@article {Rischke1352, author = {H. Christian Rischke and Matthias R. Benz and Damian Wild and Michael Mix and Rebecca A. Dumont and Dean Campbell and Jochen Seufert and Thorsten Wiech and Jochen R{\"o}ssler and Wolfgang A. Weber and Hartmut P.H. Neumann}, title = {Correlation of the Genotype of Paragangliomas and Pheochromocytomas with Their Metabolic Phenotype on 3,4-Dihydroxy-6-18F-Fluoro-l-Phenylalanin PET}, volume = {53}, number = {9}, pages = {1352--1358}, year = {2012}, doi = {10.2967/jnumed.111.101303}, publisher = {Society of Nuclear Medicine}, abstract = {Paragangliomas and pheochromocytomas are genetically heterogeneous diseases. The purpose of this study was to determine the sensitivity and specificity of PET with 3,4-dihydroxy-6-18F-fluoro-L-phenylalanin (18F-DOPA) for the detection and staging of pheochromocytomas/paragangliomas. Furthermore, we assessed whether the genotypes of pheochromocytomas and paragangliomas correlate with the uptake of 18F-DOPA. Methods: We retrospectively analyzed 101 consecutive patients who underwent 18F-DOPA PET or 18F-DOPA PET/CT for known or suspected pheochromocytomas or paragangliomas. Maximum 18F-DOPA tumor uptake was quantified relative to uptake in the liver. Results: Histopathology, cross-sectional imaging, and follow-up indicated the presence of paragangliomas and pheochromocytomas in 68 patients and the absence of a tumor in 33 patients. The average 18F-DOPA uptake by paragangliomas and pheochromocytomas, expressed as a tumor-to-liver ratio, was 5.9 {\textpm} 5.2. There was no significant difference in uptake among patients with von Hippel Lindau syndrome (VHL; n = 19), succinate dehydrogenase B{\textendash}D mutation (n = 21), neurofibromatosis type 1 (n = 1), RET (n = 1), no germline mutation (n = 20), or unknown mutation status (n = 6) (P = 0.84). All 8 patients with an SDHD mutation were true-positive on 18F-DOPA PET. There were 2 cases of false-negative results each in the group with SDHB (2/12) and VHL mutations (2/19) and 1 false-negative result in the subgroup of patients with unknown mutation status (1/6). Overall, 18F-DOPA PET yielded a sensitivity of 93\% and a specificity of 88\% for the detection of paragangliomas and pheochromocytomas on a patient basis (positive and negative predictive value, 94\% and 85\%, respectively). Conclusion: 18F-DOPA PET is a sensitive and specific imaging modality for the detection and staging of pheochromocytomas and paragangliomas in different genotypes, including VHL-, SDHB-, and SDHD-mutation carriers, and in patients with no germline mutation.}, issn = {0161-5505}, URL = {https://jnm.snmjournals.org/content/53/9/1352}, eprint = {https://jnm.snmjournals.org/content/53/9/1352.full.pdf}, journal = {Journal of Nuclear Medicine} }