MRI of the cervical spine immediately post op and at 6 months excluded any residual or recurrence. There were no complications.įollow-up of the patient at 6 months showed complete resolution of symptoms. Part of the apical and alar ligaments were removed due to infiltration by the tumor. Drilling out of the right side of the C1 arch provided adequate exposure for the tumour to be excised en bloc. The tumour was found in the interspace between the rim and upper border of the C1 and dens, with extension along the space lateral to the tumour. The previous incision was opened and extended down to expose the C1 arch and the anterior rim of the foramen magnum.
After stereotaxis was used to identify the C1 arch, the soft palate was divided along the midline and to the left of the uvula. Using a Crockard transoral retractor system, the posterior pharyngeal wall and previous surgical incision were visualized. This was performed via a stereotactic transoral wide local excision. Given the histological diagnosis, the patient underwent a gross total resection of the lesion. There was mild nuclear pleomorphism of these mononuclear cells. The specimen was of fibrotendinous connective tissue, with proliferation of mononuclear epithelioid to spindled cells within the fibrotic stroma. Histology of the biopsy demonstrated a low grade fibrohistiocytic tumour and favoured the diagnosis of tenosynovial giant cell tumour. It was hard, fibrous and had ill-defined margins. The lesion was found in the space between the basion and the C1 arch and biopsied. After an inferiorly based flap was made in the nasopharynx, stereotaxis was used to identify the basion and the upper border of the C1 arch. Given its location, the lesion was biopsied surgically via a stereotactic endoscopic endonasal approach with otorhinolaryngologist support. We hope that this paper can add to the current literature to improve understanding, diagnosis, and management of future cases of TSGCTs. Previous tumours with a similar anterior upper cervical location were deemed inoperable and were either managed with radiological surveillance or immunotherapy. Our first case (C1/2) describes a unique tumour location as well as a new endoscopic endonasal tissue biopsy method and a new transoral surgical approach which allowed for a successful gross total resection. This case report includes two patients with TSGCSTs in the cervical spine: one with a lesion between C1/2 and another at C6/7. 9 of the 13 previous upper cervical cases underwent gross total resection, while 3 were managed conservatively with serial MRI scans and 1 underwent immunotherapy. However, in recurrent or inoperable cases, immunotherapy, radiation therapy and radiological surveillance are used. Gross total resection is recommended for all TSGCTs due to their locally aggressive nature and the subsequent risk of joint instability from bone erosion and of neurological deficit from spinal cord compression. Lesions of the upper cervical spine (C1/2) are extremely rare, with only 13 cases reported in the literature. They are rarely found in the spine but, when present, are generally found in the lower cervical and lumbar regions. They generally occur in large load bearing joints such as the hips and knees. Tenosynovial giant cell tumours (TSGCTs) are benign but locally aggressive primary fibrohistiocytic tumours that usually arise from the synovial membranes of tendon sheaths, bursa and joints.