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Most paragangliomas are asymptomatic, present as a painless mass, or create symptoms such as hypertension, tachycardia, headache, and palpitations. [3] While all contain neurosecretory granules, only in 1–3% of cases is secretion of hormones such as catecholamines abundant enough to be clinically significant; in that case manifestations often resemble those of pheochromocytomas (intra ...
Chromaffin paragangliomas are issued from chromaffin cells, and are known as pheochromocytomas. Adrenal pheochromocytomas are usually benign while extra-adrenal ones are more malignant. [ 3 ] They are most of the time in the adrenals, and only rarely outside of the abdomen.
An additional benefit of the DOTA analogues is the ability for treatment with peptide receptor radionuclide therapy, which will be discussed in the treatment section below. [ 98 ] Also, HED-PET has shown to be an accurate tool to diagnose and rule out pheochromocytoma in complex clinical scenarios and to characterise equivocal adrenal tumours.
Glomus tumors are modified smooth muscle cells that control the thermoregulatory function of dermal glomus bodies. As stated above, these lesions should not be confused with paragangliomas, which were formerly also called glomus tumors in now-antiquated clinical usage. Glomus tumors do not arise from glomus cells, but paragangliomas do.
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Paragangliomas in these patients are mainly localized to the abdomen whereas somatostatinomas are found in the second portion of the duodenum, as shown by imaging or biochemistry. [1] This syndrome is of special interest as finding more than one type of neuroendocrine tumor in one individual is unusual. [ 1 ]