Malignant neoplasms of adrenal gland

Description

Tumours arising from the adrenal cortex include adenomas and carcinomas. These are rare neoplasms but may cause a variety of hormonal symptoms, including hyperaldosteronism, Cushing syndrome, and virilisation. A small fraction of adrenocortical tumours are associated with an inherited tumour syndrome, including Li-Fraumeni syndrome and Carney complex. Benign and malignant phaeochromocytomas arise in the adrenal medulla and are derived from chromaffin cells of neural crest origin. Phaeochromocytomas may occur in the setting of several hereditary conditions, including multiple endocrine neoplasia types 2a and 2b, von Hippel Lindau disease and neurofibromatosis. Extra adrenal paragangliomas arise from chromaffin cells in sympathoadrenal and parasympathetic paraganglia. They occur in many parts of the body and can pose a significant challenge to surgeons and oncologists. Some function as chemoreceptors, others are endocrinologically active. Familial paragangliomas are associated with mutations of the mitochondrial complex II genes.

Coding note from above levels

For use of this category, reference should be made to the mortality coding rules and guidelines in the Reference Guide.

Excludes

• 2E07 – Malignant neoplasm metastasis in adrenal gland
• http://id.who.int/icd/entity/1965082709 – Malignant mesenchymal neoplasms

Exclusions from above levels

• http://id.who.int/icd/entity/879154182 – Neoplasms of central nervous system or related structures
• http://id.who.int/icd/entity/944754984 – Neoplasms of haematopoietic or lymphoid tissues