Vol 70, No 3 (2019)
Case report
Published online: 2018-10-23

open access

Page views 2853
Article views/downloads 1886
Get Citation

Connect on Social Media

Connect on Social Media

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

Sandra Pekic1, Vladimir Jovanovic2, Goran Tasic2, Ivan Paunovic3, Svetislav Tatic4, Dusko Dundjerovic4, Mirjana Doknic1, Dragana Miljic1, Marko Stojanovic1, Marina Nikolic Djurovic1, Milan Petakov1, Vera Popovic5
Pubmed: 30351443
Endokrynol Pol 2019;70(3):298-303.


Pheochromocytomas and sympathetic paragangliomas are rare catecholamine-secreting tumours that represent very rare causes of intracerebral haemorrhage in the young, with only a few cases reported. A 32-year-old man presented to our emergency department because of sudden onset of severe headache. He had a six-month 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 haematoma. He was admitted to the Clinic for Neurosurgery and the haematoma was evacuated. The patient was comatose, 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 < 90 pg/mL). Abdominal CT scans revealed a large mass (6 cm) in the right adrenal gland. After adequate control of the hypertension was achieved with nonselective alpha- and beta-adrenergic blockers the tumour was excised. The histopathologic findings confirmed the diagnosis of pheochromocytoma. The genetic analysis demonstrated a duplication in exon 1 of the VHL gene. We reported a rare, potentially fatal complication of pheochromocytoma — an intracerebral haemorrhage. This case and review of similar rare cases in the literature illustrate the importance of early recognition of the characteristic symptoms of catecholamine excess in young patients with hypertension.

Article available in PDF format

View PDF Download PDF file


  1. Ruíz-Sandoval JL, Cantú C, Barinagarrementeria F. Intracerebral hemorrhage in young people: analysis of risk factors, location, causes, and prognosis. Stroke. 1999; 30(3): 537–541.
  2. Douma S, Doumas M, Petidis K. Intracerebral hemorrhage as the presenting feature of concurrent pheochromocytoma and paragangliomas. J Clin Hypertens (Greenwich). 2008; 10(12): 941–944.
  3. Havekes B, van der Klaauw AA, Weiss MM, et al. Pheochromocytomas and extra-adrenal paragangliomas detected by screening in patients with SDHD-associated head-and-neck paragangliomas. Endocr Relat Cancer. 2009; 16(2): 527–536.
  4. Waguespack SG, Rich T, Grubbs E. A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 2010; 95(5): 2023–2037.
  5. Newell KA, Prinz RA, Pickleman J. Pheochromocytoma multisystem crisis. A surgical emergency. Arch Surg. 1988; 123(8): 956–959.
  6. Whitelaw BC, Prague JK, Mustafa OG, et al. Phaeochromocytoma crisis. Clin Endocrinol (Oxf). 2014; 80(1): 13–22.
  7. Moraca-Kvapilová L, Op de Coul AA, Merkus JM. Cerebral haemorrhage in a pregnant woman with a multiple endocrine neoplasia syndrome (type 2A or Sipple's syndrome). Eur J Obstet Gynecol Reprod Biol. 1985; 20(4): 257–263.
  8. Scardigli K, Biller J, Brooks MH. Pontine hemorrhage in a patient with pheochromocytoma. Arch Intern Med. 1985; 145(2): 343–344.
  9. Aikawa N, Suga S, Inamasu J. A 35-year-old man with cerebral hemorrhage and pheochromocytoma: the second brain-dead organ donor in Japan. Keio J Med. 2000; 49(3): 117–130.
  10. Moritani H, Sakamoto M, Yoshida Y. Pheochromocytoma of the urinary bladder revealed with cerebral hemorrhage. Intern Med. 2001; 40(7): 638–642.
  11. Chuang HL, Hsu WH, Hsueh C. Spontaneous intracranial hemorrhage caused by pheochromocytoma in a child. Pediatr Neurosurg. 2002; 36(1): 48–51.
  12. Park SK, Lee JK, Joo SP. Spontaneous intracerebral haemorrhage caused by extra-adrenal phaeochromocytoma. J Clin Neurosci. 2006; 13(3): 388–390.
  13. Petramala L, Cavallaro G, Polistena A. Multiple catecholamine-secreting paragangliomas: diagnosis after hemorrhagic stroke in a young woman. Endocr Pract. 2008; 14(3): 340–346.
  14. Luiz HV, da Silva TN, Pereira BD. Malignant paraganglioma presenting with hemorrhagic stroke in a child. Pediatrics. 2013; 132(6): e1709–e1714.
  15. Mizukami H, Hara S, Kobayashi M. An autopsy case of bilateral adrenal pheochromocytoma-associated cerebral hemorrhage. Leg Med (Tokyo). 2013; 15(2): 91–95.
  16. Elder EE, Elder G, Larsson C. Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol. 2005; 89(3): 193–201.
  17. Moser M. Pheochromocytoma: "a disease with a thousand faces". J Clin Hypertens (Greenwich). 2009; 11(2): 87–88.
  18. Savage MW, Mah PM, Weetman AP. Endocrine emergencies. Postgrad Med J. 2004; 80(947): 506–515.
  19. Ionescu CN, Sakharova OV, Harwood MD. Cyclic rapid fluctuation of hypertension and hypotension in pheochromocytoma. J Clin Hypertens (Greenwich). 2008; 10(12): 936–940.
  20. Jindal V, Baker ML, Aryangat A. Pheochromocytoma: presenting with regular cyclic blood pressure and inverted Takotsubo cardiomyopathy. J Clin Hypertens (Greenwich). 2009; 11(2): 81–86.
  21. Park M, Hryniewicz K, Setaro JF. Pheochromocytoma presenting with myocardial infarction, cardiomyopathy, renal failure, pulmonary hemorrhage, and cyclic hypotension: case report and review of unusual presentations of pheochromocytoma. J Clin Hypertens (Greenwich). 2009; 11(2): 74–80.
  22. Rosas AL, Kasperlik-Zaluska AA, Papierska L, et al. Pheochromocytoma crisis induced by glucocorticoids: a report of four cases and review of the literature. Eur J Endocrinol. 2008; 158(3): 423–429.
  23. Scholten A, Cisco RM, Vriens MR. Pheochromocytoma crisis is not a surgical emergency. J Clin Endocrinol Metab. 2013; 98(2): 581–591.
  24. Neumann HPH, Bausch B, McWhinney SR, et al. Freiburg-Warsaw-Columbus Pheochromocytoma Study Group. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002; 346(19): 1459–1466.
  25. Amar L, Bertherat J, Baudin E. Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol. 2005; 23(34): 8812–8818.
  26. Gimenez-Roqueplo AP, Dahia PL, Robledo M. An update on the genetics of paraganglioma, pheochromocytoma, and associated hereditary syndromes. Horm Metab Res. 2012; 44(5): 328–333.
  27. Martins R, Bugalho MJ. Paragangliomas/Pheochromocytomas: clinically oriented genetic testing. Int J Endocrinol. 2014; 2014: 794187.
  28. Bausch B, Borozdin W, Neumann HPH, et al. European-American Pheochromocytoma Study Group. Clinical and genetic characteristics of patients with neurofibromatosis type 1 and pheochromocytoma. N Engl J Med. 2006; 354(25): 2729–2731.
  29. Brito JP, Asi N, Bancos I, et al. Testing for germline mutations in sporadic pheochromocytoma/paraganglioma: a systematic review. Clin Endocrinol (Oxf). 2015; 82(3): 338–345.