作者: A. Patalano , V. Brancato , F. Mantero
DOI: 10.1159/000178049
关键词: Asymptomatic 、 Virilization 、 Rare disease 、 Mitotane 、 Cushing syndrome 、 Chemotherapy 、 Medicine 、 Vascular endothelial growth factor 、 Oncology 、 Immunology 、 Internal medicine 、 Etoposide
摘要: Background: With a reported incidence of 1 to 2 cases per million, adrenocortical cancer (ACC) is rare disease with poor prognosis. Age distribution shows two peaks: early childhood and between age 40 50 years, females more frequently affected. Sequelae can include Cushing syndrome, virilization hypertension or local symptoms consistent abdominal obstruction. Although most ACC are sporadic origin, they may also occur as part congenital familial in which the genetic abnormalities well established. be discovered incidentally asymptomatic individuals. In ACC, some molecular modifications commonly observed (i.e., overexpression insulin-like growth factor II vascular endothelial somatic mutations tumor protein 53). When surgical resection impossible ineffective, chemotherapy etoposide, doxorubicin cisplatin plus mitotane streptozotocin used; however, overall survival rates disappointing. Conclusions: Hormonal evaluation essential diagnose prognosis depends on many factors. New treatments, such I receptor antibodies, tyrosine kinase inhibitors other antiangiogenic compounds, now being intensively investigated identify better therapies for this extremely severe malignant neoplasia.