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UPDATE ON TUMOURS OF THE ADRENAL GLAND AND EXTRA‐ADRENAL PARAGANGLIOMAS

McNicol, Anne Marie

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Pathology - Journal of the RCPA: 2010 - Volume 42 - Issue - p S13–S14
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Adrenal cortical adenomas occur in about 5% of the population while adrenal cortical carcinoma is a rare, but very aggressive, tumour. Most carcinomas are easily diagnosed because they are obviously invasive or metastatic at presentation. However, it is important to identify malignant potential in intra‐adrenal tumours. Multifactorial histological approaches will be discussed. Molecular genetic differences have been reported between benign and malignant tumours, but are not yet more sensitive or specific than histology.

Paragangliomas are of two types, sympathetic and parasympathetic. Phaeochromocytoma is a term reserved for intra‐adrenal tumours. Others should be defined by type and site. Malignancy is defined only by the presence of metastases. No single histological feature defines malignant potential but attempts to develop multifactorial analyses look promising. Over 30% of paragangliomas are familial and the genetic backgrounds will be discussed. There is evidence for genotype/phenotype correlations in some of these. The genes involved in the pathogenesis of sporadic tumours appear to be different from the familial forms, but the same signalling pathways seem to be involved.

© 2010 Royal College of Pathologists of Australasia