Recent Advances in Genetics, Diagnosis, Localization, and Treatment of Pheochromocytoma
- Moderator: Karel Pacak, MD, PhD, DSc;
- Discussants: W. Marston Linehan, MD;
- Graeme Eisenhofer, PhD;
- McClellan M. Walther, MD; and
- David S. Goldstein, MD, PhD
-
From the National Institutes of Health, Bethesda, Maryland.
-
Figure 1. Diploid cells have two copies of each gene. A tumor suppressor gene, such as the von Hippel–Lindau gene, is consistent
with the Knudson model. In the hereditary form of cancer, the patient inherits a mutation of one copy of the gene. In the
tumor, such as a pheochromocytoma in a patient with von Hippel–Lindau disease, the second copy is inactivated by a mechanism
such as mutation or deletion. The Knudson two-hit model.
-
Figure 2. Values in parentheses indicate expected numbers of patients with and without pheochromocytoma at different steps
during diagnosis, assuming a 2% incidence of the tumor in a population of 1000 patients undergoing testing for clinically
suspected pheochromocytoma (that is, 20 patients with and 980 without pheochromocytoma). CT = computed tomography; MRI = magnetic
resonance imaging. * For continuation of the algorithm, see . Algorithm showing use of biochemical tests for diagnosis of pheochromocytoma.Figure 4
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Figure 3. F 6-[ 18 F]Fluorodopamine positron emission tomography before and after left adrenelectomy for a large pheochromocytoma.
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Figure 4. CT = computed tomography; MIBG = metaiodobenzylguanidine; MRI = magnetic resonance imaging; PET = positron emission
tomography. Imaging algorithm for patients whose results on biochemical tests are consistent with pheochromocytoma.
- Copyright ©2004 by the American College of Physicians
-
Ann Intern Med
February 20, 2001
vol. 134
no. 4
315-329
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Figure 1. Diploid cells have two copies of each gene. A tumor suppressor gene, such as the von Hippel–Lindau gene, is consistent with the Knudson model. In the hereditary form of cancer, the patient inherits a mutation of one copy of the gene. In the tumor, such as a pheochromocytoma in a patient with von Hippel–Lindau disease, the second copy is inactivated by a mechanism such as mutation or deletion. The Knudson two-hit model.
Figure 2. Values in parentheses indicate expected numbers of patients with and without pheochromocytoma at different steps during diagnosis, assuming a 2% incidence of the tumor in a population of 1000 patients undergoing testing for clinically suspected pheochromocytoma (that is, 20 patients with and 980 without pheochromocytoma). CT = computed tomography; MRI = magnetic resonance imaging. * For continuation of the algorithm, see . Algorithm showing use of biochemical tests for diagnosis of pheochromocytoma.Figure 4
Figure 3. F 6-[ 18 F]Fluorodopamine positron emission tomography before and after left adrenelectomy for a large pheochromocytoma.
Figure 4. CT = computed tomography; MIBG = metaiodobenzylguanidine; MRI = magnetic resonance imaging; PET = positron emission tomography. Imaging algorithm for patients whose results on biochemical tests are consistent with pheochromocytoma.
