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A Rare Presentation of Axial Chordoma and the Approach to Management

Patient: Female, 50 Final Diagnosis: Axial chordoma Symptoms: Back ache • numbness • urine incontinence • weaknes of lower limbs Medication: — Clinical Procedure: — Specialty: Plastic Surgery OBJECTIVE: Rare disease BACKGROUND: Chordoma is a primary bone tumor that most commonly arises in the sacroc...

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Autores principales: Muneer, Mohammed, Badran, Saif, Al-Hetmi, Talal
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6561141/
https://www.ncbi.nlm.nih.gov/pubmed/31152144
http://dx.doi.org/10.12659/AJCR.913678
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author Muneer, Mohammed
Badran, Saif
Al-Hetmi, Talal
author_facet Muneer, Mohammed
Badran, Saif
Al-Hetmi, Talal
author_sort Muneer, Mohammed
collection PubMed
description Patient: Female, 50 Final Diagnosis: Axial chordoma Symptoms: Back ache • numbness • urine incontinence • weaknes of lower limbs Medication: — Clinical Procedure: — Specialty: Plastic Surgery OBJECTIVE: Rare disease BACKGROUND: Chordoma is a primary bone tumor that most commonly arises in the sacrococcygeal vertebrae and the spheno-occipital areas. Chordoma is a malignant tumor that should be distinguished from benign notochordal cell tumor (BNCT) of the spine. This report is of a rare case of axial chordoma and describes the challenging approach to diagnosis and management. CASE REPORT: A 50-year-old woman presented with a one-year history of a slowly growing swelling in the sacral region. She complained of numbness and progressive weakness in both lower limbs, and urinary incontinence. Computed tomography (CT) imaging showed a large destructive lesion involving the sacrum and coccyx with cranial extension to level S2 and invasion of the right and left S2–S3 neural foramina, sacral nerves, left gluteus maximums muscle, and adjacent subcutaneous tissue. The management approach included the early involvement of a multidisciplinary clinical team. En bloc resection of the tumor through an anterior and posterior approach was performed, and the defect was reconstructed later using bilateral rotational gluteal fascial flaps. CONCLUSIONS: Axial chordoma is a very rare, locally aggressive, and highly recurrent primary tumor of bone. The clinical management is challenging and requires early involvement of a multidisciplinary team. Following surgical resection, careful selection from limited available reconstructive surgical options is necessary to ensure that the surgical defect is repaired.
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spelling pubmed-65611412019-06-26 A Rare Presentation of Axial Chordoma and the Approach to Management Muneer, Mohammed Badran, Saif Al-Hetmi, Talal Am J Case Rep Articles Patient: Female, 50 Final Diagnosis: Axial chordoma Symptoms: Back ache • numbness • urine incontinence • weaknes of lower limbs Medication: — Clinical Procedure: — Specialty: Plastic Surgery OBJECTIVE: Rare disease BACKGROUND: Chordoma is a primary bone tumor that most commonly arises in the sacrococcygeal vertebrae and the spheno-occipital areas. Chordoma is a malignant tumor that should be distinguished from benign notochordal cell tumor (BNCT) of the spine. This report is of a rare case of axial chordoma and describes the challenging approach to diagnosis and management. CASE REPORT: A 50-year-old woman presented with a one-year history of a slowly growing swelling in the sacral region. She complained of numbness and progressive weakness in both lower limbs, and urinary incontinence. Computed tomography (CT) imaging showed a large destructive lesion involving the sacrum and coccyx with cranial extension to level S2 and invasion of the right and left S2–S3 neural foramina, sacral nerves, left gluteus maximums muscle, and adjacent subcutaneous tissue. The management approach included the early involvement of a multidisciplinary clinical team. En bloc resection of the tumor through an anterior and posterior approach was performed, and the defect was reconstructed later using bilateral rotational gluteal fascial flaps. CONCLUSIONS: Axial chordoma is a very rare, locally aggressive, and highly recurrent primary tumor of bone. The clinical management is challenging and requires early involvement of a multidisciplinary team. Following surgical resection, careful selection from limited available reconstructive surgical options is necessary to ensure that the surgical defect is repaired. International Scientific Literature, Inc. 2019-06-01 /pmc/articles/PMC6561141/ /pubmed/31152144 http://dx.doi.org/10.12659/AJCR.913678 Text en © Am J Case Rep, 2019 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Articles
Muneer, Mohammed
Badran, Saif
Al-Hetmi, Talal
A Rare Presentation of Axial Chordoma and the Approach to Management
title A Rare Presentation of Axial Chordoma and the Approach to Management
title_full A Rare Presentation of Axial Chordoma and the Approach to Management
title_fullStr A Rare Presentation of Axial Chordoma and the Approach to Management
title_full_unstemmed A Rare Presentation of Axial Chordoma and the Approach to Management
title_short A Rare Presentation of Axial Chordoma and the Approach to Management
title_sort rare presentation of axial chordoma and the approach to management
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6561141/
https://www.ncbi.nlm.nih.gov/pubmed/31152144
http://dx.doi.org/10.12659/AJCR.913678
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