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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 .
A gangliocytic paraganglioma is a rare tumour that is typically found in the duodenum and consists of three components: (1) ganglion cells, (2) epithelioid cells (paraganglioma-like) and, (3) spindle cells (schwannoma-like).
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.
Elevated plasma free metanephrines is considered the gold standard diagnosis for pheochromocytoma. [63] Over 10 studies have confirmed that the sensitivity and specificity of this test is 97% and 93% respectively; however, there is still concern for false positive results in the correct clinical scenario. [ 6 ]
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Micrograph highlighting the sustentacular cells in a paraganglioma. S100 immunostain. A sustentacular cell is a type of cell primarily associated with structural support, they can be found in various tissues.
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)
Desmoplastic small round cell tumour, with desmoplasia surrounding nests of cancer cells.. In medicine, desmoplasia is the growth of fibrous connective tissue. [1] It is also called a desmoplastic reaction to emphasize that it is secondary to an insult.