Neuron-specific enolase (NSE) is an isoform of the glycolytic enzyme enolase specific to neural tissues, and is found in high levels in the serum in different endocrine tumors like carcinoids, medullary thyroid carcinoma, pituitary adenomas, islet cell tumors, small-cell lung carcinoma, malignant pheochromocytoma, ganglioneuromas, schwannomas and NBs (126–132). The gene discussed is ENO2; the disease is malignant adrenal gland pheochromocytoma.