NYMC Faculty Publications
Intrasellar Hemorrhagic Chordoma Masquerading as Pituitary Apoplexy: Case Report and Review of the Literature
Author Type(s)
Resident/Fellow, Faculty
DOI
10.1080/02688697.2021.1941761
Journal Title
British Journal of Neurosurgery
First Page
1685
Last Page
1688
Document Type
Article
Publication Date
12-1-2023
Department
Neurosurgery
Second Department
Pathology, Microbiology and Immunology
Third Department
Neurology
Abstract
BACKGROUND AND IMPORTANCE: Chordomas are centrally located, expansile soft tissue neoplasms that arise from the remnants of the embryological notochord. Hemorrhagic presentation is exceedingly rare and can resemble pituitary apoplexy. Moreover, a purely intrasellar location of a chordoma is extremely uncommon. We report a case of a hemorrhagic intrasellar chordoma in an adult male, which presented similarly to pituitary apoplexy and was resolved with surgical resection. CLINICAL PRESENTATION: A 69-year-old male presented with a 4 week history of acute onset headache and concurrent diplopia, with significantly reduced testosterone and slightly reduced cortisol. His left eye demonstrated a sixth cranial nerve palsy. Magnetic resonance imaging of the brain showed a large hemorrhagic mass in the pituitary region with significant compression of the left cavernous sinus and superior displacement of the pituitary gland. The patient underwent an endoscopic endonasal transsphenoidal approach for the resection of the lesion. Near total resection was achieved. Final pathology revealed chordoma with evidence of intratumoral hemorrhage, further confirmed by immunopositive stain for brachyury. Post-operatively, the patient had improved diplopia and was discharged home on low dose hydrocortisone. At 3-month follow-up, his diplopia was resolved and new MRI showed stable small residual disease. CONCLUSIONS: Apoplectic chordomas are uncommon given chordoma's characteristic lack of intralesional vascularity and represent a diagnostic challenge in the sellar region. Our unique case demonstrates that despite our initial impression of pituitary apoplexy, this was ultimately a case of apoplectic chordoma that responded well to endoscopic endonasal surgery.
Recommended Citation
Li, B., Kim, M. G., Dominguez, J. F., Feldstein, E., Kleinman, G., Al-Mufti, F., Kim, M., & Hanft, S. (2023). Intrasellar Hemorrhagic Chordoma Masquerading as Pituitary Apoplexy: Case Report and Review of the Literature. British Journal of Neurosurgery, 37 (6), 1685-1688. https://doi.org/10.1080/02688697.2021.1941761