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Medline ® Abstract for Reference 48

of 'Clinical features, diagnosis, and management of von Hippel-Lindau disease'

48
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Clinical utility of functional imaging with¹⁸F-FDOPA in Von Hippel-Lindau syndrome.
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Weisbrod AB, Kitano M, Gesuwan K, Millo C, Herscovitch P, Nilubol N, Linehan WM, Kebebew E
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J Clin Endocrinol Metab. 2012;97(4):E613. Epub 2012 Jan 18.
 
CONTEXT: Von Hippel-Lindau (VHL) syndrome is an inherited cancer syndrome in which patients are at risk of developing multiple tumors in different organs. 6-L-¹⁸F-fluorodihydroxyphenylalanine (¹⁸F-FDOPA) positron emission tomography (PET) is a relatively new metabolic imaging tracer proposed for the use of localizing sites of neuroendocrine tumors. There are limited data on the clinical utility of using¹⁸F-FDOPA PET for identifying neuroendocrine tumors in a high-risk population such as VHL.
OBJECTIVE: The aim of this prospective study was to evaluate the clinical utility of¹⁸F-FDOPA PET in patients with VHL-related tumors.
DESIGN: Radiological findings were prospectively collected from four imaging modalities: computed tomography, magnetic resonance imaging (MRI),¹⁸F-fluorodeoxyglucose PET, and¹⁸F-FDOPA PET.¹⁸F-FDOPA PET findings were compared with those from other imaging modalities, as well as with clinical and laboratory data, and pathology findings if patients underwent an operation.
RESULTS: In 52 patients with VHL, 390 lesions were identified by computed tomography (n = 139), MRI (n = 117),¹⁸F-fluorodeoxyglucose PET (n = 94), and¹⁸F-FDOPA PET (n = 40).¹⁸F-FDOPA PET identified 20 pancreatic and 20 extrapancreatic tumors, including lesions in the adrenal gland (n = 11), kidney (n = 3), liver (n = 4), lung (n = 1), and cervical paraganglioma (n = 1). These tumor sites were not seen by conventional imaging studies in 9.6% of patients and 4.4% of lesions. Seven of eight patients who had an¹⁸F-FDOPA PET-positive lesion underwent resection, and pathology showed a neuroendocrine tumor. Four of 10 patients with positive adrenal uptake had elevated catecholamine levels, and six of 10 patients had a discrete mass on axial imaging.
CONCLUSIONS: ¹⁸F-FDOPA PET is a useful complementary imaging study to detect neuroendocrine tumors in patients with VHL undergoing surveillance, especially in those suspected to have adrenal pheochromocytoma or unusual ectopic locations.
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Endocrine Oncology Section, Surgery Branch, National Cancer Institute, National Institutes of Health, Clinical Research Center, Building 10-CRC, Room 3-3940, 10 Center Drive, MSC 1201, Bethesda, Maryland 20892, USA.
PMID