A "Pheo" Lurks: Novel Approaches for Locating Occult Pheochromocytoma
Karel Pacak,
David S. Goldstein,
John L. Doppman,
Barry L. Shulkin,
Robert Udelsman and
Graeme Eisenhofer
Pediatric and Reproductive Endocrinology Branch, National Institute
of Child Health and Human Development (K.P.); Clinical Neurocardiology
Section, National Institute of Neurological Disorders and Stroke
(D.S.G., G.E.); and Department of Radiology, Clinical Center (J.L.D.),
National Institutes of Health, Bethesda, Maryland 20892; Division of
Nuclear Medicine, Department of Radiology, University of Michigan
(B.L.S.), Ann Arbor, Michigan 48109; and Division of Endocrine and
Oncologic Surgery, The Johns Hopkins University (R.U.), Baltimore,
Maryland 21218
Address all correspondence and requests for reprints to: Karel Pacak, M.D., Ph.D., D.Sc., Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 9D42, 10 Center Drive, MSC-1583, Bethesda, Maryland 20892-1583. E-mail:
karel{at}mail.nih.gov
Abstract
Most, but not all, pheochromocytomas can be localized by computed
tomographyor magnetic resonance imaging. Here we introduce two novel
approachesfor localization of pheochromocytoma in a patient in whom
conventionalimaging modalities failed to show the tumor. First, we
establishthat measurements of plasma free metanephrines coupled with
venacaval sampling are useful for localizing occult pheochromocytoma,
particularlywhen elevations in plasma catecholamines are slight or
intermittent.Second, we show that positron emission tomographic
scanningusing the imaging agent 6-[18F]fluorodopamine as
a substratefor the norepinephrine transporter offers a highly
effectivemethod for tumor localization. These novel approaches may be
ofvalue in difficult cases, where biochemical and clinical evidenceof
pheochromocytoma is compelling, yet conventional imagingmodalities
fail to locate the tumor.
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