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Ecchordosis physaliphora – a case report and a review of notochord-derived lesions
Affiliations- Acibadem Hospital, Department of Pathology, Istanbul/TURKEY, 34718 Istanbul, Türkiye
- Student of Medical Faculty, Jagiellonian University Medical College, Krakow
- 1 Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland
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Abstract
Some notochord cells remain along the axis of the vertebral column after embryogenesis. These ‘notochordal remnants’ have some similarities, but their biological behaviour varies considerably. They can give rise to benign lesions such as ecchordosis physaliphora (EP) and ‘benign notochordal cell tumour’ (BNCT), or aggressive ones like chordoma.
We review the problems of the differential diagnosis of notochordal remnants apropos of a case of the incidental autopsy finding of EP in a 78-year-old man, who died due to heart infarction. The 6-mm asymptomatic gelatinous lesion was fixed to the basilar artery on its ventral aspect.
Small EPs can be easily overlooked in autopsy. Ecchordosis physaliphora and intradural chordoma share some similarities that may be misleading and may even result in the wrong diagnosis and therapy. The recently reported new entity BNCT poses a similar problem. We review the literature illustrating the most important features of notochord-derived lesions and discuss the relationships between these lesions with regard to molecular genetics.
Abstract
Some notochord cells remain along the axis of the vertebral column after embryogenesis. These ‘notochordal remnants’ have some similarities, but their biological behaviour varies considerably. They can give rise to benign lesions such as ecchordosis physaliphora (EP) and ‘benign notochordal cell tumour’ (BNCT), or aggressive ones like chordoma.
We review the problems of the differential diagnosis of notochordal remnants apropos of a case of the incidental autopsy finding of EP in a 78-year-old man, who died due to heart infarction. The 6-mm asymptomatic gelatinous lesion was fixed to the basilar artery on its ventral aspect.
Small EPs can be easily overlooked in autopsy. Ecchordosis physaliphora and intradural chordoma share some similarities that may be misleading and may even result in the wrong diagnosis and therapy. The recently reported new entity BNCT poses a similar problem. We review the literature illustrating the most important features of notochord-derived lesions and discuss the relationships between these lesions with regard to molecular genetics.
Keywords
chordoma, notochord, basilar artery, nucleus pulposus, autopsy
About this articleTitle
Ecchordosis physaliphora – a case report and a review of notochord-derived lesions
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
169-173
Page views
478
Article views/downloads
1305
DOI
10.1016/S0028-3843(14)60029-3
Bibliographic record
Neurol Neurochir Pol 2011;45(2):169-173.
Keywords
chordoma
notochord
basilar artery
nucleus pulposus
autopsy
Authors
Dariusz Adamek
Michalina Malec
Natalia Grabska
Anna Krygowska-Wajs
Krystyna Gałązka
