SUN-920 Familial Paraganglioma Syndrome: A Rare Case of Secondary Hypertension in Young People

Paragangliomas are rare tumors originating in the autonomic nervous system, whose clinical manifestations are the result of excessive production of catecholamines. We present a case of a 26-year-old female with 5 years of disease characterized by episodic profuse sweating, headaches and high blood pressure refractory to antihypertensive treatment. She also had intermittent palpitations which intensified 1 month before admission. Patient was cataloged with diagnosis of endocrine hypertension. She had elevated urinary fractionated metanephrines, elevated plasma normethanephrin and plasma chromogranin A (CgA). Subsequently, an abdominal CT study was performed, finding a solid ovarian mass of defined edges located in retroperitoneal space, an intercave region immediately preceding the L2-L3 intervertebral disc that measured 26.2 x 23.9 x 28.8 mm. It was also found bilateral tumours at cervical level of 14 mm in right side and 10 mm in left side, suggestive of paranganglioma by magnetic resonance imaging (MRI). With a suspected diagnosis of paraganglioma of Zuckerkandl’s organ, beta and alpha-adrenergic blockage were carried out and surgical intervention was done by a block resection of the tumour. Anatomopathological diagnosis confirmed the suspicion of well-delimited capsulated paraganglioma of 2.0 X 1.0 X 0.3 cm, with low mitotic index (<2) and a result of positive sinaptophisin by immunohistochemistry. She reached complete remission and normal determinations in urine of catecholamines and methanephrines. Currently the patient is in follow-up with favorable evolution and succinate dehydrogenase type B (SDHB) gene test is pending. Despite infrequency of paragangliomas, it is important to take them into account in the differential diagnosis of hypertension, especially in young patients, due to their malignant potential and the effects of catecholamine secretion on the cardiovascular system.