Giant Cell Ependymoma of Cervicomedullary Junction: A Case Report of a Long-Term Survivor and Literature Review.

Background: Ependymoma accounts for 3%-9% of all neuroepithelial tumors. Giant cell ependymoma (GCE) is a rare and distinct variant, with only 22 cases described in the literature. The 2007 World Health Organization classification first acknowledged this rare subtype. The cytologic features of GCE include the presence of pleomorphic giant cells with several cellular atypias, which at intraoperative frozen diagnosis may appear to be high-grade glial lesions. Despite its apparently malignant histology, GCE seems to be a neoplasm with a relatively good prognosis. Extended tumor removal is the gold standard without adjuvant treatment.

Case Description: We describe the first case, to our knowledge, of GCE situated at the cervicomedullary junction in a 62-year-old patient. Surgery was performed with combined intraoperative monitoring of motor evoked potentials and somatosensory evoked potentials. Intraoperative frozen diagnosis revealed a high-grade glial neoplasm; however, gross total resection was achieved. The definitive diagnosis was GCE. At follow-up evaluation 11 years after surgery, the patient did not present with any tumor recurrence.

Conclusions: As the intraoperative diagnosis can be misleading, whenever a cleavage plane is recognized, it is essential to perform a gross total resection with the aid of intraoperative neurophysiologic monitoring, to improve prognosis and neurologic outcome. Data reported in the literature show that prognosis is mainly influenced by grade of resection.