Histological and genetic analysis of anaplastic pleomorphic xanthoastrocytoma suspected of malignant progression over a 12‐year clinical course

2019 ◽  
Vol 69 (10) ◽  
pp. 608-613
Author(s):  
Hiroko Fukushima ◽  
Yoshiko Nakano ◽  
Naomi Ishii ◽  
Nozomi Nozuchi ◽  
Takahiro Okuno ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Clinical Course ◽  
Pleomorphic Xanthoastrocytoma ◽  
Malignant Progression

Lowe syndrome in a 20-year-old male: the clinical course and genetic analysis in OCRL1

2011 ◽  
Vol 24 (2) ◽  
pp. 211-217
Author(s):  
Yuichirou Tsuji ◽  
Takahiro Murata ◽  
Hiroshi Watanabe ◽  
Toshikazu Shinbo ◽  
Sei Ishiguro ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Clinical Course ◽  
Lowe Syndrome

Pleomorphic xanthoastrocytoma presenting with life-threatening hemorrhage in a child

2009 ◽  
Vol 3 (2) ◽  
pp. 157-159 ◽  
Author(s):  
Joshua J. Wind ◽  
P. Benjamin Kerr ◽  
Jennifer A. Sweet ◽  
Vivek R. Deshmukh
Keyword(s):  
Intracerebral Hemorrhage ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Case Reports ◽  
Young Patients ◽  
Pleomorphic Xanthoastrocytoma ◽  
First Case ◽  
Life Threatening

Pleomorphic xanthoastrocytomas are glial-based tumors that arise most frequently in young patients and usually follow a more benign and indolent clinical course than their other glial-based tumor counterparts. These tumors most frequently present with seizures, and only 3 previous case reports exist of hemorrhagic tumor as the clinical presentation. The authors present the first case of life-threatening intracerebral hemorrhage from pleomorphic xanthoastrocytoma in a child.

Malignant Progression of a Pleomorphic Xanthoastrocytoma in a Child

2010 ◽  
Vol 41 (02) ◽  
pp. 69-71 ◽  
Author(s):  
G. A. Alexiou ◽  
M. Moschovi ◽  
K. Stefanaki ◽  
C. Prodromou ◽  
G. Sfakianos ◽  
...  
Keyword(s):  
Pleomorphic Xanthoastrocytoma ◽  
Malignant Progression

scholarly journals GENE-30. PRELIMINARY STUDY ON WHOLE-EXOME SEQUENCING TECHNOLOGY IN THE GENETIC ANALYSIS OF PEDIATRIC PATIENTS WITH GLIOMAS

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi104-vi104
Author(s):  
Mingyao Lai ◽  
Juan Li ◽  
Junjie Zhen ◽  
jiangfen zhou ◽  
Qingjun Hu ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Signaling Pathways ◽  
Exome Sequencing ◽  
Whole Exome Sequencing ◽  
Pediatric Patients ◽  
Genetic Mutation ◽  
Pleomorphic Xanthoastrocytoma ◽  
Pathological Examination ◽  
Sequencing Technology ◽  
Whole Exome

Abstract OBJECTIVE To analyze the genes related to the signaling pathways in pediatric gliomas and drug-related genes with whole-exome sequencing technology. METHODS The tumor tissues and matched blood samples of 17 enrolled patients were detected with whole-exome sequencing technology. There were 3 cases of diffuse midline gliomas, 2 cases of childhood glioblastomas, 3 cases of disffuse astrocytoma, 1 case of pleomorphic xanthoastrocytoma, 1 case of ganglioglioma, 6 cases of anaplastic ependymoma and 1 case of ependymoma in this study. All the enrolled patients who were no more than 14 years old received surgery in the Department of Neurosurgery, Guangdong Sanjiu Brain Hospital. The diagnosis was confirmed by pathological examination and the sample acquisition was approved by hospital ethics committee. RESULTS With the use of whole-exome sequencing technology, a total of 31 related genetic mutations were detected in 15 cases, while no genetic mutation was detected in the other 2 cases. The genes related to the signaling pathways in pediatric gliomas included ATRX, ASL1, BCOR, EP300, FGFR1, H3F3A, IGF1R, MED12, PIK3R1, PRKDC, RB1, SETD2, SMARCA4, SOX2, TGFBR2, and the drug-related genes included AKT1, BCL2, BRAF, BRCA2, CCND1, CCND2, CDK6, EGFR, FGF3, KRAS, MET, PDGFRA, PIK3CA, PTEN, TP53, TSC1. One patient only had genes related to the signaling pathways, and 14 patients had drug-related genes. CONCLUSION Applying whole-exome sequencing technology in the genetic analysis of pediatric patients with gliomas has remarkable guiding significance for revealing the mechanism of disease, searching for therapeutic targets and adopting individualized treatment, which can bring potential benefits to pediatric patients. However, more samples and further data analysis and verification are needed in future study.

Combined Pleomorphic Xanthoastrocytoma-Ganglioglioma of the Cerebellum

2000 ◽  
Vol 124 (11) ◽  
pp. 1707-1709 ◽  
Author(s):  
Andrew J. Evans ◽  
Imran Fayaz ◽  
Michael D. Cusimano ◽  
Norman Laperriere ◽  
Juan M. Bilbao
Keyword(s):  
Mitotic Activity ◽  
High Power ◽  
Clinical Course ◽  
Ganglion Cells ◽  
Postoperative Radiotherapy ◽  
Pleomorphic Xanthoastrocytoma ◽  
Mitotic Figure ◽  
Total Resection ◽  
Endothelial Proliferation

Abstract Combined pleomorphic xanthoastrocytoma-ganglioma is a rare neoplasm, occurring in patients younger than 30 years. The clinical course of these tumors is difficult to predict because of their rarity. We report a case of combined pleomorphic xanthoastrocytoma-ganglioma that, in addition to the patient's age, is unusual in several respects. The lesion was located in the cerebellar vermis of a 60-year-old man and was radiographically solid. Histologically, there was an admixture of markedly pleomorphic astrocytic cells and neoplastic ganglion cells, with permeation of the overlying leptomeninges and surrounding cerebellum. In addition, there was focal capillary endothelial proliferation. There was no necrosis, and mitotic activity was rare at 1 mitotic figure per 40 high-power fields. The patient underwent a near gross total resection and postoperative radiotherapy and remains well through 16 months of follow-up.

scholarly journals Molecular Genetic Analysis of Anaplastic Pleomorphic Xanthoastrocytoma

2003 ◽  
Vol 26 (2) ◽  
pp. 120-125 ◽  
Author(s):  
Noor Azam Nasuha ◽  
Abd. Hamid Daud ◽  
Mazira Mohamad Ghazali ◽  
Abdul Aziz Mohamed Yusoff ◽  
Norafiza Zainuddin ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Molecular Genetic ◽  
Pleomorphic Xanthoastrocytoma ◽  
Molecular Genetic Analysis

scholarly journals Polyostotic Fibrous Dysplasia in a Child with Bone Fractures and Neuropsychiatric Symptoms, Case Report

2019 ◽  
Vol 3 (1) ◽  
pp. 1-5
Author(s):  
Hala A Al Shaikh
Keyword(s):  
Genetic Analysis ◽  
Fibrous Dysplasia ◽  
Clinical Course ◽  
Bone Fractures ◽  
Neuropsychiatric Symptoms ◽  
Polyostotic Fibrous Dysplasia ◽  
Pathological Fractures ◽  
Mccune Albright Syndrome ◽  
Gnas Gene ◽  
Albright Syndrome

Fibrous dysplasia of the bone in children is a rare, genetic, heterogeneous disorder, associated with extra - skeletal manifestations in the McCune Albright Syndrome . It results from a post - zygotic activating mutation in the (GNAS) gene coding for (Gs alfa) protein. This leads to replacement of the lamellar bone with under - mineralized fibrous tissue, presenting clinically with limping, pain, and pathological fractures . Objective: To demonstrate the clinical course, challenges and treatment of a child with polyostotic fibrous dysplasia presenting with limping, extra skeletal manifestations, neuropsychiatric symptoms and bone fractures. Method : The clinical information and lab results were obtained directly from the mother and the computerized medical records at the treating hospitals. Genetic analysis was done at Washington University School of Medicine, USA. Informed publication consent was obtained from the parents. R esults : The child presented at the age of 3years with limping and was found to have polyostotic fibrous dysplasia and café au lait spot. Later on, he developed neurological symptoms. He had bone fractures in the proximal femur, requiring surgical intervent ion. Bone pain and frequency of fractures improved after Zoledronate injections. Blood genetic analysis of the GNAS - gene was negative. Conclusion : Polyostotic fibrous dysplasia is a rare cause of limping and recurrent pathological fractures in children. Me dical treatment with Biphosphanate injections can positively affect the clinical course of the disease. A negative blood genetic analysis of the GNAS gene does not exclude the McCune Albright Syndrome, and indicates analysis from the affected tissue. Neuro psychiatric symptoms may manifest with fibrous dysplasia without any endocrine involvement.

Delayed occurrence of cerebellar pleomorphic xanthoastrocytoma after supratentorial pleomorphic xanthoastrocytoma removal

1995 ◽  
Vol 82 (1) ◽  
pp. 116-118 ◽  
Author(s):  
Ryan S. Glasser ◽  
Amyn M. Rojiani ◽  
J. Parker Mickle ◽  
Thomas A. Eskin
Keyword(s):  
Glioblastoma Multiforme ◽  
Posterior Fossa ◽  
Clinical Characteristics ◽  
Clinical Course ◽  
Pleomorphic Xanthoastrocytoma ◽  
Gross Total Resection ◽  
Total Resection ◽  
Content Type ◽  
Atypical Features ◽  
Fine Print

✓ The authors report the case of a 36-year-old woman who underwent gross total resection of a right cerebellar pleomorphic xanthoastrocytoma with atypical features. She had undergone surgery 16 years previously for what was thought to be a right frontal glioblastoma multiforme. In retrospect, based on the histopathology and the clinical course, both lesions were considered to represent atypical variants of pleomorphic xanthoastrocytoma. This report examines the histological and clinical characteristics of this posterior fossa lesion, which exhibited histologically malignant features but has run a relatively indolent course.

Malignant progression in pleomorphic xanthoastrocytoma: Personal experience and review of the literature

2007 ◽  
Vol 252 (2) ◽  
pp. 144-153 ◽  
Author(s):  
Elisabetta Marton ◽  
Alberto Feletti ◽  
Enrico Orvieto ◽  
Pierluigi Longatti
Keyword(s):  
Personal Experience ◽  
Pleomorphic Xanthoastrocytoma ◽  
Malignant Progression ◽  
Review Of The Literature

Epidemiology, clinical course, and genetic analysis of pheochromocytomas/paragangliomas: A single centre tertiary care experience over 16 years from crete-greece

2021 ◽  
Author(s):  
Maria Chrysoulaki ◽  
Florentia Fostira ◽  
Vasiliki Daraki ◽  
Grigoria Betsi ◽  
Maria Sfakiotaki ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Clinical Course ◽  
Tertiary Care ◽  
Single Centre ◽  
Care Experience
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