
PURPOSE: The purpose of this study is to investigate the incidence of cystic malacia in long-term survivors of pediatric brain tumors treated with high-dose cranial irradiation. MATERIALS AND METHODS: Between 1997 and 2015, we treated 41 pediatric patients (26 males, 15 females; age ranging from 3.3 to 15.7 years, median 9-year-old) of pediatric brain tumors [17 medulloblastomas, 7 primitive neuroectodermal tumors (PNET), 3 pineoblastomas, 6 non-germinomatous germ cell tumors (NGGCT), 8 gliomas (including 4 ependymomas, 1 anaplastic astrocytoma, 1 oligodendroglioma, 1 pilocytic astrocytoma, 1 astroblastoma)] with high-dose craniospinal irradiation...
June 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28375941/two-tumors-in-1-what-should-be-the-therapeutic-target-pediatric-germ-cell-tumor-with-somatic-malignant-transformation
#20
Cecile Faure Conter, Brice Fresneau, Estelle Thebaud, Amandine Bertrand, Frederique Dijoud, Angelique Rome, Cecile Dumesnil, Marie Pierre Castex, Anguella Ghanem, Daniel Orbach
BACKGROUND: Germ cell tumors with somatic malignant transformation (GCT with SMT) are rare in children and poorly described. Data are missing to determine if therapies should target the GCT, the SMT compound, or both simultaneously. PATIENTS AND METHODS: A retrospective national study was conducted in the Société Française des cancers de l'Enfant (SFCE) Centers. Medical records from patients aged 0 to 18 years diagnosed with GCT with SMT between 2000 and 2015 were analyzed...
July 2017: Journal of Pediatric Hematology/oncology
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