Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2023) 90 P710 | DOI: 10.1530/endoabs.90.P710

ECE2023 Poster Presentations Pituitary and Neuroendocrinology (123 abstracts)

Chordomas of the parasellar region: description of 4 cases of a rare disease

Ines Cosme , Ema Nobre & Maria João Bugalho


Centro Hospitalar Universitário Lisboa Norte, Endocrinology, Lisbon, Portugal


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.

Volume 90

25th European Congress of Endocrinology

Istanbul, Turkey
13 May 2023 - 16 May 2023

European Society of Endocrinology 

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