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A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). When the same type of tumor is found in the adrenal gland, they are referred to as a pheochromocytoma .
It can be the source of paraganglioma. [8]The organ of Zuckerkandl is of pathological significance in the adult as a common extra-adrenal site of pheochromocytoma though the most common extra-adrenal site is in the superior para-aortic region between the diaphragm and lower renal poles.
The criteria in 2024 included all patients with paraganglioma and all patients with unilateral pheochromocytoma aged under 60. [46] The most recent data indicates that there are 25 pheochromocytoma susceptibility genes; however, just 12 are recognized as part of a well-known syndrome. [ 9 ]
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Chromogranin-A (CgA) or parathyroid secretory protein 1 is encoded in the human by the gene CHGA.Cga is a member of the granin family of neuroendocrine secretory proteins. As such, it is located in secretory vesicles of neurons and endocrine cells such as islet beta cell secretory granules in the pancreas.
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M8680/3 paraganglioma, malignant M8681/1 Sympathetic paraganglioma M8682/1 Parasympathetic paraganglioma M8683/0 Gangliocytic paraganglioma (C17.0) M8690/1 Glomus jugulare tumor, NOS (C75.5) Jugular/jugulotympanic paranglioma; M8691/1 Aortic body tumor (C75.5) Aortic/aorticopulmonary paraganglioma; M8692/1 Carotid body tumor/paraganglioma (C75.4)
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