Review Article

A mini review of paragangliomas with presentation of two cases

E. Kader
South African Journal of Radiology | Vol 3, No 4 | a1562 | DOI: https://doi.org/10.4102/sajr.v3i4.1562 | © 2018 E. Kader | This work is licensed under CC Attribution 4.0
Submitted: 10 August 2018 | Published: 30 November 1998

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E. Kader, Department of Radiology, University of Cape Town, South Africa

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Abstract

Paragangliomas are rare tumours of the paraganglion system, which may, in approximately 80% of cases, be hormonally active with potentially lethal consequences. In this mini review, the classification, pathology, pathophysiology and clinical management are described. An attempt is also made to evaluate the relative advantages and disadvantages of the most commonly used diagnostic imaging modalities, viz. Magnetic Resonance Imaging (MRI), Computed Tomography (CT) and radionuclide scintigraphy. Paragangliomas are divided into four anatomical classes:
1. Paragangliomas arising from the adrenal medulla
2. Aortico-sympathetic paragangliomas
3. Parasympathetic and visceroautonomic paragangliomas
4. Paragangliomas not otherwise specified. They occur sporadically or in families, and have recognised associations with other diseases. The majority are benign. Ninety percent of tumours are adrenal and 1O% are extra-adrenal. Patients commonly present with hypertension or paroxysmal crises and the laboratory diagnosis hinges on the demonstration of elevated catecholamine levels. Initial management involves stabilisation with alpha-blockade followed by preoperative tumour localisation.
MRI has similar accuracy to CT in diagnosis of adrenal tumours. MRI surpasses metaiodobenzylguanidine (MIBG) scanning in detecting adrenal disease and non-functional paragangliomas. MIBG has greater sensitivity and specificity than MRI in the detection of extra-adrenal tumours, although both are superior to CT. MRI is currently the investigation of choice, with MIBG being used in cases of disease recurrence, metastases or equivocal MRI. Surgical cure is possible in up to 85% of cases. The recurrence rate is between 5 and 10%, with a 5 year survival rate of 80 to 95%.

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