ABSTRACT
Pheochromocytomas (PCCs) are rare neuroendocrine tumors derived from the chromaffin cells of the adrenal medulla. When these tumors have an extra-adrenal location, they are called paragangliomas (PGLs) and arise from sympathetic and parasympathetic ganglia, particularly of the para-aortic location. Up to 25% of PCCs/PGLs are associated with inherited genetic disorders. The majority of PCCs/PGLs exhibit indolent behavior. However, according to their affiliation to molecular clusters based on underlying genetic aberrations, their tumorigenesis, location, clinical symptomatology, and potential to metastasize are heterogenous. Thus, PCCs/PGLs are often associated with diagnostic difficulties. In recent years, extensive research revealed a broad genetic background and multiple signaling pathways leading to tumor development. Along with this, the diagnostic and therapeutic options were also expanded. In this review, we focus on the current knowledge and recent advancements in the diagnosis and treatment of PCCs/PGLs with respect to the underlying gene alterations while also discussing future perspectives in this field.
Subject(s)
Adrenal Gland Neoplasms , Paraganglioma , Pheochromocytoma , Humans , Pheochromocytoma/diagnosis , Pheochromocytoma/genetics , Pheochromocytoma/therapy , Paraganglioma/diagnosis , Paraganglioma/genetics , Paraganglioma/therapy , Carcinogenesis , Cell Transformation, Neoplastic , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/therapyABSTRACT
Review summarizes state of the art of contrast-enhanced ultrasonography (CEUS), pinpoints technical basics and also economical comparison with other imaging methods. Most often indications of CEUS in different anatomical regions with differential diagnosis are discussed.