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Pheochromocytoma

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Principles and Management of Adrenal Cancer

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Abstract

A pheochromocytoma is a chromaffin cell tumor which has the ability to synthesize and secrete catecholamines, and these in turn produce symptoms in the afflicted patient. The catecholamine secretion, rather than the underlying neoplasm, renders a pheochromocytoma clinically important and dangerous, yet a potentially curable form of secondary hypertension. The chromaffin cell which gives rise to a pheochromocytoma derives its name from its ability to synthesize and store catecholamines, and therefore stain brown on treatment with chromium salts. These cells are found mainly in the adrenal medulla, but also appear along the ganglia and paraganglia of the sympathetic chain and organs of Zuckerkandl located anteriorly at the bifurcation of the aorta. Chromaffin cells are derived from the primitive neural crest and share with other neural crest cell lines the ability to take up amine precursors and decarboxylate them, giving rise to the acronym APUD cells (amine precursor uptake and decarboxylation) [64,69]. This relationship of APUD cells explains the presence of pheochromocytoma as one part of familial dystrophies of APUD cells, as in multiple endocrine adenomas type II (MEA-II) [6]. Sympathogonia, the primordial stem cells arising from the neural crest, migrate out of the central nervous system and differentiate into ganglion cells, neuroblasts, or chromaffin cells [14]. Tumors may arise from each of these cell lines, namely neuroblastoma, ganglioneuroma, and pheochromocytoma.

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  1. Section of Urologic Oncology, Department of Surgery, School of Medicine, University of Maryland, University Hospital, Baltimore, Maryland, 21201, USA

    Nasser Javadpour MD (Professor and Director) (Professor and Director)

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Hamilton, B.P.M. (1987). Pheochromocytoma. In: Javadpour, N. (eds) Principles and Management of Adrenal Cancer. Springer, London. https://doi.org/10.1007/978-1-4471-3134-2_10

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  • DOI: https://doi.org/10.1007/978-1-4471-3134-2_10

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