Primary malignant tumors of the adrenal glands

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.

Clinical analysis of adrenal lesions larger than 5 cm in diameter (an analysis of 251 cases)

“…In this case, surgical treatment was not the most appropriate method. In other studies, adrenocortical carcinoma patients had a very poor prognosis with a 5-year overall survival below 30% in most series [31]. Age, tumor property, general health, comorbidities, and patient preference should be taken into account in order to maximize the benefits to patients.…”

The Role of Biomarkers in Adrenocortical Carcinoma: A Review of Current Evidence and Future Perspectives

“…In the literature, different cut off values can be found to determine it: >95, >50, >100 g, but also in some cases tumors <50 g had malignant potential [ 72 , 73 , 74 , 75 ]. Most morphological studies confirm the size of the malignant lesion to be greater than 50–65 mm, ranging from 20 to 196 mm [ 72 , 73 , 74 , 76 ]. It is important to keep in mind that tumor size might be underestimated by radiological investigation and not correlate with the real size of tumor lesion [ 76 , 77 ].…”

“…Most morphological studies confirm the size of the malignant lesion to be greater than 50–65 mm, ranging from 20 to 196 mm [ 72 , 73 , 74 , 76 ]. It is important to keep in mind that tumor size might be underestimated by radiological investigation and not correlate with the real size of tumor lesion [ 76 , 77 ]. Except that above mentioned, further examination should include evaluation of capsule integrity and the presence of hemorrhage, necrosis and invasion [ 76 ].…”

“…Ki-67 is a protein expressed in all cell cycle phases except G0 and represents a cell proliferation index. Ki-67 labeling index of more than 5% confirms the diagnosis of ACC [ 7 , 76 ]. Ki67 index >10% correlates with higher risk of recurrence in ACCs and it is associated with worse overall survival in patients with advanced disease or rapid disease recurrence [ 76 , 81 ].…”

“…Ki-67 labeling index of more than 5% confirms the diagnosis of ACC [ 7 , 76 ]. Ki67 index >10% correlates with higher risk of recurrence in ACCs and it is associated with worse overall survival in patients with advanced disease or rapid disease recurrence [ 76 , 81 ]. Although practical utility of Ki-67 staining was indisputable and confirmed in many studies, one should keep in mind that it is hard to set a diagnostic threshold because of possible interobserver variations [ 82 ].…”