Fig. 5

A 25 year old female with SDHB germline mutation and metastatic PGL. Sites of disease show very high F-18 FDG uptake (left) with relative low Ga-68 DOTATATE uptake (middle) and MIBG (right). The findings indicated lack of suitability for either radionuclide therapy with radiolabelled DOTATATE or MIBG. The patient had a rapidly progressive disease course despite treatment with chemotherapy (combination of cyclophosphamide, vincristine, dacarbazine) and subsequently sunitinib. The lack high SSTR expression is unusual for a SDHB phenotype and was indicative of de-differentiated disease with consequent aggressive phenotype