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The cases
Published in Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young, Paediatric Radiology for MRCPCH and FRCR, 2020
Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young
These are very rare tumours arising from the catecholamine-producing chromaffin cells of the adrenal medulla. (An extra-adrenal paraganglioma is the equivalent arising from sympathetic or parasympathetic paraganglia). They may be benign or malignant.
Hereditary Pheochromocytoma and Paraganglioma Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Pheochromocytoma (pheo, brown; chromo, chromo stain; cytoma, tumor) is a neuroendocrine tumor that arises from chromaffin cells (i.e., brown-black colored cells due to oxidization and polymerization of catecholamines by potassium dichromate) of the adrenal medulla (which contains the largest paraganglia made up of the aggregated cell nuclei of the autonomic nervous system) and accounts for 80% of catecholamine-secreting tumors. Paraganglioma (tumor of the paraganglia) is a neuroendocrine tumor that develops at the paraganglia located outside of the adrenal gland [3].
Miscellaneous
Published in Joseph Kovi, Hung Dinh Duong, Frozen Section In Surgical Pathology: An Atlas, 2019
The extra-adrenal paraganglion system is formed by small nodules and microscopic cell groups associated with the autonomic nervous system. Ultrastructurally, the individual cells contain granules which have an electron-dense core after double fixation with glutaraldehyde and osmium tetroxide. The dense-core granules store catecholamines.315 Both functioning and non-functioning carotid body tumors have been reported. The rare functional carotid body tumors secrete epinephrine or norepinephrine. Microscopically, the tumor has an organoid pattern and resembles the normal carotid body. The neoplastic cells form small nests or islands and are separated by a loose connective tissue stroma which is rich in capillaries. The individual cells are moderately large, cuboid or polygonal, and possess regular, vesicular nuclei. Mitotic activity is absent. The eosinophilic cytoplasm is somewhat granular (Figure 164).
Para-aortic paraganglioma mimicking lymph node metastasis in an ovarian carcinoma: a case report
Published in Journal of Obstetrics and Gynaecology, 2018
Gozde Sahin, Ceyhan Ugurluoglu, Serra Akar, Ayhan Gul, Aysegul Kebapcilar, Cetin Celik
Paragangliomas are rare neuroendocrine tumours that arise from specialised paraganglion cells, which are spread over the entire body from the skull to the pelvic floor. Paragangliomas are known to arise in specific locations, such as the carotid body, jugular foramen, middle ear, aortico-pulmonary region, posterior mediastinum and abdominal para-aortic region, including Zuckerkandl’s body, or the aortic body (Feng et al. 2009). The adrenal medulla has the largest collection of receptor cells, which accounts for the high rate of tumour occurrence at this site. In contrast, extra-adrenal retroperitoneal paragangliomas arise from the symmetrically distributed paraganglia situated close to the aorta and sympathetic chain (Wen et al. 2010).
Genotype-phenotype associations in paragangliomas of the temporal bone in a multi-ethnic cohort
Published in Acta Oto-Laryngologica, 2023
Simon I. Angeli, Juan A. Chiossone K, Stefania Goncalves, Fred F. Telischi
Paragangliomas are neuroendocrine neoplasms that arise from paraganglia tissue. In the temporal bone, paragangliomas are named after their site of origin: glomus tympanicum (GT) from the middle ear paraganglia, glomus jugulare (GJ) from the jugular foramen paraganglia. [1] The clinical presentation of temporal bone paragangliomas (TBPGL) is variable, most of them with an indolent behaviour, but occasionally with aggressive presentations that include local-regional invasiveness and distant metastasis. In consequence, the assessment of the individual risk profile allows personalized therapy to reduce the treatment-induced and tumour-induced morbidity and mortality. [2]
The carotid body and associated tumors: updated review with clinical/surgical significance
Published in British Journal of Neurosurgery, 2019
Nasir Butt, Woong Kee Baek, Stefan Lachkar, Joe Iwanaga, Asma Mian, Christa Blaak, Sameer Shah, Christoph Griessenauer, R. Shane Tubbs, Marios Loukas
Paragangliomas are neuroendocrine neoplasms of the paraganglion system (Figure 2). Carotid body tumors (CBTs), though rare, constitute over 65% of the paragangliomas of the head and neck.12 Von Luschka was the first to describe a carotid body tumor in 1862. In 1880, Reigner was the first to excise a carotid body tumor, which resulted in the patient’s death.13 Scudder reported the first successful removal of a carotid body tumor in the United States in 1903.4,14