A unique presentation of retroclival chordoma.DR Warakaulle, P Anslow
Department of Neuroradiology, Radcliffe Infirmary, Woodstock Road, Oxford OX2 6HE, UK.
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 12571385
Source of Support: None, Conflict of Interest: None
Chordomas are rare tumours which arise from remnants of the primitive notochord. They occur primarily in the sacrum, clivus and cervical regions. We report a case of retroclival chordoma which presented as an extradural haemorrhage following minor trauma. The underlying tumour was not apparent on imaging performed immediately following the event, and chordoma presenting in this manner has not previously been described in the literature. The tumour became apparent on subsequent imaging, and progressed despite surgical debulking and radiotherapy.
Keywords: Adult, Brain Neoplasms, diagnosis,therapy,Case Report, Chordoma, diagnosis,therapy,Disease Progression, Human, Male,
Retroclival chordomas are rare tumours, accounting for approximately 1% of all intracranial tumours. They usually present with cranial nerve palsies, visual disturbances and orbito-frontal headaches. They rarely have intracerebral haemorrhage as the presenting feature. We report on a patient with retroclival chordoma, whose first presentation was with an extradural haemorrhage following minor trauma. This clinical and radiological presentation of retroclival chordoma has not previously been reported.
A 29-year-old man presented with an eight day history of headache after a low-speed road traffic accident, where he was shunted from behind whilst driving his car at approximately 30mph. On examination, his Glasgow Coma score was 15/15, and he had bilateral cranial nerve VI palsies and an absent gag reflex.
A CT scan [Figure - 1] showed an extradural haematoma compressing and distorting the brainstem. Subsequent MRI [Figure - 2] confirmed the presence of a subacute extradural haematoma anterior to the brainstem. No source of bleeding was identified.
The patientís cranial nerve palsies progressed, and he developed diplopia. He then underwent diagnostic cerebral angiography. This study proved difficult to interpret due to the mass effect from the haematoma, but no definite vascular malformation was identified.
The patient then underwent repeat MRI scanning one month after the initial presentation. This scan identified a mass lesion at the upper aspect of the subacute extradural haematoma [Figure - 3]. The mass was well-defined, slightly lobulated and showed pathological enhancement following the administration of gadolinium-DTPA. He then went on to have a craniotomy for evacuation of the haematoma with biopsy and debulking of the tumour mass. The histology [Figure - 4] showed tumour cells in sheets and cords within a mucoid background. The cells were uniformly cuboidal with some large physalliphorous cells with foamy cytoplasm. The findings were consistent with a diagnosis of chordoma.
His symptoms and neurological deficit improved following surgery, and he went on to have a course of proton beam radiotherapy. However, a follow-up MRI scan [Figure - 5] showed progression of the lesion despite treatment.
Chordomas are malignant neoplasms derived from embryonic notochordal remnants. While the majority of chordormas occur in the sacral region, more than one third arise in the skull base. As the terminus of the notochord is in the sphenoid bone inferior to the sella turcica, skull base chordomas arise adjacent to the clivus. These tumours usually produce marked destruction of the sphenoid bone and extend into the sphenoid sinus and nasopharynx.
Cranial nerve palsies due to tumour extension into the neural foramina is the most frequent presentation of clivus chordoma. There may be associated headache, signs and symptoms of raised intracranial pressure and pyramidal signs. Acute presentation following intraparenchymal haemorrhage has also been described.
The CT appearance of a clivus chordoma is usually a midline lesion with bone destruction and a soft-tissue mass. The bone adjacent to the tumour does not have a sclerotic margin, and large calcific fragments are frequently seen. There is a variable appearance on MRI, with a hypointense to isointense soft-tissue mass seen on T1-weighted images and high signal on T2-weighted scans. Cystic areas containing haemorrhage are frequently present, and produce high signal on T1-weighted images.,
Proton beam radiotherapy following surgical debulking is the preferred treatment of these tumours, which are rarely amenable to complete resection. This combined treatment can achieve 3-year survival rates of upto 91%.
Chordomas are frequently haemorrhagic, and can rarely present as a primary intracerebral haemorrhage. However, the underlying tumour has been demonstrated on imaging at presentation in all previously reported cases. Chordoma presenting as an extradural haemorrhage following trauma and remaining occult on initial imaging has not previously been reported. This case demonstrates the importance of seeking an underlying cause for an extradural haematoma which occurs at an unusual site following relatively minor trauma.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]