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Review
. 2008 Aug;35 Suppl 1(Suppl 1):S27-34.
doi: 10.1016/j.nucmedbio.2008.04.007.

A clinical overview of pheochromocytomas/paragangliomas and carcinoid tumors

Ioannis Ilias  1 , Karel Pacak
Affiliations
Review

A clinical overview of pheochromocytomas/paragangliomas and carcinoid tumors

Ioannis Ilias et al. Nucl Med Biol. 2008 Aug.

Abstract

Pheochromocytomas/paragangliomas are rare tumors; most are sporadic. Biochemical proof of disease is better with measurement of plasma metanephrines and less cumbersome than determinations in urine; its implementation is expanding. Anatomical imaging with computed tomography or magnetic resonance imaging should be followed by functional (nuclear medicine) imaging: chromaffin tumor-specific methods are preferred. Treatment is surgical; for nonoperable disease other options are available. Overall 5-year survival is 50%. Carcinoid tumors derive from serotonin-producing enterochromaffin cells in the fore-, mid- or hindgut. Biochemical screening (and follow-up) is done with measurements of 5-hydroxyindoloacetic acid in urine. For most carcinoids, functional imaging is better than other modalities in localizing primary tumors. Surgery is the treatment of choice; nonresectable tumors are treated with somatostatin analogs or chemotherapy. Overall 5-year survival for patients with carcinoids is 67%.

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Figures

Figure 1
Figure 1
Biosynthetic pathways in pheochromocytomas/paragangliomas and carcinoids; c1: tryptophan hydroxylase; c2: hydroxytryptophan decarboxylase; p1: tyrosine hydroxylase; p2: DOPA decarboxylase; p3: dopamine-beta-hydroxylase; p4: phenylethanolamine-N-methyltransferase
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