18F-fluorodeoxyglucose positron emission tomography from a 14-year-old adolescent with metastatic paraganglioma associated with a germline mutation in succinate dehydrogenase subunit B

FDG-PET scan in a 14-year-old adolescent with metastatic paraganglioma due to a germline SDHB gene mutation (frame shift mutation in exon 7). At age 10 years, she was diagnosed with hypertension. A right hydronephrotic dysplastic nonfunctioning kidney was identified and an arteriogram showed minimal arterial supply to the right kidney; she was treated with a right nephrectomy. However, hypertension persisted and she developed paroxysms of marked hypertension, headaches, and sweating. These symptoms led to the diagnosis of a catecholamine-secreting tumor at 14 years of age. The FDG-PET total body coronal image shows the primary paraganglioma (thick arrow) lateral to the inferior vena cava at the level of L2-3. In addition, metastatic right adnexal disease (arrow) is seen. There is also metastatic disease adjacent to the right external iliac artery, posterior pelvis bilaterally, inferior right lobe of the liver, and boney metastases in T10, T11 and in the right iliac crest. At laparotomy a 5 × 3 × 2.8 cm right lower abdominal paraganglioma was resected; it had encompassed the right ureter and the reason for the presentation of hydronephrosis at age 10. In addition, the right ovary and 6 pelvic lymph nodes were removed due to metastatic disease. The liver metastasis was also resected.