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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 .
Tumors of the paraganglionic tissues are known as paragangliomas, though this term tends to imply the nonchromaffin type, and can occur at a number of sites throughout the body. Chromaffin paragangliomas are issued from chromaffin cells, and are known as pheochromocytomas .
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 ]
Ganglioneuroma (occasionally called a "ganglioma") is a rare and benign tumor of the autonomic nerve fibers arising from neural crest sympathogonia (undifferentiated cells of the sympathetic nervous system). [1]
Mutations in the genes encoding alpha subunits of hypoxia-inducible factors (HIF-alpha) have not previously been identified in any cancer. [4]In the Pacak–Zhuang syndrome, patients have somatic gain of function mutations in the genes encoding for HIF2A, leading to prolonged HIF-2α activity and, thus, an increase in its half-life. [5]
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)