Fact-sheet: Paraganglioma


Updated on 09/17/2021 at 12:16 PM

Note : 0/10
Share

View all RADEOS cases associated with this fact-sheet

Definition

Rare neuroendocrine tumors of chromaffin cells arising from extra-adrenal chromaffin embryonic remnants or extra-adrenal paraganglionic cells, accounting together with Pheochromocytoma (adrenal origin) for less than 1 in 1000 cases of hypertension. Malignant in 10% of cases. Incidence: poorly known, estimated at approximately 1/30,000. Genetics: 70% sporadic, 30% hereditary (MEN II, NF2, VHL, Sturge-Weber, tuberous sclerosis). Genetic forms present earlier, are often bilateral or even multifocal, and are more prone to recurrence and/or malignancy. Location: defines TWO TYPES - Head and neck Paragangliomas: arising from the parasympathetic system, cervical and skull base location. Usually nonfunctional. Classic forms at the carotid body, tympanic glomus, and jugular glomus. - Thoracoabdominopelvic Paragangliomas: arising from the sympathetic system, 90% infradiaphragmatic along the para-aortic ganglion chains, at the organ of Zuckerkandl, the abdominal and thoracic pre- and paravertebral ganglion chains, the pelvis, ovaries, testes, vagina, prostate, urethra, bladder, and liver

Clinical features

Thoracoabdominopelvic Paraganglioma: most often secreting. Hypertension: paroxysmal > sustained. Malaise, headaches, tachycardia, sweating. Weight loss, diabetes. Sometimes associated with renal artery stenosis. Cervical or skull base Paraganglioma: usually nonsecreting; cervical mass, sometimes pulsatile, with tinnitus, hearing loss, dysphonia

Laboratory findings

Secretion of catecholamines and/or their metabolites.
Plasma metanephrine assay: if negative, rules out a symptomatic secreting Pheochromocytoma.
Nonsecreting tumors are rare

Ultrasound

Ultrasound: soft-tissue mass

CT

Lesions most often large, markedly or, less commonly, moderately enhancing after contrast administration.
Often heterogeneous.
Calcifications in 10% of cases

MRI

Iso- or hypointense relative to renal cortex on SE T1.
Hyperintense (70%) or isointense on T2.
Intense enhancement after gadolinium administration on T1

Nuclear medicine

Iodine-131 MIBG scintigraphy or octreoscan: increased uptake.
Specificity 95%. Sensitivity close to 90%.
18F-DOPA and 18F-fluorodopamine PET: usefulness currently under evaluation

Management

Management within a multidisciplinary care network: national RENATEN and INCA COMETE networks.
Surgical treatment after medical preparation.
Genetic counseling: any patient with a Paraganglioma, especially if under 35 years of age, or with multiple lesions or a malignant Paraganglioma, should be offered genetic testing for diagnostic purposes