Abstract

ObjectiveParaganglioma (PGL) and pheochromocytoma (PCC) are neuroendocrine tumors most often benign associated with hereditary syndromes in about 30% of cases. This study aims to define the impact of tumor location and patient genotype on the clinical value of 18F-FDopa PET by assessing in detail the false negative occurrences. Patients and methodsA retrospective study was conducted on a cohort of 53 cases with non-metastatic sporadic or inherited PGL/PCC (SDHx or VHL related syndromes), investigated with 18F-FDopa PET. ResultsOverall detection sensitivity of 18F-FDopa PET was 88%. Seventy-three lesions were found using this technique, including 49 head-and-neck PGL (HNP), two thoracic PGL (1 sympathetic and 1 parasympathetic), eight extra-adrenal retroperitoneal PGL and 15 PCC. The 10 missed lesions were seven extra-adrenal abdominal PGL (2 SDHB, 2 SDHD), two HNP (1 sporadic, 1 SDHD) and one PCC (1 SDHD). Conclusion18F-FDopa PET is a sensitive technique for the evaluation of non-metastatic head and neck and adrenal PGLs. Exploration of extra-adrenal retroperitoneal PGL associated with SDHB or SDHD syndrome is the main limitation of this technique, encouraging the use of alternative functional imaging modalities like FDG-PET. Negativity of 18F-FDopa PET in the initial assessment of a PGL should prompt to search for a SDHx mutation.

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