A clinical overview of pheochromocytomas/paragangliomas and carcinoid tumors.
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%.
Ilias
Ioannis
I
Department of Endocrinology, E. Venizelou Hospital, Athens GR-11521, Greece. iiliasmd@yahoo.com
Pacak
Karel
K
D002276:Carcinoid Tumor / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D010235:Paraganglioma / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D010673:Pheochromocytoma / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D049268:Positron-Emission Tomography; D011379:Prognosis; D014057:Tomography, X-Ray Computed