Extracranial presentation of craniocervical chordoma.

Abstract

Chordomas are rare tumors of notochordal origin which arise in the sacral, vertebral, and craniocervical areas. Most patients with craniocervical chordomas have either neurological or ophthalmological symptoms. A patient presented with a parotid mass which was initially diagnosed as benign mixed tumor. One and one-half years later, this patient was found to have an extensive intracranial tumor which was diagnosed as chordoma. Treatment consisted of subtotal surgical excision and radiotherapy. This case demonstrates an unusual presentation of an unusual tumor and the difficulty that may arise in in diagnosing these tumors.

Chondroid Chordoma of the Lateral Skull Base

“…In the case of ventral intradural chordomas, the neoplasm may originate from an ecchordosis physaliphora [16][17][18][19][20][21][22][23][24][25][26][27][28][29]. The primarily extradural location of the tumor in this case diminishes the likelihood that it was derived from an ecchordosis physaliphora.…”

Bone Lesions

“…[283][284][285] By the fifth week of embryonic development, the notochord becomes encased by the anlage of the bones of the skull and vertebral column and gradually disappears, although microscopic vestiges of this notochord persist and are found in 0.5% to 2% of autopsies. 287,288 Clinical Features. 285 In the adult, the nucleus pulposus of the intervertebral disc represents the remnant of the primitive notochord; however, chordomas arise not from the nucleus pulposus but from the vestigial remnants because the anatomic location of these tumors roughly parallels the site frequency of occurrence of these remnants along the craniospinal axis.…”

A rare case of surgical pathway implantation of clival chordoma presenting as a neck mass

“…(1) Chordomas originate in the sacral (29.2%), cranial (32%) and spinal (32.8%) regions. (2) Clival chordomas represent approximately half of cranial chordomas and they are more likely to occur in women and younger patients.…”