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• W2080890406 abstract "Medullary thyroid cancer (MTC) is a rare, neuroendocrine tumour (NET) of parafollicular C cells comprising 5–8% of all thyroid cancers with variable biological behaviour and prognosis [1]. Overall mean survival in MTC is 75–85% at 10 years [1–3]. Management of both hereditary (20–30%) and sporadic MTC is challenging due to early cervical lymph node metastases occurring in 50% of patients and distant metastases to the liver, lung and bones being found in 10–20% at diagnosis [1, 3]. MTC secretes several neuroendocrine peptides, of which calcitonin (Ct) and less-specific carcinoembryonic antigen (CEA) are useful tumour markers for diagnosis, surveillance and prognosis. Yet despite the high sensitivity of an elevated serum Ct value as a predictor of disease presence, MTC is an elusive and notoriously problematic tumour to image [1, 3]. Anatomy-based imaging with CT and MRI has exhibited limited sensitivity due to the frequent small size of the metastatic deposits. Although a variety of specific and nonspecific scintigraphic techniques such as Tl, Tc-sestamibi, Tc-tetrofosmin, I/I-metaiodobenzylguanidine, Tc-(V)dimercaptosuccinic acid (DMSA), and Inoctreotide have been used to image MTC, none has emerged as the imaging technique of choice [4, 5]. This raises the question as to why we have been unable to successfully image MTC. The rarity of MTC implies that no single centre can collect a sufficient number of cases, and to date there have been no large, cooperative studies to establish optimal imaging protocols. It has become apparent that MTCs are a heterogeneous group of neoplasms, and the biological behaviour of one MTC may differ significantly from that of another. Imaging approaches that have proven successful, for example using F-fluorodeoxyglucose (FDG) PET/CT in lymphoma and solid cancers and In-octreotide for somatostatin receptor scintigraphy (SRS) in carcinoids, may be too simplistic an imaging approach for MTC, which often appears intermediate in biological behaviour between more aggressive neoplasms characterized by high Ki-67 indices, and hormonally active NETs in which FDG PET has limited value. Genetic testing provides a valuable insight into the phenotypic expression of MTC. Genetic mapping has confirmed the central role of germline mutations of the rearranged during transfection (RET) protooncogene in the pathogenesis of inherited MTC and has shown that somatic RET mutations occur in 50–60% of patients with sporadic MTC, and there have been advances in our understanding of how activated RET tyrosine kinase signalling pathways affect tumour growth and angiogenesis [1, 6–8]. Insights from genetic testing allow informed management decisions. As an example, the dilemma of the timing of prophylactic thyroidectomy in multiple endocrine neoplasia type 2 (MEN2), an autosomal D. Rubello (*) :M. C. Marzola : S. Chondrogiannis Department of Nuclear Medicine, Medical Physics, Radiology, Santa Maria della Misericordia Hospital, Via Tre Martiri 140, 45100 Rovigo, Italy e-mail: domenico.rubello@libero.it" @default.
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• W2080890406 date "2012-01-05" @default.
• W2080890406 modified "2023-09-25" @default.
• W2080890406 title "Evolving paradigms for successful molecular imaging of medullary thyroid carcinoma" @default.
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• W2080890406 doi "https://doi.org/10.1007/s00259-011-2035-2" @default.
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