title: Role of positron emission tomography and bone scintigraphy in the evaluation of bone involvement in metastatic pheochromocytoma and paraganglioma: specific implications for succinate dehydrogenase enzyme subunit B gene mutations.
abstract: We performed a retrospective analysis of 71 subjects with metastatic pheochromocytoma and paraganglioma (30 subjects with mutation of succinate dehydrogenase enzyme subunit B (SDHB) gene and 41 subjects without SDHB mutation). Sixty-nine percent presented with bone metastases (SDHB +/-: 77% vs 63%), 39% with liver metastases (SDHB +/-: 27% vs 47%), and 32% with lung metastases (SDHB +/-: 37% vs 29%). The most common sites of bone involvement were thoracic spine (80%; SDHB+/-: 83% vs 77%), lumbar spine (78%; SDHB +/-: 78% vs 75%), and pelvic and sacral bones (78%; SDHB +/-: 91% vs 65%, P=0.04). Subjects with SDHB mutation also showed significantly higher involvement of long bones (SDHB +/-: 78% vs 30%, P=0.007) than those without the mutation. The best overall sensitivity in detecting bone metastases demonstrated positron emission tomography (PET) with 6-[(18)F]-fluorodopamine ([(18)F]-FDA; 90%), followed by bone scintigraphy (82%), computed tomography or magnetic resonance imaging (CT/MRI; 78%), 2-[(18)F]-fluoro-2-deoxy-d-glucose ([(18)F]-FDG) PET (76%), and scintigraphy with [(123/131)I]-metaiodobenzylguanidine (71%). In subjects with SDHB mutation, imaging modalities with best sensitivities for detecting bone metastases were CT/MRI (96%), bone scintigraphy (95%), and [(18)F]-FDG PET (92%). In subjects without SDHB mutations, the modality with the best sensitivity for bone metastases was [(18)F]-FDA PET (100%). In conclusion, bone scintigraphy should be used in the staging of patients with malignant pheochromocytoma and paraganglioma, particularly in patients with SDHB mutations. As for PET imaging, [(18)F]-FDG PET is highly recommended in SDHB mutation patients, whereas [(18)F]-FDA PET is recommended in patients without the mutation.
authors.affiliation: Reproductive Biology and Medicine Branch, NICHD, NINDS, NIH, Bethesda, MD 20892, USA.
authors.forename: Tomás
authors.initials: T
authors.lastname: Zelinka
authors.forename: Henri J L M
authors.initials: HJ
authors.lastname: Timmers
authors.forename: Anna
authors.initials: A
authors.lastname: Kozupa
authors.forename: Clara C
authors.initials: CC
authors.lastname: Chen
authors.forename: Jorge A
authors.initials: JA
authors.lastname: Carrasquillo
authors.forename: James C
authors.initials: JC
authors.lastname: Reynolds
authors.forename: Alexander
authors.initials: A
authors.lastname: Ling
authors.forename: Graeme
authors.initials: G
authors.lastname: Eisenhofer
authors.forename: Ivica
authors.initials: I
authors.lastname: Lazúrová
authors.forename: Karen T
authors.initials: KT
authors.lastname: Adams
authors.forename: Millie A
authors.initials: MA
authors.lastname: Whatley
authors.forename: Jirí
authors.initials: J
authors.lastname: Widimsky
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
mesh_terms: D019797:3-Iodobenzylguanidine; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000000981:diagnostic imaging* / Q000235:genetics / Q000473:pathology; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000000981:diagnostic imaging* / Q000235:genetics / Q000556:secondary; D002648:Child; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D007457:Iodine Radioisotopes; D007506:Iron-Sulfur Proteins / Q000235:genetics*; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000556:secondary; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000556:secondary; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D010235:Paraganglioma / Q000000981:diagnostic imaging* / Q000235:genetics / Q000473:pathology; D010673:Pheochromocytoma / Q000000981:diagnostic imaging* / Q000235:genetics / Q000473:pathology; D049268:Positron-Emission Tomography; D011379:Prognosis; D011877:Radionuclide Imaging; D012189:Retrospective Studies; D013385:Succinate Dehydrogenase / Q000235:genetics*; D014055:Tomography, Emission-Computed
chemical_list: D007457:Iodine Radioisotopes; D007506:Iron-Sulfur Proteins; D019788:Fluorodeoxyglucose F18; D019797:3-Iodobenzylguanidine; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase