title: 18F-DOPA PET/CT in the evaluation of hereditary SDH-deficiency paraganglioma-pheochromocytoma syndromes.
abstract: OBJECTIVE
  This study aims to evaluate the role of F-DOPA PET/CT in staging and follow-up of paraganglioma syndromes succinate dehydrogenase (SDH)-mutation-related patients, comparing F-DOPA PET/CT results with morphological imaging and biochemical results.
  
  
  METHODS
  We retrospectively studied 10 consecutive patients (3 F, 7 M, mean age 32 yrs), all with a genetically demonstrated SDH mutation (5 SDH-D, 4 SDH-B, and 1 SDH-C) and all addressed to F-DOPA PET/CT scan. Seven patients had already been operated on for one or more pheochromocytomas and/or paragangliomas and were submitted to F-DOPA PET/CT scan according to clinical, biochemical, or radiological suspicion of recurrence, while 3 were only genetically positive, with no previous symptom/sign of the disease. For all patients, biochemical analysis (plasma and/or urinary catecholamine) and results of high-resolution morphological imaging studies (CT and/or MRI) were available. Histologic/cytologic findings or imaging and biochemical follow-up were taken as gold standard in all cases.
  
  
  RESULTS
  Seven out of 10 patients showed one or more areas of pathological F-DOPA accumulation. PET/CT demonstrated the presence of the disease in 4/6 patients with no increase in catecholamine levels ("biochemically silent"). Positive detection rate was 100% in SDH-D and 40% in "non-SDHD". Analyzing per lesion, F-DOPA PET/CT demonstrated more lesions than anatomical imaging (16 vs. 7) especially in head and neck paragangliomas.
  
  
  CONCLUSIONS
  F-DOPA PET/CT seems to be the more accurate method for staging and restaging patients with SDH-mutations-related paraganglioma syndromes. F-DOPA is particularly useful in detecting head and neck and biochemically silent paragangliomas, and also in apparently healthy mutation-carrying people.
authors.affiliation: From the *Department of Nuclear Medicine, PET/CT Centre, †Medical Physics Unit, "Santa Maria della Misericordia Hospital", Rovigo, Italy; ‡Department of Medicine-DIMED, University of Padova, Padova, Italy; §Familial Cancer Clinic & Oncoendocrinology, Veneto Institute of Oncology, IRCCS, Milan, Italy; and ¶Department of Radiology, University of Southern California, Los Angeles, CA.
authors.forename: Maria Cristina
authors.initials: MC
authors.lastname: Marzola
authors.forename: Sotirios
authors.initials: S
authors.lastname: Chondrogiannis
authors.forename: Gaia
authors.initials: G
authors.lastname: Grassetto
authors.forename: Lucia
authors.initials: L
authors.lastname: Rampin
authors.forename: Anna Margherita
authors.initials: AM
authors.lastname: Maffione
authors.forename: Alice
authors.initials: A
authors.lastname: Ferretti
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Opocher
authors.forename: Francesca
authors.initials: F
authors.lastname: Schiavi
authors.forename: Patrick M
authors.initials: PM
authors.lastname: Colletti
authors.forename: Domenico
authors.initials: D
authors.lastname: Rubello
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000201:enzymology / Q000235:genetics; D000328:Adult; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging; D009154:Mutation; D010673:Pheochromocytoma / Q000175:diagnosis* / Q000201:enzymology / Q000235:genetics; D049268:Positron-Emission Tomography; D012189:Retrospective Studies; D013385:Succinate Dehydrogenase / Q000172:deficiency* / Q000235:genetics; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: C043437:fluorodopa F 18; D004295:Dihydroxyphenylalanine; D013385:Succinate Dehydrogenase