Primary malignant tumors of the adrenal glands

Authors

  • Madson Q. Almeida Universidade de Sao Paulo. Faculdade de Medicina. Hospital das Clinicas. Servico de Endocrinologia e Metabologia
  • João Evangelista Bezerra-Neto Universidade de Sao Paulo. Faculdade de Medicina. Hospital das Clinicas. Instituto do Cancer do Estado de Sao Paulo
  • Berenice B. Mendonça Universidade de Sao Paulo. Faculdade de Medicina. Hospital das Clinicas. Instituto do Cancer do Estado de Sao Paulo
  • Ana Claudia Latronico Universidade de Sao Paulo. Faculdade de Medicina. Hospital das Clinicas. Instituto do Cancer do Estado de Sao Paulo
  • Maria Candida B.V. Fragoso Universidade de Sao Paulo. Faculdade de Medicina. Hospital das Clinicas. Instituto do Cancer do Estado de Sao Paulo

DOI:

https://doi.org/10.6061/clinics/2018/e756s

Keywords:

Adrenocortical Carcinoma, Pheochromocytoma, Paraganglioma, Treatment

Abstract

Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7–2 cases per year and a worldwide prevalence of 4–12 cases per million/year. However, a much higher incidence of these tumors (415 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.

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Published

2019-02-18

Issue

Section

Review Articles

How to Cite

Almeida, M. Q., Bezerra-Neto, J. E., Mendonça, B. B., Latronico, A. C., & Fragoso, M. C. B. (2019). Primary malignant tumors of the adrenal glands. Clinics, 73(Suppl. 1), e756s. https://doi.org/10.6061/clinics/2018/e756s