Management of Clival Chordomas in the Pediatric Population: Report of Two Cases

Introduction: Clival chordoma represents a challenging tumor in both the adult and pediatric patient populations. These rare tumors in children tend to have local invasion of the skull base. Maximum safe resection remains the primary treatment of choice and, depending on the extent of tumor invasion, the need for occipital-cervical stabilization may be required. Local recurrence can be high with a need for adjuvant treatment, such as proton beam irradiation.

Case Description: Case 1:A 10-year-old otherwise healthy female patient presented with a 2-year history of neck pain and occipital headaches that increased to waking her up at night. She had no neurological deficits on examination. MRI revealed a mass lesion of clivus with ventral brain stem compression extending from the sphenoid sinus to the dens. It was moderately heterogeneously enhancing with hyperintensity on T2 sequence consistent with clival chordoma. She underwent a staged occipital to C2 fusion followed by an endoscopic endonasal resection with planned adjuvant radiotherapy.

Case 2:A 7-year-old otherwise healthy female patient presented with a diplopia and abnormal eye movement. On examination, she had a right sixth nerve palsy and dysmetria. MRI revealed a mass lesion centered on the clivus with significant osseous destruction extending from the sphenoid sinus to the dens. It had heterogeneous enhancement with mixed foci of T2 hyperintensity with mostly T2 hypointensity. Given the appearance of this clival lesion, an endoscopic endonasal biopsy was performed consistent with an atypical chordoma. She underwent an endoscopic endonasal resection followed by a retrosigmoid craniotomy for resection of extensive clival chordoma invasion. Her course was complicated by CSF leak and radiation and tumor induced basilar artery vasculitis. Tumor and basilar artery vasculitis were both progressive and, despite adjuvant chemotherapy, she succumbed to this aggressive disease 1.5 years from diagnosis.

Discussion: Pediatric clival chordoma remains an extremely rare and difficult entity to treat. Endoscopic endonasal approach for maximum safe resection appears to be a useful approach that may need to be combined with other skull base approaches. Skull base reconstruction after endonasal surgery or craniotomy is important and may use a variety of techniques including vascularized flaps and free tissue grafts. The need for adjunct treatment must be considered. Special consideration must be addressed when dealing with potential longevity in the pediatric population. A team-based multispecialty approach may provide the best care for these aggressive tumors.