scholarly journals Fraser syndrome: review of the literature illustrated by a historical adult case

2020 ◽  
Vol 49 (10) ◽  
pp. 1245-1253 ◽  
Author(s):  
J. Bouaoud ◽  
M. Olivetto ◽  
S. Testelin ◽  
S. Dakpe ◽  
J. Bettoni ◽  
...  
Keyword(s):  
Review Of The Literature ◽  
Adult Case ◽  
Fraser Syndrome

scholarly journals Cardiac Epithelioid PEComa: Report of Two Cases and Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Huilin Niu ◽  
F. W. Wang ◽  
Paul J. Zhang ◽  
Zhanyong Bing
Keyword(s):  
Clear Cell ◽  
Coagulation Necrosis ◽  
Review Of The Literature ◽  
Epithelioid Cells ◽  
Adult Case ◽  
Pediatric Case ◽  
High Mitotic Activity ◽  
Atypical Mitosis ◽  
Malignant Behavior ◽  
Hmb 45

Cardiac PEComa is very rare. We reported two cases of epithelioid PEComas, one in an adult and one in a 2-year-old child. Both tumors were composed of sheets of epithelioid cells with coagulation necrosis. In addition, the adult case showed marked nuclear atypia and high mitotic activity with atypical mitosis and the pediatric case showed unusual clear cell features. Immunohistochemically, both tumors were positive for HMB-45 and SMA and negative for S100 and cytokeratin. Electron microscopy was performed in the pediatric case and showed premelanosomes. The adult patient developed extensive metastasis indicating malignant behavior. Prior to the two cases, only 5 other cases of cardiac PEComa were reported and the literatures are reviewed.

scholarly journals Late-Onset N-Acetylglutamate Synthase Deficiency: Report of a Paradigmatic Adult Case Presenting with Headaches and Review of the Literature

2018 ◽  
Vol 19 (2) ◽  
pp. 345
Author(s):  
Catia Cavicchi ◽  
Chiara Chilleri ◽  
Antonella Fioravanti ◽  
Lorenzo Ferri ◽  
Francesco Ripandelli ◽  
...  
Keyword(s):  
Late Onset ◽  
Review Of The Literature ◽  
Adult Case ◽  
Acetylglutamate Synthase

Klippel-Feil Syndrome in Association with a Craniocervical Dermoid Cyst Presenting as Aseptic Meningitis in an Adult: Case Report

Neurosurgery ◽  
1989 ◽  
Vol 25 (4) ◽  
pp. 652-655 ◽  
Author(s):  
Bernadette Diekmann-Guiroy ◽  
Peter S. Huang
Keyword(s):  
Case Report ◽  
Dermoid Cyst ◽  
Aseptic Meningitis ◽  
Rare Case ◽  
Craniocervical Junction ◽  
Spinal Tumors ◽  
Intracranial Tumors ◽  
Review Of The Literature ◽  
Adult Case

Abstract Intracranial tumors associated with Klippel-Feil syndrome usually occur in children, with spinal tumors being more common in adults affected by the syndrome. A rare case of a dermoid cyst at the craniocervical junction presenting as aseptic meningitis in an adult with Klippel-Feil syndrome is described. A review of the literature on tumors associated with this syndrome is also presented.

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