Chondroid chordoma - Report of a case with unusual localisation

Chondroid chordoma is a rare variant of chordoma, a rare slow-growing tumour of notochordal cell rests. Unlike classic chordoma, arising predominantly in the sacrococcyeal region, chondroid chordoma is localised in, the skull base, mainly in the sphenoido-occipital region, in younger individuals and has a better prognosis than classic chordoma. This variant, long deemed a low grade chondrosarcoma, was distinguished as a separate entity with the advent of immunohistochemistry. We describe a patient presenting with a chondroid chordoma in very rare localisation arising in the jugular foramen and extending extracranially into the parapharyngeal region. The tumour was resected and analysed histologically. Neuroradiological investigation (CT and MR) disclosed a lesion with an inhomogeneous structure located in the right lateral portion of the posterior fossa compressing the cerebellar tissue and extending into and eroding the jugular foramen. Through the jugular foramen the mass extended towards the laterocervical region to occupy the carotid compartment. The unusual tumour location allowed surgical resection in one sitting using a combined lateral trans-jugular and laterocervical approach. From an anatomopathological standpoint, our case has clear-cut features of chondroid differentiation including foci of calcification and enchondral ossification. For this reason, the tumour cannot be distinguished from low grade chondrosarcoma morphologically, but requires immunohistochemical techniques which disclose a codified Pattern of epithelial markers like cytokeratin, EMA and CEA in chordoid tumours.