Primary extranotochordal cervical chordoma masquerading as a thyroid neoplasm: a case report

Chordoma is a rare malignant neoplasm of notochord origin that occurs along the craniospinal axis, and extranotochordal chordoma is extremely unusual. An aberrant origin of this neoplasm can pose diagnostic pitfalls. We report a case of primary cervical chordoma of extranotochordal origin clinically presenting as a thyroid neoplasm. The cytomorphologic features of chordoma and its cytologic differential diagnoses are discussed.

An 82-year-old man presented with hoarseness of voice and difficulty in swallowing. He had a mass in the anterolateral aspect of the right side of the neck. Magnetic resonance imaging of the neck with contrast study revealed an ill-defined, contrast-enhancing tumor mass located in the right retrolaryngeal and parapharyngeal space at the level of C4, invading the perithyroidal soft tissue. Aspiration biopsy cytology of the tumor demonstrated epithelial-like cells with varied cytomorphologic features causing differential diagnostic problems with thyroid and soft tissue. However, the diagnosis of extranotochordal chordoma was established by the subsequent histologic examination of the excisional biopsy specimen.

Since chordoma is capable of exhibiting a spectrum of cytomorphologic characteristics and can have cytologic features overlapping with other malignant neoplasms, a high index of suspicion is crucial to make an accurate diagnosis on fine needle aspiration biopsy. This case highlights the occurrence of a rare neoplasm at an aberrant site and the diagnostic challenges that can pose. However, awareness of the abnormal locations and characteristic cytomorphologic features of chordoma together with radiologic findings will usually keep the cytopathologist from making a wrong diagnosis.