Epidemiology
ACC = 90 % of all adrenal cancers
The annual incidence is 1–2 in a 1,000,000 population
Bimodal age incidence: below 5 years in children and between age 40 and 50 years
Vast majority are sporadic. Some genetically linked associations
At presentation, only 30 % of ACC are confined to the adrenal gland
Only about 1 ACC out of every 4,000 adrenal tumours
Poor prognosis
Symptoms and signs
In functioning tumours (62 % of cases): Virilization in females and feminization of males
Ipsilateral loin or abdominal pain, fever, anorexia, weight loss
Palpable mass on physical examination, which means a large tumour burden
Rarely varicocoele, acute abdominal signs
Incidental findings (incidentaloma)
References
1.
2.
Wajchenberg BL, Albergaria Pereira MA, Medonca BB, et al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer. 2000;88(4):711–36.PubMed
3.
Crucitti F, Bellantone R, Ferrante A, Boscherini M, Crucitti P. The Italian Registry for Adrenal Cortical Carcinoma: analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery. 1996;119(2):161–70.CrossRefPubMed
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