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primitive neuroectodermal tumor

Hanane Eddaoualline, Khadija Mazouz, Bouchra Rafiq, Ghizlane El Mghari Tabib, Nawal El Ansari, Rhizlane Belbaraka, Abdelhamid El Omrani, Mouna Khouchani
BACKGROUND: Ewing sarcoma/primitive neuroectodermal tumor is a family of highly malignant proliferation of neuroectodermal origin, most often skeletal, adrenal localization is extremely rare. Only few cases have been reported in the literature. Classical management includes radical surgery with adjuvant chemotherapy or radiotherapy or both. This case report is the only one where recurrence was surgically removed, and it confirms the importance of adjuvant treatment, and the efficacy of neoadjuvant chemotherapy...
March 16, 2018: Journal of Medical Case Reports
Revathi Rajagopal, Marianne Phillips, Nicholas G Gottardo
The initial signs of hepatic sinusoidal obstruction syndrome (HSOS) can be challenging to recognize in children, especially outside the hematopoietic stem cell transplantation setting. To assist clinicians to promptly identify HSOS, the European Society for Blood and Marrow Transplantation has proposed pediatric HSOS diagnostic criteria which emphasize unexplained consumptive and transfusion-refractory thrombocytopenia. To highlight the importance of these "bellwether" early signs of HSOS and the efficacy of pre-emptive treatment with defibrotide, we describe the case of a child with a right 11th rib primitive neuroectodermal tumor who developed HSOS following focal radiotherapy and actinomycin-D treatment...
March 14, 2018: Pediatric Blood & Cancer
Robert Y Shih, Kelly K Koeller
Embryonal tumors of the central nervous system (CNS) are highly malignant undifferentiated or poorly differentiated tumors of neuroepithelial origin and have been defined as a category in the World Health Organization (WHO) classification since the first edition of the "Blue Book" in 1979. This category has evolved over time to reflect our ever-improving understanding of tumor biology and behavior. With the most recent update in 2016, many previous histologic diagnoses incorporate molecular parameters for the first time (genetically defined entities)...
March 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Kirti Gupta, Vikram Singh, Ashish Aggarwal, Pravin Salunke
We describe a rare example of infratentorial primitive neuroectodermal tumor categorized as embryonal tumor with multilayered rosettes diagnosed on intra-operative squash smear and frozen section and discuss its key diagnostic features, pitfalls and differentials. Correct interpretation at the time of frozen section is crucial as it helps in deciding the further course of surgery. To the best of our knowledge, this is the first such report in the literature.
March 7, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Xiaoping Yi, Moling Zhou, Shenghui Liao, Guanghui Gong, Hongling Yin, Yongheng Chen, Weihua Liao
AIM: To summarize the imaging features of spinal peripheral primitive neuroectodermal tumor (spPNET) patients. MATERIAL AND METHODS: The computed tomography (CT) and magnetic resonance imaging (MRI) features of ten spPNET patients were retrospectively analyzed, and the clinicopathological data were reviewed. RESULTS: Four men and six women with a mean age of 24.7 years (range, 3-44 years) were included. A total of ten spPNET lesions were found in the ten patients, including six extradural and four intradural extramedullary lesions...
January 9, 2018: Turkish Neurosurgery
Sreedharan Thankarajan Arunraj, Girish Kumar Parida, Nishikant Avinash Damle, Saurabh Arora, Sreenivasa Reddy, Dhritiman Chakraborty, Meghna Prabhu, Madhavi Tripathi, Chandrasekhar Bal
Medulloblastoma, also known as cerebellar primitive neuroectodermal tumor, is the most common brain tumor in children and arises in the posterior cranial fossa. We present the case of a patient with desmoplastic type of medulloblastoma, which showed recurrence more than once. When Ga-DOTANOC PET-CT was done, the lesions showed somatostatin receptor expression, opening another potential therapeutic option for this patient.
February 27, 2018: Clinical Nuclear Medicine
Laetitia Padovani, Frédérique Chapon, Nicolas André, Mohamed Boucekine, Anne Geoffray, Franck Bourdeau, Julien Masliah-Planchon, Line Claude, Aymeri Huchet, Anne Laprie, Stephane Supiot, Bernard Coche-Dequéant, Christine Kerr, Claire Alapetite, Julie Leseur, Tandat Nguyen, Sophie Chapet, Valerie Bernier, Pierre-Yves Bondiau, Georges Noel, Jean Louis Habrand, Stephanie Bolle, François Doz, Christelle Dufour, Xavier Muracciole, Christian Carrie
PURPOSE: To identify the incidence of patients with perihippocampal metastases to assess the risk of brain relapse when sparing the hippocampal area. Medulloblastoma (MB) represents 20% of pediatric brain tumors. For high-risk MB patients, the 3- to 5-year event-free survival rate has recently improved from 50% to >76%. Many survivors, however, experience neurocognitive side effects. Several retrospective studies of patients receiving whole brain irradiation (WBI) have suggested a relationship between the radiation dose to the hippocampus and neurocognitive decline...
March 15, 2018: International Journal of Radiation Oncology, Biology, Physics
Levent Soydan, Ali Aslan Demir, Elif Sayman, Burcu Onomay Celik, Bala Basak Oven Ustaalioglu
Ewing sarcoma and peripheral primitive neuroectodermal tumor belong to the Ewing sarcoma (ES) family of tumors originating from a primitive neural tube. We report a 31-year-old man who was admitted to the urology clinic with complaints of fever, nausea, and dysuria. A right-sided adrenal mass was detected during ultrasonography. The lesion was then evaluated with magnetic resonance imaging, which showed areas of necrosis amid heterogeneous solid areas. Whole body scan with 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography and bone scan studies showed pulmonary and osseous metastatic foci...
December 2017: Radiology Case Reports
Rosalba De Nola, Edoardo Di Naro, Luca Maria Schonauer, Giuseppe Lucarelli, Michele Battaglia, Maria Grazia Fiore, Salvatore Andrea Mastrolia, Giuseppe Loverro
RATIONALE: PNETs (primitive neuroectodermal tumors) are a family of highly malignant neoplasms characterized by small round cells of neuroepithelial origin. They usually involve bone and soft tissues, and have a higher incidence in childhood. PATIENT CONCERNS: In this case report, we describe the obstetric and oncological outcome of a huge mass diagnosed as a leiomyoma in a 39-year-old pregnant woman who complained of low back pain, dysuria, and urinary frequency at 22 weeks of gestation...
January 2018: Medicine (Baltimore)
Xin He, Zhongping Chen, Yutong Dong, Dan Tong
RATIONALE: Central nervous system primitive neuroectodermal tumors (CNS PNETs) mostly occur in children and present as cerebellar medulloblastoma. A few cases of PNETs occur in the cerebral hemisphere. The presence of a PNET in ventricles is extremely rare. The prognosis of CNS PNET is extremely poor, and the 5-year survival rate does not exceed 35%. In the present study, we describe the first case of a PNET in the ventricles with good prognosis. PATIENT CONCERNS: The case of a 36-year-old man is reported, who presented with a progressively worsening headache for 2 months...
February 2018: Medicine (Baltimore)
G C Zhu
No abstract text is available yet for this article.
February 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
James O Galle, David E Long, Tim Lautenschlaeger, Richard C Zellars, Gordon A Watson, Susannah G Ellsworth
PURPOSE: To analyze effects of closure of an academic proton treatment center (PTC) on pediatric case volume, distribution, and resident education. METHODS AND MATERIALS: This was a review of 412 consecutive pediatric (age ≤18 years) cases treated at a single institution from 2012 to 2016. Residents' Accreditation Council for Graduate Medical Education case logs for the same years were also analyzed. Characteristics of the patient population and resident case volumes before and after closure of the PTC are reported...
March 1, 2018: International Journal of Radiation Oncology, Biology, Physics
Satyashiva Munjal, Amit Srivastava, Shivya Tucker, Neha Bakshi, Sunita Bhalla, V S Mehta
Primary spinal primitive neuroectodermal tumor (psPNET) is a rare entity with few cases reported in literature. We report a case of a 50-year-old female who presented to us with paraplegia and was diagnosed with extradural dorsal spine psPNET. The diagnosis was not suspected at presentation or on radiology but was established on histopathological examination. It is important to distinguish it from central nervous system primitive neuroectodermal tumors and from other spinal tumors since it follows a different clinical course and therapeutic outcome...
October 2017: Journal of Craniovertebral Junction and Spine
B Sipos
Neuroendocrine neoplasms (NENs) are infrequent in sensory organs. There are well-differentiated neuroendocrine neoplasms that should be classified as neuroendocrine tumors, in analogy to their gastrointestinal counterparts, however the nomenclature is inconsistent. The best defined entities are neuroendocrine tumors in the middle ear and ectopic pituitary adenoma in the sphenoid region. Poorly differentiated NENs most often arise in the olfactory organ and nasal cavity that are represented by olfactory neuroblastomas and poorly differentiated neuroendocrine carcinomas...
February 1, 2018: Der Pathologe
Qing Li, Yong Liu, Yang Yu
RATIONALE: Peripheral primitive neuroectodermal tumor (PNET) is a kind of small round cell tumor derived from primitive neuroectodermal tumor. PATIENT CONCERNS: PNET is a highly malignant tumor that is subordinated to Ewing sarcoma. It occurs predominantly in soft tissue and bone and rarely in the bronchi and lung. Traditional surgery, radiotherapy, and chemotherapy are used for the treatment of PNET, but are usually ineffective. DIAGNOSES: There was a rare case of a 17 year-old man diagnoses with primary pulmonary PNET...
December 2017: Medicine (Baltimore)
Pei-Sen Yao, Guo-Rong Chen, Huang-Cheng Shang-Guan, Qing-Song Lin, Xing-Fu Wang, Shu-Fa Zheng, De-Zhi Kang
RATIONALE: Intracranial ganglioneuroblastoma represents a rare subtype of primitive neuroectodermal tumor. Here, we report a hippocampal ganglioneuroblastoma and a literature review of cerebral anglioneuroblastoma is carried out. PATIENT CONCERNS: We report a 16-year-old male patient presenting with absence seizure and high-infiltration hippocampal ganglioneuroblastoma. INTERVENTIONS: Magnetic resonance imaging (MRI) indicates a space-occupying lesion with a well-defined margin in the right temporal lobe and hippocampus...
December 2017: Medicine (Baltimore)
Muhammad W Saif, Kristin Kaley
Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare but aggressive and malignant tumor, and has been reported in various sites such as the lungs, biliary tract, kidney, prostate, stomach, esophagus, oral cavity, salivary glands, urinary bladder, uterus, cervix, gonads, and vagina. However, the pancreas is considered to be an extremely uncommon site and only a handful of cases have been published to date. We present here another case of a pancreatic ES/PNET. Our case intensifies the importance to recognize this rare type of tumor in the pancreas as there is a broad spectrum of tumors with a similar morphology that includes sheets of small, round blue cells...
November 26, 2017: Curēus
Peter Paul Lim, Jagadeesh Ramdas, Michal Ann Miller, Conrad Schuerch, George Wu
Primary leptomeningeal primitive neuroectodermal tumors (PNETs) are extremely rare childhood central nervous system malignancies harboring a very poor prognosis. There is no consensus treatment for these tumors to date. We report a case of a 10-year-old male who presented with mental status change, hydrocephalus, intracranial and spinal diffuse leptomeningeal enhancement without a primary mass upon cranial imaging and a negative initial biopsy until five months into his presentation. He responded significantly well to initial chemotherapy and radiotherapy...
December 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
Ana S Guerreiro Stucklin, Vijay Ramaswamy, Craig Daniels, Michael D Taylor
PURPOSE OF REVIEW: Brain tumors are the most common solid tumors and leading cause of cancer-related death in children. The advent of large-scale genomics has resulted in a plethora of profiling studies that have mapped the genetic and epigenetic landscapes of pediatric brain tumors, ringing in a new era of precision diagnostics and targeted therapies. In this review, we highlight the most recent findings, focusing on studies published after 2015, and discuss how new evidence is changing the care of children with brain tumors...
February 2018: Current Opinion in Pediatrics
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