Neoplasms of adrenal medulla

Adrenal phaeochromocytoma: uncommon neoplasm arising from medullary neuroendocrine cells (phaeochromocytes); may be

• Sporadic

OR

• Familial: often bilateral  other familial syndromes (e.g. MEN II)

AND

• Secretory: (catecholamine production) associated with paroxysmal or persistent hypertension headache, sweating, tachycardia, fatigue, tremor, GIT and cardiac complications; may lead to other endocrine disturbances (e.g. Cushing syndrome)

OR

• Non-secretory

Most are benign (90%) though histological distinction is difficult – only certain criterion is metastasis; diagnosed by radiographic imaging and plasma catecholamine assay.

Note: paraganglioma is identical neoplasm arising in extra-adrenal neuroendocrine cells (paraganglionic tissue) related to ANS in head and neck.

Tumours of extra-adrenal paraganglia include:

• Neuroblastoma (malignant)

• Ganglioneuroma (benign)

Category: Pathology Notes