Adrenocortical carcinoma

Definition

Epidemiology

Adrenocortical carcinoma is a rare tumour of the adrenal cortex with an incidence of about on per million. Woman are affected more often than men. There is a bimodal age distribution with peaks in early childhood and over the age of 60 years.

Clinical features

Radiology

Macroscopic appearances

Histopathology

Necrosis, haemorrhage, vascular invasion mitotic count and atypical mitoses are indicators of malignancy. Scoring systems for malignancy have been proposed.

Immunohistochemistry

A Ki-67 index has been proposed as indicative of malignancy, with cut off values from various authors from 2.5% to 4%. Expression of MMP-2 may be a useful indicator of malignancy1.

Ultrastructure

Differential diagnosis

• Adrenal adenoma

Management

Prognosis

The mortality is in excess of 90%, with a median survival of 30 months.

References

1 Volante M, Sperone P, Bollito E, et al. Matrix metalloproteinase type 2 expression in malignant adrenocortical tumors: Diagnostic and prognostic significance in a series of 50 adrenocortical carcinomas. Mod Pathol 2006; 19:1563-9

This page last revised 5.1.2007.

©SMUHT/PW Bishop