TY - JOUR
T1 - Comparison of clinical and imaging features in succinate dehydrogenase-positive versus sporadic paragangliomas
AU - Venkatesan, Aradhana M.
AU - Trivedi, Hari
AU - Adams, Karen T.
AU - Kebebew, Electron
AU - Pacak, Karel
AU - Hughes, Marybeth S.
N1 - Funding Information:
This work was supported in part by the NIH Center for Interventional Oncology and by the NIH Intramural Research Program .
PY - 2011/12
Y1 - 2011/12
N2 - Background: Limited data exist concerning the clinical and imaging features that distinguish sporadic from familial paragangliomas (PGLs). Methods: Clinical, genetic (succinate dehydrogenase [SDHB] vs no SDHx), and imaging (computed tomography [CT], magnetic resonance imaging, 18F-fluoro- deoxy-glucose positron emission tomography [ 18F-FDG-PET]) features obtained during a decade in 124 PGL patients were studied. Data were analyzed by Fisher's exact test or Wilcoxon rank-sum test. Results: Mean age at diagnosis was younger in the SDHB-positive (SDHB+) group compared with the sporadic (no SDHx) group (28 vs 39 years, respectively, P <.001). Rate of supradiaphragmatic neoplasms were greater in the SDHB+ group (16.7 vs 4.7%, P =.11). Metastasis rates were greater in the SDHB+ group (78.9 vs 48.3%, P <.001), as was the existence of metastases or multiple PGLs at presentation (38.5 vs 16.7%, P <.05). Tumor volumes >250 mL were exclusively observed in SDHB+ patients (P <.05). On CT, SDHB+ tumors were more enhanced (P <.05). On 18F-FDG-PET, SDHB+ tumors' had greater mean standard uptake values (12.3 vs 8.0, P <.05). Conclusion: Clinically young age, large tumor volume, greater rate of metastatic and multifocal PGLs, greater SUV values on 18F-FDG-PET, and increased CT enhancement are observed in SDHB+ PGLs. These findings may warrant genetic screening. Because SDHB+ patients demonstrate more supradiaphragmatic lesions, whole-body imaging may be of particular value in these patients.
AB - Background: Limited data exist concerning the clinical and imaging features that distinguish sporadic from familial paragangliomas (PGLs). Methods: Clinical, genetic (succinate dehydrogenase [SDHB] vs no SDHx), and imaging (computed tomography [CT], magnetic resonance imaging, 18F-fluoro- deoxy-glucose positron emission tomography [ 18F-FDG-PET]) features obtained during a decade in 124 PGL patients were studied. Data were analyzed by Fisher's exact test or Wilcoxon rank-sum test. Results: Mean age at diagnosis was younger in the SDHB-positive (SDHB+) group compared with the sporadic (no SDHx) group (28 vs 39 years, respectively, P <.001). Rate of supradiaphragmatic neoplasms were greater in the SDHB+ group (16.7 vs 4.7%, P =.11). Metastasis rates were greater in the SDHB+ group (78.9 vs 48.3%, P <.001), as was the existence of metastases or multiple PGLs at presentation (38.5 vs 16.7%, P <.05). Tumor volumes >250 mL were exclusively observed in SDHB+ patients (P <.05). On CT, SDHB+ tumors were more enhanced (P <.05). On 18F-FDG-PET, SDHB+ tumors' had greater mean standard uptake values (12.3 vs 8.0, P <.05). Conclusion: Clinically young age, large tumor volume, greater rate of metastatic and multifocal PGLs, greater SUV values on 18F-FDG-PET, and increased CT enhancement are observed in SDHB+ PGLs. These findings may warrant genetic screening. Because SDHB+ patients demonstrate more supradiaphragmatic lesions, whole-body imaging may be of particular value in these patients.
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U2 - 10.1016/j.surg.2011.09.026
DO - 10.1016/j.surg.2011.09.026
M3 - Article
C2 - 22136839
AN - SCOPUS:82755186198
SN - 0039-6060
VL - 150
SP - 1186
EP - 1193
JO - Surgery
JF - Surgery
IS - 6
ER -