Abstract
BACKGROUND: Tenosynovial giant cell tumor (TGCT) occurs most commonly in the appendicular skeleton and is only rarely found in the vertebral column. Lesions of the craniocervical junction are particularly rare, with only 4 cases reported in the literature. The authors describe the case of a diffuse-type TGCT at the craniocervical junction. OBSERVATIONS: A patient presented with a 1-year history of right-sided neck pain and bilateral neurological symptoms in the distribution of the right occipital nerve. A 20-mm homogeneously contrast-enhancing mass in the suboccipital and posterior C1 region was discovered on magnetic resonance imaging of the cervical spine. The tumor was operated on via a posterior approach, and gross-total resection (GTR) was achieved. Immunohistochemical (IHC) examination revealed a diffuse-type TGCT. The patient had an uneventful recovery. LESSONS: TGCT can arise at the craniocervical junction and is easily misdiagnosed because of its rare occurrence. IHC examination of a tumor specimen should be done to confirm the diagnosis. GTR is the objective when treating these tumors, especially when they are the diffuse type, as they have a high recurrence rate. Radiation and small-molecule therapies are viable postoperative therapies if GTR cannot be achieved or in cases of recurrence.