Endocrine Abstracts

Introduction

Endocrinopathies represent about 1–3% of secondary hypertension.The non-head-neck paragangliomas are rare neuroendocrine tumors that arise from the ganglia of the sympathetic nervous system. About 75% are intra-adbdominal, thus they are often mistaken for adrenal pheocromocitoma.They usually secret normetanephrine and chromogranin A, which are responsible of 0.2–0.6% of secondary hypertension.

Objective

A case report of a young man with a 6 years history of paroxysmal, well tolerated hypertension.

Case report

A 26-year-old male presented in our clinic with a long-term history of hypertension crisis associated with palpitations, headache, cold sweats and tremor, triggered by physical effort and mental stress, that attenuated spontaneously in the last two years. Abdominal MRI performed 2 years ago suggested a vascular tumor on the left adrenal gland (40/42/46 cm). The urinary metanephrines were elevated. No treatment was performed at that time.

Results

Current laboratory studies point out both an elevated plasma and urinary normetanephrine level as well as chromogranin A. Matinal cortisol and ACTH were normal. The MRI revealed one tumoral node (48/44/47.5 cm) without demarcation in relation to the pancreas, left paraaortic, focally adjacent to the splenic and left renal vascular pedicle. Paraganglioma was suspected and treatment with alpha1-selective adrenergic receptor blockers was initiated, followed by beta-adrenergic receptor blockers. This treatment has been prescribed for 2 weeks as preoperative preparation. Laparoscopic surgery was performed without any complications. Postoperative, the blood pressure values were maintained in normal range. Immunocytochemical analysis showed that tumor cells were positive for chromogranin A, S100, synaptofisin and Ki67 3%, with a GAPP score =3/10. The histologic features combined with the immunocytochemical and ultrastructural findings confirmed the diagnosis of paraganglioma. Genetic testing showed a germline mutation in the SDHB gene. Follow-ups after the 1st, 3rd, 6th, 12th, 24th months after surgery revealed normal hormonal profile and negative PET-CT scan.

Conclusion

Although our patient has multiple negative risk factors (young age, voluminous tumor, SDHB gene mutation), the clinic was oligosymptomatic and the evolution was favorable, without any recurrence at the present.