title: Clinical utility of functional imaging with ¹⁸F-FDOPA in Von Hippel-Lindau syndrome.
abstract: BACKGROUND
  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 using ¹⁸F-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 of ¹⁸F-FDOPA PET in patients with VHL-related tumors.
  
  
  METHODS
  Radiological findings were prospectively collected from four imaging modalities: computed tomography, magnetic resonance imaging (MRI), ¹⁸F-fluorodeoxyglucose PET, and ¹⁸F-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), and ¹⁸F-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 an ¹⁸F-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.
authors.affiliation: 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.
authors.forename: Allison B
authors.initials: AB
authors.lastname: Weisbrod
authors.forename: Mio
authors.initials: M
authors.lastname: Kitano
authors.forename: Krisana
authors.initials: K
authors.lastname: Gesuwan
authors.forename: Corina
authors.initials: C
authors.lastname: Millo
authors.forename: Peter
authors.initials: P
authors.lastname: Herscovitch
authors.forename: Naris
authors.initials: N
authors.lastname: Nilubol
authors.forename: W Marston
authors.initials: WM
authors.lastname: Linehan
authors.forename: Electron
authors.initials: E
authors.lastname: Kebebew
mesh_terms: D000328:Adult; D016543:Central Nervous System Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D015331:Cohort Studies; D003287:Contrast Media; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives*; D005260:Female; D005462:Fluorine Radioisotopes; D006801:Humans; D007680:Kidney Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D049268:Positron-Emission Tomography; D011446:Prospective Studies; D006623:von Hippel-Lindau Disease / Q000503:physiopathology*
chemical_list: D003287:Contrast Media; D005462:Fluorine Radioisotopes; C043437:fluorodopa F 18; D004295:Dihydroxyphenylalanine