Imaging plays a critical role in the management of pheochromocytomas and paragangliomas and often guides treatment. The discovery of susceptibility genes associated with these tumors has led to better understanding of clinical and imaging phenotypes. Functional imaging is of prime importance because of its sensitivity and specificity in subtypes of pheochromocytoma and paraganglioma. Several radiopharmaceuticals have been developed to target specific receptors and metabolic processes seen in pheochromocytomas and paragangliomas, including I/I-metaiodobenzylguanidine, 6-F-fluoro-l-3,4-dihydroxyphenylalanine, F-FDG, and Ga-DOTA-somatostatin analogs. Two of these have consequently been adapted for therapy. This educational review focuses on the current imaging approaches used in pheochromocytomas and paragangliomas, which vary among clinical and genotypic presentations.
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http://dx.doi.org/10.2967/jnumed.120.259689 | DOI Listing |
Exp Ther Med
February 2025
Molecular Pathology, Azienda USL-IRCCS di Reggio Emilia, I-42123 Reggio Emilia, Italy.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with an annual incidence of ~2 cases per million worldwide. The hereditary form is more likely to present in younger patients. To date, PPGL is considered a complex pathology that is difficult to diagnose.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
January 2025
Metabolic Diseases Branch, Bldg. 10/Rm 8C-101, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD 20892.
Establishing genotype-phenotype correlations in disorders of hereditary endocrine neoplasia is important for clinical screening, genetic counseling, prognostication, surveillance, and surgical strategy, and may also provide clues about disease pathogenesis. Important genotype-phenotype correlations are recognized, for example, in pheochromocytoma/paraganglioma and multiple endocrine neoplasia type 2A. The presence of such correlations has been less clear in other familial endocrine disorders associated with primary hyperparathyroidism including multiple endocrine neoplasia type 1 (MEN1), and the hyperparathyroidism-jaw tumor syndrome (HPT-JT).
View Article and Find Full Text PDFAndes Pediatr
October 2024
Escuela de Medicina, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Unlabelled: Adrenal tumors in children are frequently neoplastic and malignant, and surgical resection is the first management option. Minimally invasive surgery (MIS) has proven to be a safe management alternative and is suggested as a preferred alternative approach.
Objective: To report the surgical outcomes of patients with adrenal tumors treated by MIS.
Cureus
December 2024
Internal Medicine, Texas Tech University Health Sciences Center, El Paso, USA.
Carotid body tumors (CBTs), rare neuroendocrine neoplasms near the carotid bifurcation, are mostly asymptomatic but may cause discomfort and autonomic dysfunction. Computed tomography angiography (CTA) is used for diagnosis, eliminating the need for a biopsy to avoid the risk of hemorrhage. Surgical excision is the preferred treatment, while radiotherapy is an option when surgery is impractical.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
January 2025
Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH), CH-8091 Zurich, Switzerland.
Context: Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors that frequently produce catecholamines. Catecholamine-induced cardiometabolic complications substantially contribute to increased morbidity and mortality in PPGL patients prior to surgical resection.
Objective: To determine whether markers of elevated cardiometabolic risk persist in patients following PPGL resection.
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