Intracerebral hemorrhage as a first sign of pheochromocytoma: case report and review of the literature.

Pheochromocytomas and sympathetic paragangliomas are rare catecholamine-secreting tumors and represent very rare causes of intracerebral hemorrhage in young, with only few cases reported. A 32-year-old man presented to our emergency department because of sudden onset of severe headache. He had a six months history of paroxysmal headache, palpitations and sweating. During examination he became somnolent and developed left-sided hemiplegia. A computed tomographic (CT) scan of the brain showed a right temporoparietal hematoma. He was admitted to the Clinic for Neurosurgery and hematoma was evacuated. The patient was comatous, on assisted respiration, with frequent hypertensive crises. An examination for possible secondary causes of hypertension was undertaken. Plasma metanephrine value was elevated (414 pg/ml, reference values < 90pg/ml). Abdominal CT scans revealed a large mass (6cm) in the right adrenal gland. After adequate control of the hypertension was achieved with an nonselective α and β adrenergic blockers, the tumor was excised. The histopathologic findings confirmed the diagnosis of pheochromocytoma. The genetic analysis demonstrated a duplication in exon 1 of VHL gene. We reported a rare, potentially fatal complication of pheochromocytoma, an intracerebral hemorrhage. This case and review of the similar rare cases in the literature illustrate the importance of early recognition of the characteristic symptoms of catecholamine excess in young patients with hypertension.