Intrathoracic Pheochromocytoma with Signs of Cortical Hyperfunction

A case of intrathoracic pheochromocytoma in a child 3 years old with hirsutism, physical and mental underdevelopment, increased urinary corticoids output and some others unusual clinical features is described. The case is particularly interesting not only for the rarety of the localization, but for the even more rare association between medullary and cortical hyperfunction. The latter has been described in few cases, but never in an extradrenal pheochromocytoma. The patient has been diagnosed preoperatively, operated upon successfully and followed up for about 2 years; particular attention has been put in the ECG which preoperatively showed signs of marked left ventricular hypertrophy and postoperatively an increasing of the signs, which disappeared only after several months. Likewise the catecolamines output which was very high at the beginning has remained over normal even several months after removal of the tumor. Conversely disappearance of the physical signs and improuvement in the general conditions has been dramatic soon after the operation. In regard of the pathogenesis of the syndrome, the authors describe the two main interpretations: a) stimulation of the cortical by the hyperadrenalinaemia through the hypophysis and b) association of cortical and medullary hyperfunction in the same tumor. They think the latter one is the more probable.