Primary intracranial Parachordoma: An unusual tumor in brain

Abstract

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.

Parachordoma: a rare recurring case at a rare site

“…2 Parachordomas have been reported to occur in the pelvis and gastric serosa also. 1 Present case occurred in 65 year old male in the neck with history of recurrence twice.…”

“…Laskowski first described this entity as extra-axial chordoma in 1995, with a series of 5 cases. 1 It is attributed to be ectopic rests of notochord, Schwann cells, myoepithelial cells or specialized synovial cells with no convincing evidence one way or another. 2 Parachordomas are usually seen in adults but the 101case series has reported an age range of 3 to 83 with a mean age of 39.4 The lesion is reported to be seen more commonly in males.…”

“…The differential diagnosis for this tumor includes extraskeletal myxoid chondrosarcoma, chordoid meningioma, and chordoma. 1 Immunohistochemistry is essential to differentiate this tumor from extraskeletal myxoid chondrosarcoma and chordoma. In the former, the cells are usually smaller and they do not stain with cytokeratin (CK).…”

“…A small number of these tumors develop local recurrences and are rarely seen to metastasize. 1 We report a case of recurrent parachordoma in the neck with duration of 20 years.…”

Mioepitelioma de la región selar simulando macroadenoma hipofisiario: reporte de primer caso en Colombia

“…Estas neoplasias afectan por igual tanto a hombres como mujeres sin ningún tipo de predilección por edad o ninguna condición específicoa (12). Se han reportado casos en pacientes con edades que oscilan entre los 10 meses y los 48 años de edad, cuyas localizaciones a nivel intracraneal incluyen el cavum de Meckel (5), los hemisferios cerebrales (13,14,15), el seno cavernoso (6), la región de la hoz del cerebro (7,16), la órbita, la duramadre de la fosa craneal media (17), y el ángulo pontocerebeloso (18).…”