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Paraganglioma, Mediastinal

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Pathology of the Pleura and Mediastinum

Part of the book series: Encyclopedia of Pathology ((EP))

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Mediastinal paragangliomas are rare neuroendocrine neoplasm derived from the autonomic nervous center (Michalowska et al. 2016). They account for approximately 2% of all paragangliomas. They may rise from the paraganglia in the region of the cardiac plexus (anterior or middle mediastinum) and are called aorticopulmonary pargangliomas or they may arise along the aorticosympathetic chain in the costovertebral sulcus (posterior mediastinum) (Qedra 2009). Aorticopulmonary paragangliomas occur in patients over the age of 40 and are usually asymptomatic. Though they are asymptomatic/nonfunctioning, they may occasionally cause chest pain. These paragangliomas are frequently found incidentally on an imaging study. Aorticosympathetic paraganglioma happen in younger patients with an average age of 29 years. Approximately 50% of these patients will have symptoms from because of functionality of the tumor (Takashima et al. 2015).

Clinically, mediastinal paragangliomas may be incidentally found or...

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References and Further Reading

  • Baysal, B. E., et al. (2000). Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma. Science, 287(5454), 848–851.

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  • Kalhor, N., & Moran, C. A. (2010). Mediastinal neuroendocrine tumours. Diagnostic Pathology, 16(5), 237–242.

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  • Michalowska, I., et al. (2016). Mediastinal paragangliomas related to SDHx gene mutations. Kardiochirurgia i Torakochirurgia Polska, 13(3), 276–282.

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  • Qedra, N., et al. (2009). Aorticopulmonary paraganglioma with severe obstruction of the pulmonary artery: Successful combined treatment by stenting and surgery. The Annals of Thoracic Surgery, 87(4), 1284–1286.

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  • Takashima, Y., et al. (2015). Mediastinal paraganglioma. Japanese Journal of Radiology, 33(7), 433–436.

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  • Wald, O., Shapira, O. M., Murar, A., & Izhar, U. (2010). Paraganglioma of the mediastinum: Challenges in diagnosis and surgical management. Journal of Cardiothoracic Surgery, 31(5), 19.

    Article  Google Scholar 

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Correspondence to Merce Jorda .

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Sanders, R., Jorda, M. (2018). Paraganglioma, Mediastinal. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_3264

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  • DOI: https://doi.org/10.1007/978-3-319-66796-6_3264

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-66795-9

  • Online ISBN: 978-3-319-66796-6

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