Chondroid chordoma of the base of the skull: Orbital and other neuro-ophthalmological symptoms

Abstract

Key words: chordoma of the base of the skull, chondroid chordoma, orbital ap_ex syndrome due to chordoma, ophthalmoplegia dolorosa due to chordoma Abstract. Chondroid chordoma of the base of the skull has a significantly more favourable prognosis than the classical chordoma. The clinical and radiological symptoms of this relatively benign form, and its histological and therapeutic aspects, are discussed in connection with the case of a 50-year-old patient.

Features and management of strabismus from skull base chordoma

“…2 These soft, pale-grey tumours produce variable symptoms depending on their location and direction of growth. 5 Because of the proximity of clival chordomas to the brainstem, the slow growth of these tumours, and their potential for local invasion, these tumours frequently cause cranial nerve palsies 6 Clival chordomas, which account for just over a third of chordomas, cause three groups of symptoms characteristically accompanied by headache: symptoms owing to involvement of the cerebellopontine angle, homonymous hemianopia or other parasellarassociated symptoms, and symptoms suggestive of brainstem compression. 7 Neuro-ophthalmic symptoms are common, particularly paralysis of extraocular muscles, but also may include visual field disorders, and less commonly, patients can present with orbital symptoms such as exophthalmos.…”

“…7 Neuro-ophthalmic symptoms are common, particularly paralysis of extraocular muscles, but also may include visual field disorders, and less commonly, patients can present with orbital symptoms such as exophthalmos. 5 The treatment of these tumours, in particular clival tumours, is challenging and involves a combination of surgery and adjuvant radiation therapy. 2 The variability in presentations and the timing of the associated strabismus present a challenge in their surgical management from an ophthalmologic perspective.…”

“…Skull base chordomas most often present with strabismus owing to paralysis of cranial nerve VI. 5 Rarely, chordomas of the clivus can present with rhinorrhea owing to cerebrospinal fluid leak. 1 Survival ranges from 50%À68% at 5 years to 28%À40% at 10 years.…”

“…X-ray can be useful in diagnosis when the tumour is advanced and demonstrates bony destruction with a soft tissue mass, but computed tomography (CT) and MRI are more useful imaging modalities. 5,11 CT typically demonstrates a locally destructive soft tissue mass and lytic lesions and can outline the extension of the tumour as well as identify abnormalities in the ventricular system from compression by the tumour. 5 On MRI, the lesions appear hypodense on T1 flair images with enhancement with gadolinium, whereas they appear hyperdense on T2-weighted images.…”

“…5,11 CT typically demonstrates a locally destructive soft tissue mass and lytic lesions and can outline the extension of the tumour as well as identify abnormalities in the ventricular system from compression by the tumour. 5 On MRI, the lesions appear hypodense on T1 flair images with enhancement with gadolinium, whereas they appear hyperdense on T2-weighted images. 11 Treatment that confers the best chance of survival is en bloc resection of the tumour with margins, but this is not always possible because of the location of these tumours and the resulting reconstruction that would be necessary following tumour excision.…”