P001 AN UNCOMMON CAUSE OF HIGH BLOOD PRESSURE IN YOUNG BASKETBALL PLAYER

Paragangliomas are rare neuroendocrine tumors that affect approximately 1.5–9 patients per million. They originate from chromaffin cells of the adrenal medulla or sympathetic and parasympathetic ganglia. Paragangliomas account for 10%–20% of retroperitoneal tumors and can manifest at any age. These tumors can secrete catecholamines, leading to symptoms such as hypertension, headaches, and diaphoresis.However, palpitations or tachycardia may also be present. Definitive treatment entails surgical resection, with acceptable rates of morbidity and mortality, necessitating careful patient preparation and intraoperative management. Case Presentation: We present the case of a 29-year-old man with a BMI 26,5 kg/m2, who is physically active and without other cardiovascular risk factors, episodic hypertension, headaches and blush. A contrast CT scan of the abdomen revealed a retroperitoneal intercavoaortic mass and 24-hour urine Normetanephrines were significantly elevated, indicating a functional paraganglioma. Laboratory tests revealed an elevated plasma normetanephrine level of 1838 pg/ml (reference values 0 - 180 pg/ml). The patient was prepared for surgery with alpha blockers, followed by beta blockers. Intraoperative blood pressure elevation was managed with intravenous beta blockers. Postoperatively, the patient did not require anti-hypertensives. The tumor, measuring 6x4x3.5 cm, histopathology and immunohistochemistry confirmed the diagnosis of a normetanephrine producing paraganglioma. The genetic study revealed a pathogenic variant in the SDHB gene, specifically the c.166_170delCCTCA (p.Pro56Tyrfs*5) in heterozygosity. This variant is associated with a 49% risk of Pheochromocytoma and Hereditary Paraganglioma, a 14% risk of Renal Carcinoma and susceptibility to Gastrointestinal Stromal Tumors. The c.166_170delCCTCA variant follows an autosomal dominant inheritance pattern, indicating a 50% risk of recurrence. Conclusions: The diagnosis of extra-adrenal retroperitoneal paragangliomas remains challenging due to the silent evolution of the condition. It is essential to consider screening patients who are young, and present with resistant arterial hypertension for secondary hypertension.