Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options. 2023

Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
Department of Hematology, Oncology and Internal Medicine, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic.

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.

UI MeSH Term Description Entries
D010235 Paraganglioma A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion, such as the carotid body, or medulla of the adrenal gland (usually called a chromaffinoma or pheochromocytoma). It is more common in women than in men. (Stedman, 25th ed; from Segen, Dictionary of Modern Medicine, 1992) Paraganglioma, Gangliocytic,Paragangliomata,Gangliocytic Paraganglioma,Gangliocytic Paragangliomas,Paragangliomas,Paragangliomatas
D010673 Pheochromocytoma A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the ADRENAL MEDULLA or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of EPINEPHRINE and NOREPINEPHRINE, is HYPERTENSION, which may be persistent or intermittent. During severe attacks, there may be HEADACHE; SWEATING, palpitation, apprehension, TREMOR; PALLOR or FLUSHING of the face, NAUSEA and VOMITING, pain in the CHEST and ABDOMEN, and paresthesias of the extremities. The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1298) Pheochromocytoma, Extra-Adrenal,Extra-Adrenal Pheochromocytoma,Extra-Adrenal Pheochromocytomas,Pheochromocytoma, Extra Adrenal,Pheochromocytomas,Pheochromocytomas, Extra-Adrenal
D002471 Cell Transformation, Neoplastic Cell changes manifested by escape from control mechanisms, increased growth potential, alterations in the cell surface, karyotypic abnormalities, morphological and biochemical deviations from the norm, and other attributes conferring the ability to invade, metastasize, and kill. Neoplastic Transformation, Cell,Neoplastic Cell Transformation,Transformation, Neoplastic Cell,Tumorigenic Transformation,Cell Neoplastic Transformation,Cell Neoplastic Transformations,Cell Transformations, Neoplastic,Neoplastic Cell Transformations,Neoplastic Transformations, Cell,Transformation, Cell Neoplastic,Transformation, Tumorigenic,Transformations, Cell Neoplastic,Transformations, Neoplastic Cell,Transformations, Tumorigenic,Tumorigenic Transformations
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000310 Adrenal Gland Neoplasms Tumors or cancer of the ADRENAL GLANDS. Adrenal Cancer,Adrenal Gland Cancer,Adrenal Neoplasm,Cancer of the Adrenal Gland,Neoplasms, Adrenal Gland,Adrenal Cancers,Adrenal Gland Cancers,Adrenal Gland Neoplasm,Adrenal Neoplasms,Cancer, Adrenal,Cancer, Adrenal Gland,Cancers, Adrenal,Cancers, Adrenal Gland,Neoplasm, Adrenal,Neoplasm, Adrenal Gland,Neoplasms, Adrenal
D063646 Carcinogenesis The origin, production or development of cancer through genotypic and phenotypic changes which upset the normal balance between cell proliferation and cell death. Carcinogenesis generally requires a constellation of steps, which may occur quickly or over a period of many years. Tumorigenesis,Oncogenesis,Carcinogeneses,Oncogeneses,Tumorigeneses

Related Publications

Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
August 2006, Annals of the New York Academy of Sciences,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
September 2020, Archivos espanoles de urologia,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
April 2010, Nature reviews. Endocrinology,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
January 2001, Endocrine pathology,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
June 2009, Presse medicale (Paris, France : 1983),
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
March 2020, Medwave,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
May 2008, Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
January 1982, Indian journal of cancer,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
February 2022, Journal of the Endocrine Society,
Michal Eid, and Jakub Foukal, and Dana Sochorová, and Štěpán Tuček, and Karel Starý, and Zdeněk Kala, and Jiří Mayer, and Radim Němeček, and Jan Trna, and Lumír Kunovský
December 2023, Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists,
Copied contents to your clipboard!