Acromegaly due to a mixed growth hormone secreting adenoma-gangliocytoma - a rare cause of GH excess

Abstract

Adeno-gangliocytomas are a rare form of pituitary tumour, with less than 40 cases being described in the literature worldwide. 1 They are mixed lesions consisting of adenocytic and gangliocytic components. 2,3 These tumours have in most cases been found in patients with features of acromegaly, although less frequently can present with cushingnoid features or hyperprolactinemia. 3 Uncomplicated pituitary adenomas and neural gangliocytomas tend to exhibit a high degree of cellular differentiation and low proliferative potential. As such they are often slow benign tumours that may be resolved with complete surgical resection. 2 The clinical course of mixed Adeno-gangliocytomas are however not well characterised. The histogenesis of Adeno-gangliocytoms also remains unclear although the histological specimens show histogenetic distinction between the two components. 2,4 Reports on these rare tumours can provide insight in to the effectiveness of current treatment modalities. Conventional macro pituitary adenomas and neural gangliocytomas tend to respond well to surgery and may not need adjuvant therapy post surgery. The risk of recurrence in mixed Adeno-gangliocytomas is not well characterised. Although initial tumour reduction seems effective, further research is needed to determine the role of adjuvant medical therapies in this group of patients if complete surgical resection is achieved.