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Fetal presentation of congenital fibrosarcoma of the meninges: case report and literature review.
MedLine Citation:
PMID:  25492889     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Aim: Congenital infantile fibrosarcoma (CIFS) exceptionally occurs in the meninges, with no cases reported beforeor at birth. We report herein a meningeal CIFS diagnosed in a fetus at 40 weeks of gestation (WG). Case report: A 24-yearoldpregnant woman was referred to theobstetricsdepartment for vaginal bleeding. A severe right hydrocephalus due to a large tumor invading the left hemisphere and ventricles was discovered in the fetus, and medical termination of the pregnancy was achieved. Results: Histological examination revealed a highly cellular spindle or ovoid shaped cell proliferation organized in interlacing bundles; it was diffusely positive for vimentin, and scarcely for SMA (smooth muscle actin). NFs (neurofilaments), NeuN, S100 protein, desmin, GFAP (glial fibrillary acidic protein), Olig2, chromogranin, synaptophysin, CD31, CD34, BCL2, and EMA (epithelial membrane antigen) antibodies were negative. Ki67 antibody labeled 20% of the nuclei. A reverse transcription polymerase chain reaction assay was performed to detect the gene fusion ETV6-NTRK3 transcript. Despite negative results, it was concluded to be a CIFS of the meninges. Discussion: CIFS of the meninges during the fetal period has never been reported. Its diagnosis is based on immunohistochemistry, and, whenever possible, on the detection of the reciprocal translocation t (12;15) resulting in the gene fusion ETV6-NTRK3. Its prognosis depends on rapid growth and local invasiveness leading to cerebral structure damage.
Authors:
Florent Marguet; Lise Bergogne; Nicole Laurent; Thierry Rousseau; Annie Laquerrière
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-12-5
Journal Detail:
Title:  Clinical neuropathology     Volume:  -     ISSN:  0722-5091     ISO Abbreviation:  Clin. Neuropathol.     Publication Date:  2014 Dec 
Date Detail:
Created Date:  2014-12-10     Completed Date:  -     Revised Date:  2014-12-11    
Medline Journal Info:
Nlm Unique ID:  8214420     Medline TA:  Clin Neuropathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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