Hypopituitarism due to Chondrosarcoma: 8-year journey
H Yu Sanda, M Bashir, D Jugnarain, S Kotha, S Patel, D Lunda Ngandu, H Rehmani, M Saleem, G Ahmed, H Abera, S Elshowaya, G Mlawa, Queens Hospital, Romford Essex, UK
Introduction: Hypopituitarism is a chronic disease state that is caused by several aetiologies one of which is tumour compression of the pituitary gland. Hypopituitarism has a varied clinical manifestation and is often of gradual onset. Depending on the severity of hormone effect it can be associated with increased mortality and morbidity.
Chondrosarcomas are malignant mesenchymal tumours made up of cartilage producing cells. They are the second leading cause of bone malignancies and present with challenges in both diagnosis and therapy. Intracranial arising chondrosarcoma is rare and presents diagnostic dilemma as it may mimic radiological features of common lesions in this region such as non-functioning pituitary tumour, chordoma, craniopharyngioma, meningioma, or paraganglioma.
56-year-old man presented with episodes of retro-orbital headaches and blurred vision. Clinical examination revealed diplopia on right lateral gaze and pronator drift on the left side. Subsequent CT and MRI scans showed a right petrous apex lesion extending through the skull base with an approximate size of 3x4cm, with a large medial temporal component and an extension into right jugular foramina. The lesion encases the carotid artery on the right and is lying partly within the cavernous sinus causing right sided 6th nerve palsy and abuts the middle ear structures which caused the patient to have hearing impairment. The lesion in the right jugular foramina extending into the middle cranial fossa and Sella. The patient blood results revealed evidence of hypopituitarism: Cortisol was 38, FT4 6.4, FT3 3.4, TSH 1.85, testosterone less than 0.1, prolactin 1451, IGF1 124, and PSA less than 0.1. His current medications are Hydrocortisone 10mg BD, Levothyroxine 50mcg OD, Testosterone (tostran) 2 squirts and he has had proton beam radiotherapy for the lesion.
Discussion/conclusion: Chondrosarcoma is often a diagnostic challenge. As a result of its malignant nature, it can spread and cause new manifestations. Sellar chondrosarcoma is a rare manifestation of this malignancy that can present similarly to non-functioning pituitary tumour. In the present case the patient had chondrosarcoma that spread from right petrous apex to the Sella. Once it reached the Sella the patient developed severe manifestations of hypopituitarism which presented with abnormal thyroid function, low testosterone, and ACTH which in turn caused cortisol dysfunction.