Endocrine Abstracts

Introduction: Although the radiographic and histological features are the same as benign pheochromocytomas, malignant and metastatic pheochromocytoma is differentiated by invasion of the surrounding tissues and organ metastasis. Here, we present a young patient with a diagnosis of metastatic pheochromocytoma presenting with hypertension attacks, headache and flushing.

Case presentation: A 22-year-old woman with a history of hypertension who had been suffering from headache new-onset flushing and hypertensive attacks in holter blood plessure for 6 years and is presented. MRI scan of abdomen revealed a solid mass of 70×62×64 mm in right supra renal space which suppressed the right kidney and metastatic lymphadenomegaly suppressing the inferior vena cava at the portocaval area. Laboratory: ACTH 40.2 pg/ml, serum cortisol 20.8 μg/dL, 1-mg dexamethasone-suppression test: cortisol 0.5 μg/dL, calcitonin 4.29 pg/ml, aldosterone 339 pg/ml, renin 7.04 ng/ml per h. 24-hr-urinary cortisol and metanephrine level were normal but 24-hr-urinary normetanephrin level was elevated (40 nmol/24 hr (N: 38–208), 283 μg/24 hr (N: 276–341) and 11345/24 hr (N:88–444)). Neck ultrasound was normal. Right surrenalectomy and periportal lymph node dissection were performed. Histopathological examination of the surgical specimens was consistent with pheochromocytoma with lymph node metastasis. Genetic testing for hereditary pheochromocytoma revealed heterozygous c.890G> A /p.Arg297 His change in the 5th exon in the RET gene. In the first month after surgery 24-hr urinary cortisol, metanephrine and normetanephrine levels were found to be normal.

Discussion: Pheochromocytoma is more frequent present in 4th to 5th decades of life and is the cause of hypertension in 0.2% to 0.6% of patients. 10% of these are malignant as proven by the presence of metastases. Our case was diagnosed during the investigation of hypertension attacks at a young age. Genetic screening should not be forgotten in patients who were diagnosed as unilateral pheochromocytoma at a young age.

Keywords: Hypertension, metastatic pheochromocytoma