Association between pheochromocytoma and neurofibromatosis type I: a rare entity in the African population

Abstract

The association of pheochromocytoma in patients with neurofibromatosis type I has rarely been reported in low-income countries, especially on the African continent. A 43-year-old woman with neurofibromatosis type I was diagnosed with a right adrenal pheochromocytoma in Pretoria, South Africa. To our knowledge, this report is the first case to be published of a patient with neurofibromatosis type I diagnosed with a pheochromocytoma in Pretoria, and one of three cases on the African continent. The rarity may be due to the two associated conditions being under-reported, undiagnosed, misdiagnosed or possibly the association is rare on the African continent. The clinician dealing with these two conditions should be aware of the association.

Keywords: adrenal disorders; general surgery; genetic screening / counselling; neuroendocrinology.