Multiple paraganglioma: careful with surgery!

Extraadrenal paragangliomas are exceptional tumors. They prevail the carotid, jugulotympanic, and vagal ones. They are frequently multiple and its treatment is controversial in view of the fact that bilateral removal can go with severe morbidity. The case of a patient with bilateral paraganglioma and postoperative baroreflex dysfunction with severe arterial hypertension and hypotension episodes is presented. A 23-year-old woman with a diagnosis of left carotid and right vagal paraganglioma by TC and angiography. In January 1999 the left carotid paraganglioma was operated. The patient showed dysphonia, dysphagia, and lingual dysmotility in the postoperative course, with spontaneous improvement after some months. In December 1999, after the removal of the right vagal paraganglioma, the same complications appeared and hypertension crises (230/140), associated with headache, dizziness, and rash, and alternating with severe hypotension episodes (70/50). Blood biochemistry and the levels of cortisol, thyroid hormones, catecholamines, and metabolites were normal. Imaging techniques discarded tumor at another level and the registry of the ambulatory monitoring of blood pressure (AMBP) confirmed an important pressure lability. The neurophysiological study of the autonomous nervous system demonstrated the failure of the fast regulation mechanisms of the blood pressure. With the diagnosis of baroreceptors dysfunction and paralyses of cranial nerves IX, X and XII a treatment with clonidine was started with poor tolerability and incomplete response. This case illustrates the treatment difficulties of paragangliomas, especially when they are bilateral, and in which the surgery can go with severe morbidity. Baroreflex dysfunction should be entertained in the differential diagnosis of the extreme pressure lability.