| 11. |
- Lenders, Jacques W. M., et al.
(författare)
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Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma : a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension
- 2020
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Ingår i: Journal of Hypertension. - 0263-6352 .- 1473-5598. ; 38:8, s. 1443-1456
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Tidskriftsartikel (refereegranskat)abstract
- Phaeochromocytoma and paraganglioma (PPGL) are chromaffin cell tumours that require timely diagnosis because of their potentially serious cardiovascular and sometimes life- threatening sequelae. Tremendous progress in biochemical testing, imaging, genetics and pathophysiological understanding of the tumours has far-reaching implications for physicians dealing with hypertension and more importantly affected patients. Because hypertension is a classical clinical clue for PPGL, physicians involved in hypertension care are those who are often the first to consider this diagnosis. However, there have been profound changes in how PPGLs are discovered; this is often now based on incidental findings of adrenal or other masses during imaging and increasingly during surveillance based on rapidly emerging new hereditary causes of PPGL. We therefore address the relevant genetic causes of PPGLs and outline how genetic testing can be incorporated within clinical care. In addition to conventional imaging (computed tomography, MRI), new functional imaging approaches are evaluated. The novel knowledge of genotype-phenotype relationships, linking distinct genetic causes of disease to clinical behaviour and biochemical phenotype, provides the rationale for patient-tailored strategies for diagnosis, follow-up and surveillance. Most appropriate preoperative evaluation and preparation of patients are reviewed, as is minimally invasive surgery. Finally, we discuss risk factors for developing metastatic disease and how they may facilitate personalised follow-up. Experts from the European Society of Hypertension have prepared this position document that summarizes the current knowledge in epidemiology, genetics, diagnosis, treatment and surveillance of PPGL.
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| 12. |
- Sundin, Anders, 1954-, et al.
(författare)
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A Clinical Challenge : Endocrine and Imaging Investigations of Adrenal Masses
- 2021
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Ingår i: Journal of Nuclear Medicine. - : Society of Nuclear Medicine. - 0161-5505 .- 1535-5667 .- 2159-662X. ; 62, s. 26S-33S
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Tidskriftsartikel (refereegranskat)abstract
- Incidentalomas are reported in 3%-4% of patients who undergo abdominal anatomic imaging, making adrenal mass evaluation a common occurrence. An adrenal mass can be caused by a variety of pathologies, such as benign cortical and medullary tumors, malignant tumors (primary or secondary), cysts, hyperplasia, hemorrhage, or more rarely infection/inflammation processes. Functioning tumors usually have increased hormonal production but they are less common. Regardless of their functional status, some tumors have the potential to behave aggressively. Anatomic and functional imaging together with biologic evaluation dplay a vital role in adrenal pathology subtyping. Most patients are initially evaluated by CT or MRI, which allows for tumor characterization (to a certain extent) and can rule out malignant behavior based on the absence of tumor growth during longitudinal follow-up. In the remaining patients for whom CT or MRI fail to characterize the pathogenesis of adrenal tumors, the use of specialized molecular imaging techniques should be performed after hormonal screening. This review emphasizes well-established and emerging nuclear medicine imaging modalities and describes their use across various clinical scenarios.
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