Primary intracranial Parachordoma: An unusual tumor in brain

BACKGROUND: Parachordomas are rare soft tissue tumors commonly occurring in limbs, chest, Abdomen, and back. The World Health Organization (WHO) classification includes parachordomas in the same group as mixed tumors and myoepitheliomas. Exact histogenesis of this tumor is unclear. CASE DESCRIPTION: A 52-year-old male presented with headache and blurring of vision since one month. Preoperative computed tomography (CT) scan of brain revealed left parieto-occipital tumor extending up to the trigone. Total excision of the tumor was done. Histopathologically, the tumor was composed of relatively uniform cells with eosinophilic cytoplasm in a myxoid stroma and with cartilaginous and osseous metaplasia. The tumoral cells were immunoreactive for cytokeratin, epithelial membrane antigen (EMA), S-100, and vimentin. The constellation of findings revealed the tumor to be parachordoma. Magnetic resonance imaging (MRI) brain during follow-up at one year showed no recurrent tumor. No adjuvant therapy was given to this patient. CONCLUSION: This is the first reported case of primary intracranial parachordoma. It is difficult to diagnose the lesion preoperatively by imaging alone. Long-term follow-up is necessary in view of few reports in literature of recurrence and metastasis, of parachordomas in other anatomical locations.