open access

Vol 45, No 2 (2011)
OPIS PRZYPADKU
Submitted: 2010-10-18
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Ecchordosis physaliphora – a case report and a review of notochord-derived lesions

Dariusz Adamek, Michalina Malec, Natalia Grabska, Anna Krygowska-Wajs, Krystyna Gałązka
DOI: 10.1016/S0028-3843(14)60029-3
·
Neurol Neurochir Pol 2011;45(2):169-173.

open access

Vol 45, No 2 (2011)
OPIS PRZYPADKU
Submitted: 2010-10-18

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.

Get Citation

Keywords

chordoma, notochord, basilar artery, nucleus pulposus, autopsy

About this article
Title

Ecchordosis physaliphora – a case report and a review of notochord-derived lesions

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 45, No 2 (2011)

Pages

169-173

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

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