Anti-SDHD monoclonal antibody

Background Attention of the otorhinolaryngologist needs to be drawn to the versatile aspects of head and neck paragangliomas (PGLs). Methods. This study is a retrospective, nonrandomized clinical study of all 175 individuals with PGLs treated in our department between 1989 and 2010. A genetic analysis was performed on 86 patients. Results. The 175 patients presented 224 head and neck PGLs as well as 2 thyroid papillary carcinomas. Genetic analysis resulted in 1 patient positive for a von Hippel-Lindau (VHL) gene mutation and 34 for succinate dehydrogenase (SDH) gene mutations (22 SDHD, 7 SDHC, and 5 SDHB), 12 of the latter carrying a novel mutation. Thirty-three patients (18.9%) had multiple PGLs and 11 patients (6.3%) had a malignant paraganglioma. SDH-mutation carriers had multiple tumors in 64.7% and malignant paragangliomas in 20.6%. Conclusions. Multifocal occurrence, potential malignancy, genetic aspects, possible coincidence of thyroid carcinoma, and hormone production have to be considered in patients with head and neck PGLs. (C) 2011 Wiley Periodicals, Inc. Head Neck 34: 632-637, 2012

Purpose To evaluate and compare diagnostic performance of Ga-68-DOTA(0)-Tyr(3)-octreotate (Ga-68-DOTATATE) with F-18-fluoro-2-deoxy-D-glucose (F-18-FDG) positron emission tomography-computed tomography (PET/CT) and anatomic imaging using computed tomography and/or magnetic resonance (CT/MR) imaging in detection of SDHx-related pheochromocytomas and paragangliomas (PPGLs) in pediatric patients. Methods Nine pediatric patients (5:4, girls:boys; 14.6 +/- 2.0 years) with an SDHx-related mutation (SDHB:SDHA:SDHD, n = 7:1:1) were included in this retrospective study. At the time of initial diagnosis, 7/9 patients had metastatic disease. They underwent CT/MR imaging along with PET/CT using Ga-68-DOTATATE (n = 9), F-18-FDG (n = 8), and positron emission tomography-magnetic resonance imaging (PET/MR) using F-18-FDG (n = 1). In this manuscript, F-18-FDG PET/CT refers to both F-18-FDG PET/CT and F-18-FDG PET/MR. The per-lesion, per-region, and per-patient detection rates were compared and calculated for each of the imaging modalities. A composite of all functional and anatomic imaging studies served as the imaging comparator. Results Eight out of nine patients were positive for PPGLs on the imaging studies that demonstrated 107 lesions in 22 anatomic regions on the imaging comparator. The per-lesion detection rates for Ga-68-DOTATATE PET/CT, F-18-FDG PET/CT, and CT/MR imaging were 93.5% (95%CI, 87.0% to 97.3%); 79.4% (95%CI, 70.5% to 86.6%); and 73.8% (95%CI, 64.5% to 81.9%), respectively. The per-lesion detection rate for Ga-68-DOTATATE PET/CT was significantly higher than that of F-18-FDG PET/CT (p = 0.001) or CT/MR imaging (p < 0.001). In all of the anatomic regions except abdomen, the per-lesion detection rates for Ga-68-DOTATATE PET/CT was found to be equal or superior to F-18-FDG PET/CT, and CT/MR imaging. The per-region detection rate was 100% (95%CI, 84.6% to 100%) for Ga-68-DOTATATE PET/CT and 90.9% (95%CI, 70.8% to 98.9%) for both F-18-FDG PET/CT and CT/MR imaging. The per-patient detection rates for Ga-68-DOTATATE PET/CT, (18)FDG PET/CT, and CT/MR imaging were all 100% (95%CI, 63.1% to 100%). Conclusion Our preliminary study demonstrates the superiority of Ga-68-DOTATATE PET/CT in localization of SDHx-related PPGLs in pediatric population compared to F-18-FDG PET/CT and CT/MR imaging with the exception of abdominal (excluding adrenal and liver) lesions, and suggests that it might be considered as a first-line imaging modality in pediatric patients with SDHx-related PPGLs.