Primary malignant tumors of the adrenal glands
Clinics
;
73(supl.1): e756s, 2018. tab
Article
in English
| LILACS
| ID: biblio-974949
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 (>15 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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Paraganglioma
/
Pheochromocytoma
/
Adrenal Cortex Neoplasms
/
Adrenal Gland Neoplasms
/
Adrenocortical Carcinoma
Type of study:
Diagnostic study
/
Prognostic study
/
Risk factors
Limits:
Humans
Language:
English
Journal:
Clinics
Journal subject:
Medicine
Year:
2018
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade de Sao Paulo/BR
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