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Anaplastic ependymoma with holocordal and intracranial meningeal carcinomatosis and holospinal bone metastases.
MedLine Citation:
PMID:  23422903     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
BACKGROUND AND IMPORTANCE: : Ependymomas are the most frequent intramedullary neoplasms in adult patients. Anaplastic histology, extramedullary location, meningeal dissemination at initial diagnosis, and extraneural metastases are rare findings. We describe a case of extramedullary anaplastic ependymoma that presented with holocordal and intracranial leptomeningeal carcinomatosis and bone metastases in all the vertebral bodies and the sternum. Such an aggressive dissemination at initial diagnosis has not been previously reported.
CLINICAL PRESENTATION: : A 36-year-old woman presented with headache, multiple cranial nerve palsies, visual hallucinations, confusion, hemiparesis, hemihipoestesia, episodes of disconnection, and toxic syndrome. Magnetic resonance imaging and positron emission tomography scan revealed leptomeningeal carcinomatosis in the brainstem, the cerebellum, and along the whole spinal cord. Various nodular, intradural extramedullary lesions were present at multiple dorsal and lumbar levels. Metastatic bone disease affected all the vertebral bodies and various extraspinal bones. An intradural and bone biopsy was performed at L4, providing the diagnosis of anaplastic ependymoma (World Health Organization grade III) with focal neuronal differentiation. Despite chemotherapy, the patient's symptoms quickly progressed, and she died 7 weeks after diagnosis.
CONCLUSION: : To our knowledge, there are no previous descriptions of ependymomas with this extensive leptomeningeal, spinal, intracranial, and extraneural dissemination at clinical onset. Bone metastases in spinal ependymoma have not been previously reported. ABBREVIATION:: WHO, World Health Organization.
Authors:
Jordi Pérez-Bovet; Jordi Rimbau-Muñoz; Secundino Martín-Ferrer
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Neurosurgery     Volume:  72     ISSN:  1524-4040     ISO Abbreviation:  Neurosurgery     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-02-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7802914     Medline TA:  Neurosurgery     Country:  United States    
Other Details:
Languages:  eng     Pagination:  E497-504     Citation Subset:  IM    
Affiliation:
Neurosurgery Department, University Hospital Dr Josep Trueta, Girona (Girona), Spain.
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