title: Multiple catecholamine-secreting paragangliomas: diagnosis after hemorrhagic stroke in a young woman.
abstract: OBJECTIVE
  To describe a case of multiple catecholamine-secreting paragangliomas, with a hemorrhagic stroke as the main clinical manifestation.
  
  
  METHODS
  We present a case report with clinical, laboratory, histologic, and genetic details.
  
  
  RESULTS
  A 23-year-old woman with a history of hypertension treated with orally administered medications presented to our emergency department because of sudden onset of hemiplegia of the left side of the body. A computed tomographic scan of the brain showed a right frontoparietal hematoma, and her blood pressure was 185/115 mm Hg. She was admitted to the Department of Neurosurgery, and an external drain was inserted to evacuate the hematoma. She was then referred to the Department of Clinical Sciences, where a search for possible secondary causes of hypertension was undertaken. Substantially elevated urinary levels of vanillylmandelic acid and metanephrines were found, and a pheochromocytoma was suspected. Abdominal computed tomographic scans revealed a large retroperitoneal mass (3.6 by 4 cm) and similar smaller lesions in the right adrenal gland, between the aorta and the vena cava, and in the left paraaortic area. Iodine I 123 metaiodobenzylguanidine scintigraphy showed high uptake in those same areas, consistent with the diagnosis of multiple catecholamine-secreting paragangliomas. After adequate control of the patient's hypertension was achieved with an alpha1-adrenergic receptor blocker, a Ca2+ antagonist, and a beta-adrenergic blocking agent, the tumors were excised in the Department of Surgery. The histopathologic findings confirmed the diagnosis of multiple paragangliomas. The genetic analysis demonstrated an exon 4 mutation in codon 109 (CAA>TAA, Gln>Stop) of the SDHD gene.
  
  
  CONCLUSIONS
  Although cerebral hemorrhage is an unusual complication of pheochromocytomas or paragangliomas, early recognition of the characteristic symptoms of headache, palpitations, and diaphoresis in a patient with hypertension and prompt appropriate intervention can minimize the morbidity associated with such tumors and prevent a potentially fatal outcome.
authors.affiliation: Department of Clinical Sciences, University of Rome Sapienza, Rome, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Petramala
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cavallaro
authors.forename: Andrea
authors.initials: A
authors.lastname: Polistena
authors.forename: Dario
authors.initials: D
authors.lastname: Cotesta
authors.forename: Antonella
authors.initials: A
authors.lastname: Verrienti
authors.forename: Antonio
authors.initials: A
authors.lastname: Ciardi
authors.forename: Piernatale
authors.initials: P
authors.lastname: Lucia
authors.forename: Sebastiano
authors.initials: S
authors.lastname: Filetti
authors.forename: Emilio
authors.initials: E
authors.lastname: D'Erasmo
authors.forename: Giorgio
authors.initials: G
authors.lastname: De Toma
authors.forename: Claudio
authors.initials: C
authors.lastname: Letizia
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging* / Q000235:genetics; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging; D002395:Catecholamines / Q000378:metabolism*; D002543:Cerebral Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology; D005260:Female; D006801:Humans; D009154:Mutation / Q000235:genetics; D010235:Paraganglioma / Q000150:complications* / Q000000981:diagnostic imaging* / Q000235:genetics; D020521:Stroke / Q000000981:diagnostic imaging* / Q000209:etiology*; D013385:Succinate Dehydrogenase / Q000235:genetics; D014057:Tomography, X-Ray Computed
chemical_list: D002395:Catecholamines; C491545:SDHD protein, human; D013385:Succinate Dehydrogenase