Objective: To describe the course and anatomo-pathologic findings in a patient with a cortisol-producing adrenocortical carcinoma (ACC) who presented with disseminated invasive aspergillosis (DIA). We also review the clinical characteristics of ACC and the pathogenesis of DIA in the setting of hypercortisolemia. Methods: We describe the case of a 47-year-old man with a recent diagnosis of ACC associated with hypercortisolism, who presented with a large right basal ganglia hematoma and lesions on multiple organs thought to be metastatic. The patient deteriorated rapidly and unfortunately 6 days after presentation he died. An autopsy was performed which revealed DIA as the cause of death. Results: DIA in ACC was reported for the first time in 1981. Since then, no other case has been reported in the literature associating these 2 conditions. The lesions this patient presented on images were difficult to differentiate from metastatic lesions. The lack of inflammatory features due to hypercortisolemia accounted for this. Both ACC and DIA have very high mortality rates, even when DIA is treated appropriately. Conclusion: Opportunistic fungal infections are common in hypercortisolemia, however disseminated and invasive forms are rare. Awareness of uncommon presentations of hypercortisolemia is important for early treatment of those patients with a chance of survival. (AACE Clinical Case Rep. 2019;5:e233-237) Abbreviations: ACC = adrenocortical carcinoma; CT = computed tomography; DIA = disseminated invasive aspergillosis e234 Aspergillosis in ACC, AACE Clinical Case Rep. 2019;5(No. 4)
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