Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report

Maryam Almurshed; Lamia Alsarraf

doi:doi:10.11648/j.cnn.20230701.11

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Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report

Maryam Almurshed, Lamia Alsarraf

Published in Clinical Neurology and Neuroscience (Volume 7, Issue 1)

Received: 23 December 2022 Accepted: 30 January 2023 Published: 9 February 2023

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Abstract

Lesions of notochordal origin are unusual surgical cases that have a broad spectrum of differentiation, from remnants to benign tumors -namely “benign notochordal cell tumor BNCT”- to malignant chordoma. These lesions arise within the vertebral bodies at the ends of axial skeleton, clivus and lumbar areas. Chordoma, is a malignant destructive lesion extending from bone to surrounding soft tissue. It is hypothesized to arise from its rare benign counterpart, BNCT as evidenced by their co-existence in rare case reports, in lumbar area, an even more rare in clival region. These benign forms are even scarce to see in surgical pathology specimen and carry a histological resemblance in morphology to malignant chordoma if not recognized. Here, we report a case of chordoma arising from a benign notochordal cell tumor (BNCT), which was discovered in a 35 years old male who presented with diplopia, diagnosed as 6^th nerve palsy. MRI imaging showed an expansile clival lesion with sclerotic borders invading the cavernous sinus and encasing the carotid. Histopathology showed features of chordoma within BNCT. This case highlights the rarity of the entity especially in the clival region, the wide differential diagnosis of clear cell tumor arising in the clival region and the importance of radio-pathological correlation.

Published in	Clinical Neurology and Neuroscience (Volume 7, Issue 1)
DOI	10.11648/j.cnn.20230701.11
Page(s)	1-4
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2023. Published by Science Publishing Group

Keywords

Chordoma, Benign Notochordal Cell Tumor, Morphology, Clival Mass

References

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[3]	Nishiguchi T, Mochizuki K, Tsujio T, Nishita T, Inoue Y. Lumbar vertebral chordoma arising from an intraosseous benign notochordal cell tumour: radiological findings and histopathological description with a good clinical outcome. Br J Radiol. 2010 Mar; 83 (987): e49-53. doi: 10.1259/bjr/63846600. PMID: 20197427; PMCID: PMC3473552.
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APA Style

Maryam Almurshed, Lamia Alsarraf. (2023). Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report. Clinical Neurology and Neuroscience, 7(1), 1-4. https://doi.org/10.11648/j.cnn.20230701.11

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ACS Style

Maryam Almurshed; Lamia Alsarraf. Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report. Clin. Neurol. Neurosci. 2023, 7(1), 1-4. doi: 10.11648/j.cnn.20230701.11

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AMA Style

Maryam Almurshed, Lamia Alsarraf. Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report. Clin Neurol Neurosci. 2023;7(1):1-4. doi: 10.11648/j.cnn.20230701.11

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@article{10.11648/j.cnn.20230701.11,
  author = {Maryam Almurshed and Lamia Alsarraf},
  title = {Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report},
  journal = {Clinical Neurology and Neuroscience},
  volume = {7},
  number = {1},
  pages = {1-4},
  doi = {10.11648/j.cnn.20230701.11},
  url = {https://doi.org/10.11648/j.cnn.20230701.11},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.cnn.20230701.11},
  abstract = {Lesions of notochordal origin are unusual surgical cases that have a broad spectrum of differentiation, from remnants to benign tumors -namely “benign notochordal cell tumor BNCT”- to malignant chordoma. These lesions arise within the vertebral bodies at the ends of axial skeleton, clivus and lumbar areas. Chordoma, is a malignant destructive lesion extending from bone to surrounding soft tissue. It is hypothesized to arise from its rare benign counterpart, BNCT as evidenced by their co-existence in rare case reports, in lumbar area, an even more rare in clival region. These benign forms are even scarce to see in surgical pathology specimen and carry a histological resemblance in morphology to malignant chordoma if not recognized. Here, we report a case of chordoma arising from a benign notochordal cell tumor (BNCT), which was discovered in a 35 years old male who presented with diplopia, diagnosed as 6th nerve palsy. MRI imaging showed an expansile clival lesion with sclerotic borders invading the cavernous sinus and encasing the carotid. Histopathology showed features of chordoma within BNCT. This case highlights the rarity of the entity especially in the clival region, the wide differential diagnosis of clear cell tumor arising in the clival region and the importance of radio-pathological correlation.},
 year = {2023}
}

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TY  - JOUR
T1  - Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report
AU  - Maryam Almurshed
AU  - Lamia Alsarraf
Y1  - 2023/02/09
PY  - 2023
N1  - https://doi.org/10.11648/j.cnn.20230701.11
DO  - 10.11648/j.cnn.20230701.11
T2  - Clinical Neurology and Neuroscience
JF  - Clinical Neurology and Neuroscience
JO  - Clinical Neurology and Neuroscience
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PB  - Science Publishing Group
SN  - 2578-8930
UR  - https://doi.org/10.11648/j.cnn.20230701.11
AB  - Lesions of notochordal origin are unusual surgical cases that have a broad spectrum of differentiation, from remnants to benign tumors -namely “benign notochordal cell tumor BNCT”- to malignant chordoma. These lesions arise within the vertebral bodies at the ends of axial skeleton, clivus and lumbar areas. Chordoma, is a malignant destructive lesion extending from bone to surrounding soft tissue. It is hypothesized to arise from its rare benign counterpart, BNCT as evidenced by their co-existence in rare case reports, in lumbar area, an even more rare in clival region. These benign forms are even scarce to see in surgical pathology specimen and carry a histological resemblance in morphology to malignant chordoma if not recognized. Here, we report a case of chordoma arising from a benign notochordal cell tumor (BNCT), which was discovered in a 35 years old male who presented with diplopia, diagnosed as 6th nerve palsy. MRI imaging showed an expansile clival lesion with sclerotic borders invading the cavernous sinus and encasing the carotid. Histopathology showed features of chordoma within BNCT. This case highlights the rarity of the entity especially in the clival region, the wide differential diagnosis of clear cell tumor arising in the clival region and the importance of radio-pathological correlation.
VL  - 7
IS  - 1
ER  -

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Author Information

Maryam Almurshed

Department of Pathology, Al Sabah Hospital, Kuwait
Lamia Alsarraf

Department of Radiology, Ibn-Sina Hospital, Kuwait

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Plain Text BibTeX RIS

APA Style

Maryam Almurshed, Lamia Alsarraf. (2023). Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report. Clinical Neurology and Neuroscience, 7(1), 1-4. https://doi.org/10.11648/j.cnn.20230701.11

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ACS Style

Maryam Almurshed; Lamia Alsarraf. Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report. Clin. Neurol. Neurosci. 2023, 7(1), 1-4. doi: 10.11648/j.cnn.20230701.11

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AMA Style

Maryam Almurshed, Lamia Alsarraf. Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report. Clin Neurol Neurosci. 2023;7(1):1-4. doi: 10.11648/j.cnn.20230701.11

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@article{10.11648/j.cnn.20230701.11,
  author = {Maryam Almurshed and Lamia Alsarraf},
  title = {Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report},
  journal = {Clinical Neurology and Neuroscience},
  volume = {7},
  number = {1},
  pages = {1-4},
  doi = {10.11648/j.cnn.20230701.11},
  url = {https://doi.org/10.11648/j.cnn.20230701.11},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.cnn.20230701.11},
  abstract = {Lesions of notochordal origin are unusual surgical cases that have a broad spectrum of differentiation, from remnants to benign tumors -namely “benign notochordal cell tumor BNCT”- to malignant chordoma. These lesions arise within the vertebral bodies at the ends of axial skeleton, clivus and lumbar areas. Chordoma, is a malignant destructive lesion extending from bone to surrounding soft tissue. It is hypothesized to arise from its rare benign counterpart, BNCT as evidenced by their co-existence in rare case reports, in lumbar area, an even more rare in clival region. These benign forms are even scarce to see in surgical pathology specimen and carry a histological resemblance in morphology to malignant chordoma if not recognized. Here, we report a case of chordoma arising from a benign notochordal cell tumor (BNCT), which was discovered in a 35 years old male who presented with diplopia, diagnosed as 6th nerve palsy. MRI imaging showed an expansile clival lesion with sclerotic borders invading the cavernous sinus and encasing the carotid. Histopathology showed features of chordoma within BNCT. This case highlights the rarity of the entity especially in the clival region, the wide differential diagnosis of clear cell tumor arising in the clival region and the importance of radio-pathological correlation.},
 year = {2023}
}

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TY  - JOUR
T1  - Clival Chordoma, Arising in Pre-existing Benign Notochordal Cell Tumor, Case Report
AU  - Maryam Almurshed
AU  - Lamia Alsarraf
Y1  - 2023/02/09
PY  - 2023
N1  - https://doi.org/10.11648/j.cnn.20230701.11
DO  - 10.11648/j.cnn.20230701.11
T2  - Clinical Neurology and Neuroscience
JF  - Clinical Neurology and Neuroscience
JO  - Clinical Neurology and Neuroscience
SP  - 1
EP  - 4
PB  - Science Publishing Group
SN  - 2578-8930
UR  - https://doi.org/10.11648/j.cnn.20230701.11
AB  - Lesions of notochordal origin are unusual surgical cases that have a broad spectrum of differentiation, from remnants to benign tumors -namely “benign notochordal cell tumor BNCT”- to malignant chordoma. These lesions arise within the vertebral bodies at the ends of axial skeleton, clivus and lumbar areas. Chordoma, is a malignant destructive lesion extending from bone to surrounding soft tissue. It is hypothesized to arise from its rare benign counterpart, BNCT as evidenced by their co-existence in rare case reports, in lumbar area, an even more rare in clival region. These benign forms are even scarce to see in surgical pathology specimen and carry a histological resemblance in morphology to malignant chordoma if not recognized. Here, we report a case of chordoma arising from a benign notochordal cell tumor (BNCT), which was discovered in a 35 years old male who presented with diplopia, diagnosed as 6th nerve palsy. MRI imaging showed an expansile clival lesion with sclerotic borders invading the cavernous sinus and encasing the carotid. Histopathology showed features of chordoma within BNCT. This case highlights the rarity of the entity especially in the clival region, the wide differential diagnosis of clear cell tumor arising in the clival region and the importance of radio-pathological correlation.
VL  - 7
IS  - 1
ER  -

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