Myocardial infarction in a 30-year-old patient with pheochromocytoma and type 1 neurofibromatosis.

Abstract

Chromaffinoma of the adrenal medulla (pheochromocytoma--PHEO) is a rare cause of arterial hypertension which is diagnosed incidentally or run in a family as a component of disease syndromes of the genetic origin. PHEO is diagnosed in about 5-10% of patients with type 1 neurofibromatosis (NF1). In a patient group with diagnosed arterial hypertension and NF1, PHEO is diagnosed with a much higher frequency, i.e. 20-56%. Myocardial injury in a patient without coronary risk factors is very rare. Increased circulating levels of catecholamines in patients with chromaffinoma may cause damage to myocardium without any atherosclerotic lesions in the coronary arteries. A correct diagnosis of PHEO allows the right treatment to be administered. The present paper discusses the case of a patient with NF1 and periodic arterial hypertension in the course of unidentified chromaffinoma, which was complicated with myocardial infarction. The evaluation of the secondary arterial hypertension led to the detection of the adrenal tumor. Based on the clinical presentation and the tumor characteristics, on computed tomography, PHEO was suspected. The level of methoxycatecholamines in a 24-hour urine sample significantly exceeded the reference values. The patient underwent laparoscopic, right-sided adrenalectomy, and the histopathological examination definitely concurred with the diagnosis of PHEO. During the post-surgical period, the arterial hypertension normalized without the administration of hypotensive drugs. The patient is still cared for by the clinic. The diagnosis toward PHEO is recommended if the patient with NF1 shows arterial hypertension. Proper diagnosis and treatment protects the patient against life-threatening cardiovascular complications.

• Authors: Anna Babińska, Alicja Gnacińska, Renata Swiatkowska-Stodulska, Krzysztof Sworczak
• Journal: Polskie Archiwum Medycyny Wewnetrznej (Pol Arch Med Wewn) Vol. 118 Issue 9 Pg. 517-23 (Sep 2008) Poland
• PMID: 18846988 (Publication Type: Case Reports, Journal Article)

Chemical References

• Normetanephrine

Topics

• Adrenal Gland Neoplasms (complications, diagnostic imaging, surgery)
• Adrenalectomy
• Adult
• Female
• Humans
• Myocardial Infarction (diagnosis, etiology)
• Neurofibromatosis 1 (complications, diagnostic imaging, urine)
• Normetanephrine (urine)
• Pheochromocytoma (complications, diagnostic imaging, surgery, urine)
• Radiography