Endocrine Abstracts (2011) 26 P279

A patient with ACTH-dependent Cushing's syndrome and an unexpected neuropathogical diagnosis

I Kreitschmann-Andermahr, F J Hans, M Mull, W Karges & K Nolte

University Hospital RWTH Aachen, Aachen, Germany.

Ganglion cells in the sella turcica are rarely associated with endocrinological disease. We report the case of a 72-year-old woman who presented with typical signs and symptoms of hypercortisolism. Endocrinological work-up confirmed ACTH-dependent Cushing’s syndrome with loss of circadian cortisol rhythmicity and a pathological dexamethasone suppression test. MRI revealed right-sided sellar floor excavation but no clear-cut adenoma. Via a right-sided transnasal approach, after dissection with the microdissector, greyish, diffluent tissue with a softer consistency than normal pituitary tissue was removed. Histologically, the tissue was composed of pituitary epithelial cells admixed with bipolar ganglion cells in a glial background. The ganglion cells were weakly chromogranin-A positive, whereas the endocrine cells exhibited a stronger chromogranin-A positivity and ACTH positivity. The overall picture was characteristic for ‘pituitary adenoma with neuronal choristoma’ – PANCH- syndrome. Such tumours are very rare entities. Most patients exhibiting PANCH present with acromegaly and <10 cases associated with Cushing’s syndrome have been described in the literature. This implies that the epidemiological importance of these tumours is probably negligible. However, it cannot be excluded that they occur more frequently but are not diagnosed in the often times small amount of tissue sent in for neuropathological examination. Moreover, the entity gives rise to speculations about its tumorigenisis. Here, a transdifferentiation of adenohypophyseal cells into neuronal cells is discussed. Alternatively, a primary ganglion cell tumour could induce adenohypophyseal hypoplasia through hormonal stimulation. A third hypothesis involves the transformation into two cell types under the influence of a common tumorigenisis factor. Clinically, the patient described above exhibits no residual disease and is kept on clinical surveillance.

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