Endocrine Abstracts

Background: Chordomas are rare, invasive and slow growing bone tumours with different locations. Parasellar chordomas are even more rare and the initial manifestations include visual and/or neurological symptoms. In addition, endocrine dysfunction may be present. We describe 4 cases of parasellar chordomas.

Clinical cases: 1) Female, 55-years-old, reported headache associated with restriction of left eye movement for the past 3 months. Brain magnetic resonance imaging (MRI) revealed a solid destructive lesion in the left cavernous sinus hypointense on T1 and hyperintense on T2. The patient underwent a left frontotemporal craniotomy with subtotal tumour resection followed by conventional fractionated radiotherapy (RT). Surgical histology identified a conventional chordoma. Due to tumour recurrence, the patient was submitted to proton beam therapy. Over the 4-year follow-up period no endocrine dysfunction was documented. 2) Female, 49-years-old, complained of diplopia, right eyelid ptosis and restriction of right eye abduction. A brain MRI showed a lesion adjacent to the right cavernous sinus. A right pterional craniotomy was performed and its histology revealed a chondroid chordoma. Six years later, a centimetric tumour residuum in posterior fossa cisterns was identified and proton beam therapy was performed. Endocrine dysfunction was not identified during the entire follow-up. 3) Female, 42-years-old, reported facial paralysis and hypoesthesia for several months. A cerebral MRI identified a clival lesion hypointense in T1 and hyperintense in T2. A transsphenoidal surgery was performed and the surgical histology identified a conventional chordoma. Due to a tumoral residuum, this patient was also submitted to proton beam therapy. Her endocrine evaluation did not identify any deficit, during the 3 years follow-up period. 4) Male, 45-years-old, reported headaches, diplopia and left sixth nerve palsy was observed. The brain MRI showed a clivus lesion that was submitted to transsphenoidal surgery and radiosurgery. It was identified a conventional chordoma. Two years later, he started with third, fourth and fifth nerves paralysis and a left spheno-cavernous and orbital apex recurrence was identified on MRI. A near-total tumoral resection was done. After that, he initiated polyuria, polydipsia and severe tiredness. His endocrine evaluation revealed insipidus diabetes, central hypothyroidism and central hypocortisolism.

Conclusions: We describe 4 cases of rare tumours with a follow-up ranging from 3 to 6 years. Despite the proximity of sella turcica, pituitary deficits were rare and were associated with tumour growth and aggressive behaviour. Nevertheless, follow up of pituitary function is recommended.