P-37 MEDIASTINAL PARAGANGLIOMA AS A CAUSE OF HYPERTENSION IN A YOUNG PATIENT:A CASE REPORT
Authors: Yilmaz Ezgi̇ , Can Bülent , Tamer Gonca 
Published: January 2025

Introduction

Paragangliomas, tumors that arise from extraadrenal chromaffin cells, are rarely found in the mediastinum. It is important to diagnose and remove paragangliomas because of its critical symptoms such as hypertension and can be curable with resection.

Clinical Case

A 18-year-old man, has no known disease, presented with episodes of palpitations, sweating associated with hypertension.The patient’s blood pressure measured at office was 180/110 mmHg. Electrocardiographic monitoring showed only nonspesific ST-T waves changes. Other secondary causes of hypertension were excluded. Suspecting of pheochromocytoma, 24-hour urinary metanephrine and normetanephrine levels were examined, the normetanephrine level was found to be elevated (1367,78 mcg/24 hour; normal range 88-444), whereas metanephrine level was normal (102,16 mcg/24 hour; normal range 52-341). Plasma metanephrine level was normal (58 ng/L; normal range 0-90).However plasma normetanephrine level was elevated (163,1 ng/L; normal range 0-90).The ratio of serum aldosterone level to the plasma renin activity was normal. 1 mg dexamethasone suppression test was normal. There was no adrenal mass in the abdominal MRI, but a mass measuring 4,3x 3,2 cm was seen in the right paravertebral area of the thorax in the area included in the cross-sections. In Ga-68 Dota-tate positron emission tomography (PET),a mass lesion of 43x30 mm in size, showing intense somatostatin receptor expression (SUV-max: 31.9) was detected in the right paravertebral area at the level of the 8th thoracic vertebra, which was evaluated as paraganglioma. For more than 1 month, the patient had been prepared for the surgery administering combination doxazosine and propronol after the treatment with only doxazosin. The patient was consulted to the thoracic surgery department. Phentolamine was provided before surgery. The mass was resected via thoracotomy. In histological evaluation, widespread zelballen and pseudorosette structures were observed. There was no comedonecrosis, and there was no vascular and capsular invasion. Ki 67 proliferation index was 4-5 %. On immunohistochemical staining, the mass tested positive for synaptophysin and chromagranin and negative for cytokeratins. One month after surgery normetanephrine (147,85 mcg/24h; normal range: 88-444) and metanephrine (102,16 mcg/24 h; normal range 52-341) levels were normal. The patients blood pressure was stabilized at 120/70 mmHg and heart rate was 80 bpm. In the genetic analysis of the patient, the NDUFA6 gene was detected and mitechondrial complex 1 deficiency was associated with nuclear type 33 disease and the patient was accepted as a carrier. Screening with 24 hour urine metanephrine and normetanephrine was recommended to patient’s family members.

Conclusion

Since, there are no definite microscopic criteria for the distinction between benign and malignant tumors, radical excision is necessary. The symptoms are curable with resection of tumor, long term follow up for recurrence is important.Figure 1:Gallium-68 Dota-Tate PETShowing the mass(SUV-max:31.99)