Endocrine Abstracts

Chordomas of the skull base are rare lesions, which account for less than 0.2% of all intracranial tumours. They arise from embryonic remnants of the primitive notochord. They are described as locally aggressive, rarely metastatic tumours with a tendency to frequent relapses. They usually grow slowly with the destruction of the surrounding tissues. The symptoms are very unspecific which is the cause of the delay of the diagnosis. The most common initial symptoms are headaches and neuro-ophthalmological dysfunctions. Involvement of the suprasellar region may lead to partial or complete destruction of the pituitary gland, causing hypopituitarism and diabetes insipidus. Literature data indicates that the treatment of chordomas should be a combination of maximal surgical resection and adjuvant radiotherapy. A 60-year-old man reported impaired movements of the left eye with ptosis and secondary diplopia over two weeks before the admission to the neurology ward. He also suffered from nasal blockage with bloody discharge on the left side. Physical examination revealed a palsy of the 3rd left cranial nerve. He was transferred to our endocrinology department with clinical suggestion of pituitary apoplexy. In the meantime, brain computed tomography and magnetic resonance imaging were performed showing a large mass (78x54x55mm) in the sphenoid bone and clivus, extending to the sella, elevating upward the pituitary and optic chiasm, infiltrating into the nasopharynx, nasal cavities, bilateral cavernous sinuses, surrounding both internal carotid arteries. There was a suspicion of bleeding into the mass and thrombosis of the superior sagittal sinus and confluence of sinuses. The endocrine assessment revealed hypopituitarism with adrenal and thyroid deficiency with mild hyperprolactinemia. The patient underwent transnasal biopsy of the mass. Histopathology examination revealed a chordoma. The patient underwent cytoreductive endoscopic surgery, awaiting for the proton-beam therapy. The presenting case visualizes that the symptoms typical of pituitary adenomas also apply to the rare non-adenomatous sellar region lesions. It emphasizes the need for a multidisciplinary approach, with apart from endocrinologist and neurosurgeon, dedicated neuroradiologist, otorhinolaryngologist and radiation oncologist and confirms the need for pituitary centres of excellence.