Cervical Paraspinal Chordoma: A Literature Review with a Novel Case Report

Abstract

Chordomas are rare malignant neoplasms, accounting for 1–4% of all primary bone tumors. Most spinal chordomas occur in the sacrococcygeal region and the base of the skull; however, 6% of chordomas are observed in the cervical spine. In these cases, the lesion is mainly located in the midline. These tumors slowly grow before becoming symptomatic and encase the surrounding vascular and nerve structures. Patients with advanced chordoma have a poor prognosis due to local recurrence with infiltration and destruction of adjacent bone and tissues. Systemic chemotherapy options have not been fully effective in these tumors, especially for recurrent chordomas. Thus, new combinations of currently available targeted molecular and biological therapies with radiotherapy have been proposed as potential treatment modalities. Here, the present paper describes the case of a 41-year-old male with a C2–C4 chordoma located paravertebrally, who underwent surgical resection with a debulking procedure for a cervical chordoma. Computed tomography angiography revealed a paraspinal mass with bone remodeling and the MRI showed a paravertebral mass penetrating to the spinal canal with a widening of the intervertebral C2–C3 foramen. Initially, the tumor was diagnosed as schwannoma based on its localization and imaging features; however, the histopathology specimen confirmed the diagnosis of chordoma. This case study highlights the effectivity of radical surgical resection as a mainstay treatment for chordomas, discusses neuroimaging, diagnosis, and the use of currently available targeted therapies and forthcoming treatment strategies, as alternative treatment options for chordoma.

A case report of Sustained triumph: 15-year recurrence-free survival following surgical resection of a cervical chordoma in a 15-year-old girl

“…The most common sites of tumor development include the sacrococcygeal region (50–60%), sphenoid-occipital region (25–35%), and mobile spine (15%) 1 , 2 . Lesions of the cervical spine are rare, comprising between 3 and 7% of all chordomas and between 20 and 50% of spinal chordomas 4 , 8 .…”

“…Our case presentation demonstrates a unique presentation involving the mobile spine in a pediatric patient, which constitutes a minority of typical chordoma presentations 1 – 3 . In addition, the case demonstrated a lack of recurrence for a substantial period of time despite the hurdles faced with executing the optimal mode of treatment by en block excision of the tumor that is followed by radiotherapy 4 . Such cases are scarcely reported in the literature.…”

A rare case of large retropharyngeal pre-vertebral chordoma presented as oropharyngeal swelling

“…Chordoma is a rare type of tumor that accounts for only 1% of CNS tumors and is considered one of the main causes of midline tumors affecting the axial path. 4,5 It is a notochordal remnant and is more common in males over 40, with less than 1% of cases occurring in patients under 20. 6 Chordomas are most commonly found in the sacrococcygeal and spheno-occipital regions and the cervical region of the spine, accounting for about 6% of all cases, with a higher tendency in the upper cervical region.…”

“…Chordomas are most commonly found in the sacrococcygeal and sphenooccipital regions, with cervical region chordomas accounting for only 6% of all cases. 5 Cervical chordomas might present as retropharyngeal swelling. 4 The primary management of retropharyngeal swelling depends on the degree of airway compromise and patient stability.…”

Competing risk nomogram for predicting prognosis of patients with spinal and pelvic chordoma: A SEER-based retrospective study

“…Hence, the rates of local recurrence and distant metastasis after initial resection of spinal and pelvic chordoma are pretty high, with over 40% of patients occurring distant metastasis after treatment [8,10,11]. Furthermore, in the population over 40 years with the highest incidence of spinal and pelvic chordoma, the 10-year survival rate is only 46% [12,13]. Therefore, there is an urgent need to strengthen research on the prognosis of spinal and pelvic chordoma to guide individualized treatment and follow-up, thereby improving patient survival.…”