Comparison of 68Ga-Dotatate PET/CT and 18F-FDOPA PET/CT for the diagnosis of pancreatic neuroendocrine tumors in a MEN1 patient

Jullien, Monique; Reichert, Thibault; D’Anella, Pascal; Castinetti, Frédéric; Barlier, Anne; Brue, Thierry; Taïeb, David; Cuny, Thomas

doi:10.1016/j.ando.2019.11.001

Cited by 3 publications

(1 citation statement)

References 19 publications

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“…Consensus for optimizing personalized diagnostic and follow-up strategies is still needed, but MRI, due to its availability and noninvasive nature is considered the modality of choice [ 7 , 59 ]. The recent advances in somatostatin receptor (SSTR) positron emission tomography (PET)-CT (SSTR-PET-CT) in sporadic PanNETs have also been applied in MEN1 patients [ 60 ]. A study evaluating the role of SSTR-PET-CT in MEN1 patients showed that it can detect up to three times more PanNETs than conventional imaging [ 61 ].…”

Section: Pancreatic Nets In Men1mentioning

confidence: 99%

Hereditary Syndromes Associated with Pancreatic and Lung Neuroendocrine Tumors

Papadopoulou-Marketou,

Tsoli,

Chatzellis

et al. 2024

Cancers

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Pancreatic neuroendocrine tumors (PanNETs) and lung NETs (LNETs) represent a rare but clinically significant subgroup of neoplasms. While the majority is sporadic, approximately 17% of PanNETs and a subset of LNETs develop in the context of monogenic familial tumor syndromes, especially multiple endocrine neoplasia type 1 (MEN1) syndrome. Other inherited syndromes associated with PanNETs include MEN4, von Hippel–Lindau (VHL) syndrome, neurofibromatosis type 1 (NF1), and tuberous sclerosis complex (TSC). These syndromes are highly penetrant and their clinical manifestations may vary even among members of the same family. They are attributed to genetic mutations involving key molecular pathways regulating cell growth, differentiation, and angiogenesis. Pancreatic NETs in hereditary syndromes are often multiple, develop at a younger age compared to sporadic tumors, and are associated with endocrine and nonendocrine tumors derived from multiple organs. Lung NETs are not as common as PanNETs and are mostly encountered in MEN1 syndrome and include typical and atypical lung carcinoids. Early detection of PanNETs and LNETs related to inherited syndromes is crucial, and specific follow-up protocols need to be employed to optimize diagnosis and management. Genetic screening is recommended in childhood, and diagnostic screening starts often in adolescence, even in asymptomatic mutation carriers. Optimal management and therapeutic decisions should be made in the context of a multidisciplinary team in specialized centers, whereas specific biomarkers aiming to identify patients denoted to follow a more aggressive course need to be developed.

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