ABSTRACT Although rare, adrenocortical carcinoma (ACC) should be considered in individuals with neurofibromatosis type 1 (NF1) presenting with adrenal incidentalomas. Despite pheochromocytoma being more prevalent in NF1, appropriate functional evaluation is important in this context to rule out adrenocortical carcinoma given its aggressive nature and poor prognosis.
Pluim et al. (Sun,) studied this question.
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