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A Rare Presentation of Axial Chordoma and the Approach to Management

Challenging differential diagnosis, Rare disease

Mohammed Muneer, Saif Badran, Talal Al-Hetmi

(Department of Plastic Surgery, Hamad Medical Corporation, Doha, Qatar)

Am J Case Rep 2019; 20:773-775

DOI: 10.12659/AJCR.913678

Published: 2019-06-01


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.

Keywords: chordoma, Medicine, Surgical Flaps



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