Endocrine Abstracts

Adeno-gangliocytomas are rare tumours of the pituitary gland with less than 40 cases described worldwide. Due to the rarity of these tumours, treatment modalities largely follow that of conventional therapies for common pituitary lesions. Case reports on these tumours offer insight into their presentation and the effectiveness of treatment which helps guide future management. A 64-year-old man was admitted for stone fragmentation and ureteric stent insertion. During anaesthetic recovery he developed sudden onset severe frontal headache with nausea, photophobia and visual field disturbance. Urgent cranial CT scanning showed pituitary apoplexy. Subsequent pituitary MRI scan confirmed a pituitary macroadenoma with evidence of tumoral haemorrhage. On examination he had classical clinical features of acromegaly including prognathism, interdental separation, large hands and macroglossia. Investigations showed raised GH (5.32 mcg/l) and IGF-1 (103.8 nmol/l) with non-suppression of GH levels during an OGTT. Thyroid and gonadal axes were normal. Pituitary apoplexy was managed with Dexamethasone in the acute phase. Endoscopic trans-sphenoidal pituitary surgery was undertaken electively. At operation a hard fibrous tumour was noted and decompression of the macroadenoma was undertaken. Resection was incomplete due to the fibrous nature of the tumour and risk to surrounding structures. Histology of the excised tumour showed a composite lesion of neoplastic cells expressing GH and ganglion cells, confirming the diagnosis of a GH secreting adenoma-gangliocytoma. Post-operative assessment showed evidence of residual GH hypersecretion. Adjuvant treatment with pituitary radiotherapy is being planned along with somatostatin analogue therapy in the interim. The histogenesis of mixed pituitary adenoma-gangliocytomas is unclear. These tumors are difficult to distinguish from pituitary adenomas on neuroimaging. Treatment is along conventional lines with pituitary surgery followed by radiotherapy and somatostatin analogue therapy. It is unclear as to whether the clinical course of these tumors is different to conventional GH secreting pituitary adenomas.