Ganglioneuroma in a patient with Turner syndrome

Ganglioneuroma is a benign neuroblastal tumor that derives from immature cells of the sympathetic nerve system. It is a very rare disease and affects newborns and infants more often than adolescents and adults. The benign tumors are relatively difficult to diagnose since they usually are asymptomatic. We present a case of unusual coincidence of an ganglioneuroma and Turner syndrome (TS).

An 11.5-year-old TS patient was admitted into the hospital with good general condition. She was 7 months into growth hormone (GH) therapy. The reason for the admission was to carry out control tests. The patient had not complains or abnormalities on physical examination. On the ultrasound examination of the abdomen in the right adrenal area an adrenal tumor was identified. The ultrasound examination of the abdomen performed 13 months earlier did not show any abnormalities. Computed tomography of adrenal glands confirmed right adrenal tumor. The hormonal function of the adrenal gland was normal. The treatment with GH was terminated and the patient was urgently referred to surgical management. The right adrenal gland tumor together with a part of diaphragm was removed. On histopathological examination ganglioneuroma maturum was diagnosed. On the control MIBG examination physiological gaining of tracer in the left adrenal and in the rest tissue in the area of the right adrenal was found. Trepanobiopsy did not confirm neoplastic changes.

There is a necessity of careful monitoring of TS patients also in face of the possibility of disclosure previously not stated neoplasm after starting GH therapy. It is impossible to rule out that GH therapy promote growth of existing neoplasm.