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primitive neuroectodermal tumor

Maryam Abolhasani, Sareh Salarinejad, Mohammad Kazem Moslemi
INTRODUCTION: Ewing sarcoma/Primitive neuroectodermal tumor of the kidney (ES/PNET) is a member of Ewing's sarcoma family, occurring in young adults and has aggressive clinical behavior and poor prognosis. However, its discrimination from the renal cell carcinoma (RCC) is very difficult preoperatively. We present three cases of this rare disease that were managed in two academic centers. PRESENTATION OF CASES: Herein we report three cases of ES/PNET of the kidney, 2 young men complaining of right flank pain and gross hematuria and one young woman complaining of left subcostal pain...
October 18, 2016: International Journal of Surgery Case Reports
Alice Indini, Elisabetta Schiavello, Veronica Biassoni, Luca Bergamaschi, Maria Chiara Magni, Nadia Puma, Stefano Chiaravalli, Federica Pallotti, Ettore Seregni, Barbara Diletto, Emilia Pecori, Lorenza Gandola, Geraldina Poggi, Maura Massimino
To assess the long-term safety of administering growth hormone (GH) in patients with GH deficiency due to treatment for childhood medulloblastoma and primitive neuroectodermal tumor (PNET). Data were retrospectively retrieved on children receiving GH supplementation, assessing their disease-free and overall survival outcomes and risk of secondary malignancies using Kaplan-Meier and Cox models. Overall 65 children were consecutively collected from May 1981 to April 2013. All patients had undergone craniospinal irradiation (total dose 18-39 Gy), and subsequently received GH for a median (interquartile range, IQR) of 81 (50...
October 21, 2016: Journal of Neuro-oncology
Avas Chandra Ray, Subhra Aditya, Pulak Kumar Jana, Apratim Chatterjee, Anup Sarkar, Jay Mehta, Jotideb Mukhopadhyay
A young male labourer developed pain at the site of blunt trauma over back of chest followed by fever, cough with expectoration, breathlessness and hemorrhagic pleural effusion in the side of injury. What could have been passed as a sequel of trauma turned out to be the consequences of an underlying rare and aggressive malignant tumor of the chest wall known as Askin tumor or Primitive Neuroectodermal Tumor (PNET). CT thorax with guided FNAC, debulking operation, histopathological examination followed by immunohistochemistry of the tumor tissue led to the final diagnosis...
March 2016: Journal of the Association of Physicians of India
Snehal J, Suresh Sr
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Young-Bem Se, Dong Gyu Kim, Sung Kwon Kim, Jin Wook Kim, Yong Hwy Kim, Chul-Kee Park, Hyun-Tai Chung, Sun-Ha Paek
BACKGROUND: Supratentorial primitive neuroectodermal tumors (PNETs) are highly malignant and rare tumors of the central nervous system. OBJECTIVES: The aim of this study was to determine the role of Gamma Knife surgery (GKS) as a salvage treatment option for patients with recurrent or residual supratentorial PNETs. METHODS: Between 1998 and 2014, 11 patients with supratentorial PNETs were retrospectively analyzed. This series consisted of 7 male and 4 female patients...
October 8, 2016: Stereotactic and Functional Neurosurgery
Ivan Zokalj, Jasminka Igrec, Antonio Plesnar
INTRODUCTION: Primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (EWS) belongs to a family of neoplasms that are presumed to originate from the neuroectodermal crest. PNET/EWSs are highly aggressive malignancies that usually present in the form of bone or soft tissue masses and usually affect adolescents and young adults. Primary PNET/EWS of the kidney is very rare. CASE PRESENTATION: We present the case of a 32-year-old female patient with primary renal PNET/EWS diagnosed nine years and eight months earlier...
July 2016: Nephro-urology Monthly
Xin Jin, Jianfeng Cao, Yong Liu, Fang Bian, Qingqing Zhao, Yan Wang, Xu Lv, Yayong Huang
Primitive neuroectodermal tumors (PNETs) are small, round cell tumors that may be classified as peripheral or central, based on their site of origin. PNETs often arise in the soft tissue or bone of young adults. Although not common, PNETs have been described in other organs, including the gonads, kidneys, myocardium and pancreas, but rarely in the lungs without chest wall or pleural involvement. The present study reports a rare case of peripheral PNET (pPNET), which originated in the lung. A 37-year-old female patient presented at Xuzhou Central Hospital (Xuzhou, China) with a history of a dry cough, mild dyspnea and slight pain in the left chest...
October 2016: Oncology Letters
Ahitagni Biswas, Lakhan Kashyap, Aanchal Kakkar, Chitra Sarkar, Pramod Kumar Julka
Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal central nervous system tumor commonly affecting children <3 years of age. It roughly constitutes 1%-2% of all pediatric central nervous system tumors. Recent data show that it is the most common malignant central nervous system tumor in children <6 months of age. Management of this aggressive tumor is associated with a myriad of diagnostic and therapeutic challenges. On the basis of radiology and histopathology alone, distinction of AT/RT from medulloblastoma or primitive neuroectodermal tumor is difficult, and hence this tumor has been commonly misdiagnosed as primitive neuroectodermal tumor for decades...
2016: Cancer Management and Research
Emil Lou, Matthew Schomaker, Jon D Wilson, Mary Ahrens, Michelle Dolan, Andrew C Nelson
Medulloblastoma is an aggressive primitive neuroectodermal tumor of the cerebellum that is rare in adults. Medulloblastomas fall into 4 prognostically significant molecular subgroups that are best defined by experimental gene expression profiles: the WNT pathway, sonic hedgehog (SHH) pathway, and subgroups 3 and 4 (non-SHH/WNT). Medulloblastoma of adults belong primarily to the SHH category. Vismodegib, an SHH-pathway inhibitor FDA-approved in 2012 for treatment of basal cell carcinoma, has been used successfully in the setting of chemorefractory medulloblastoma, but not as a first-line therapy...
August 12, 2016: Cancer Biology & Therapy
Weishu Hou, Liyan Xu, Heqin Zhan, Haibao Wang, Man Xu, Yongqiang Yu
PURPOSE: The aim of this study was to analyze the radiological features of peripheral primitive neuroectodermal tumor (pPNET). MATERIALS AND METHODS: The radiological and clinical findings for 16 patients with pPNETs were retrospectively reviewed. The 16 tumors were classified into 4 groups (meninges group, n = 4; spine group, n = 3; bone group, n = 5; soft-tissue group, n = 4), and clinical data, size, and common and unique CT/MRI characteristics were assessed...
September 27, 2016: Journal of Computer Assisted Tomography
Zhe Liu, Yuan-Hong Xu, Chun-Lin Ge, Jin Long, Rui-Xia Du, Ke-Jian Guo
Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor (E-EWS/pPNET) is a rare aggressive malignant small round cell tumor. In this report, we present the case of a 15-year-old boy who suffered from acute abdominal pain accompanied by hematemesis and melena, and was eventually diagnosed with E-EWS/pPNET. To date, there have been only five reported cases of E-EWS/pPNET of the small bowel including the patient in this report. To the best of our knowledge, this is the first documentation of a pPNET of the small bowel mesentery at nonage...
September 16, 2016: World Journal of Clinical Cases
Zehra Akkaya, Elif Peker, Basak Gulpinar, Hale Karadag, Ayse Erden
BACKGROUND: Primary renal primitive neuroectodermal tumor/extraskeletal Ewing's sarcoma (PNET/EES) is a very rare renal tumor. CASE REPORT: We report a case of primary renal PNET/EES of the kidney in an adult patient and describe its computed tomography and magnetic resonance imaging findings, including diffusion weighted images along with a review of the current medical literature. CONCLUSIONS: Although very rare, a relatively large renal mass which shows very infiltrative growth pattern on CT and MR imaging and striking diffusion restriction should raise the suspicion of a renal primitive neuroectodermal tumor, in a young adult...
2016: Polish Journal of Radiology
Masako Ishiguro, Mutsumi Yuki, Tomoko Fukushige, Mikio Mizoguchi, Yasuhiko Kaneko, Takeshita Morishige, Hiroshi Iwasaki
Ewing's sarcoma/primitive neuroectodermal tumor/Askin's tumor (Ewing`s sarcoma family of tumors: ESFT) is the most common type of malignant tumor of bone and soft tissue in children and young adults, and morphologically is a member of a group of small round cell tumors. We report, here, on the establishment of two human ESFT cell lines, FU-PNET-3 and FU-PNET-4, from the iliac and the chest wall, respectively, the cells of both cell lines were tumorigenic in immunodeficient mice. Histologically, both original and xenograft tumors and cultured cells were composed of small round cells with positive immunoreactivity for CD99 and Nkx2...
September 9, 2016: Human Cell
Mouna Khmou, Abderrahmane Malihy, Najat Lamalmi, Lamia Rouas, Zaitouna Alhamany
BACKGROUND: Peripheral primitive neuroectodermal tumors are extremely rare tumors in the spine; only 18 cases of extra-dural peripheral primitive neuroectodermal tumor cervical region have been reported. The aim of this report is to highlight the challenges in diagnosis and management of this condition. CASE PRESENTATION: We present a case of 5-year-old Moroccan boy, who presented with torticollis for 1 month. Computed tomography scan and Magnetic resonance imaging of the cervical spine revealed an extradural, dumbbell-shaped mass with extra-spinal extension at the left C1-C6 level...
2016: BMC Research Notes
Maithili Mandar Kulkarni, Siddhi Gaurish Sinai Khandeparkar, Avinash R Joshi, Chitrangi Barpande
Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) family of tumors is an uncommon group of malignant neoplasms that may present in both skeletal and extraskeletal sites. PNET outside the central nervous system is called peripheral PNET (pPNET) developing from migrating embryonal cells of the neural crest. Very few cases of pPNET of the maxilla are reported in English literature. These tumors may be difficult to diagnose due to their primitive morphology. These tumors occur predominantly in infancy or early childhood...
May 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
Vinodh Pillai, David M Dorfman
Many epithelial neoplasms can be analyzed by flow cytometry (FC), particularly from serous cavity effusion samples, using EpCAM, a cell adhesion molecule expressed on most normal epithelial cells and expressed at a higher level in most epithelial neoplasms. A simple 3-color flow cytometric panel can provide a high sensitivity and specificity compared to cytomorphology. FC provides more rapid immunophenotyping than conventional immunohistochemical staining, can identify rare malignant cells that could be missed by a cytological exam alone, and can be utilized to evaluate limited samples such as cerebrospinal fluid or fine-needle aspiration samples...
2016: Acta Cytologica
Iole Cordone, Serena Masi, Mariantonia Carosi, Antonello Vidiri, Francesco Marchesi, Mirella Marino, Stefano Telera, Alessia Pasquale, Andrea Mengarelli, Laura Conti, Edoardo Pescarmona, Andrea Pace, Carmine M Carapella
BACKGROUND: Brain stereotactic biopsy (SB) followed by conventional histopathology and immunohistochemistry (IHC) is the gold standard approach for primary central nervous system lymphoma (PCNSL) diagnosis. Flow cytometry (FCM) characterization of fine-needle aspiration cytology and core needle biopsies are increasingly utilized to diagnose lymphomas however, no biological data have been published on FCM characterization of fresh single cell suspension from PCNSL SB. The aim of this study was to establish the feasibility and utility of FCM for the diagnosis and characterization of brain lymphomas from a tissue samples obtained by a single SB disaggregation...
2016: Journal of Experimental & Clinical Cancer Research: CR
Radamés Ádamo Zuquello, Giordano Tagliari, Rodrigo Bagatini, Ricardo Hohmann Camiña, Ruggero Caron, Nadia Aparecida Lorencette, Antuani Rafael Baptistella, Gabriel Manfro
BACKGROUND: Germ cell tumors are tumors composed of tissues derived from more than one of the three germinal layers. They are more common in the testes and ovaries, but can present in many different regions in the midline, including the sacral region, retroperitoneum, mediastinum, and brain. Testicular germ cell tumors generally metastasize to the retroperitoneum, lungs, and brain; metastases to soft tissue are very rare. CASE PRESENTATION: Here we describe a case of a single soft-tissue mass in the thigh of a 27-year-old man, with histology showing areas of mature teratoma tissues derived from the ectodermal and mesodermal lineages, and areas of immature teratoma tissue composed of small undifferentiated cells, with primitive neuroectodermal differentiation foci forming neuroepithelial elements - thus classified as immature teratoma...
2016: Diagnostic Pathology
Robert E Emerson, Chia-Sui Kao, John N Eble, David J Grignon, Mingsheng Wang, Shaobo Zhang, Xiaoyan Wang, Rong Fan, Timothy A Masterson, Lawrence M Roth, Liang Cheng
AIMS: Sacrococcygeal teratomas are rare tumours that occur most frequently in neonates, although adult cases also occur. The molecular pathogenesis of these tumours and their long-term prognosis is uncertain. We investigated the i(12p) status of a large number of primary sacrococcygeal teratomas in both children and adults, including cases with malignant germ cell tumour elements. METHODS AND RESULTS: Fifty-four sacrococcygeal teratoma specimens from 52 patients were identified, and available follow-up information was obtained...
August 13, 2016: Histopathology
Tao Jiang, Junmei Wang, Ying Wang, Chunde Li
BACKGROUND: Very young children with Gorlin syndrome are at risk for developing medulloblastoma. Patients with Gorlin syndrome may have multiple system abnormalities, including basal cell carcinomas, jaw cysts, desmoplastic medulloblastoma, palmar/plantar pits, rib abnormalities, and intracranial falx calcification. The early diagnosis of Gorlin syndrome in desmoplastic medulloblastoma patients is very important because these patients should receive chemotherapy as a first-line treatment and should avoid radiotherapy as much as possible...
2016: World Journal of Surgical Oncology
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