Rare case of extracranial chordoid meningioma adjacent to the carotid sheath: illustrative case

BACKGROUND: Chordoid meningioma is a rare World Health Organization (WHO) grade 2 variant of meningioma with histological features resembling those of a chordoma. This tumor type is known for having an aggressive clinical course with a propensity for local recurrence. Most cases occur within the cranium, more specifically around the cerebral convexities. Although extracranial meningiomas of various subtypes have been documented, extracranial meningioma with a chordoid subtype is an extremely rare entity. OBSERVATIONS: The authors herein report the case of a 51-year-old female who presented with a chief complaint of dysphagia and was found to have a neck mass abutting the carotid sheath. The patient underwent resection and final pathology results revealed a WHO grade 2 chordoid meningioma. LESSONS: This case report demonstrates an atypical case of an extracranial chordoid meningioma adjacent to the carotid sheath. To the authors’ knowledge, this is the first reported case of a chordoid meningioma occurring within the soft tissue of the neck.