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Specialty. Oncology. 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. They are rare tumors, with an overall estimated incidence of 1 in 300,000. [1]
Carney triad. Carney triad (CT) is characterized by the coexistence of three types of neoplasms, mainly in young women, [1] including gastric gastrointestinal stromal tumor, pulmonary chondroma, and extra- adrenal paraganglioma. [2] The underlying genetic defect remains elusive. CT is distinct from Carney complex and Carney-Stratakis syndrome.
Paraganglion. A paraganglion (pl. paraganglia) is a group of non- neuronal cells derived of the neural crest. They are named for being generally in close proximity to sympathetic ganglia. They are essentially of two types: (1) chromaffin or sympathetic paraganglia made of chromaffin cells and (2) nonchromaffin or parasympathetic paraganglia ...
Pheochromocytoma. Pheochromocytoma is a rare tumor of the adrenal medulla composed of chromaffin cells and is part of the paraganglioma (PGL) family of tumors, being defined as an intra-adrenal PGL . [2][4][5] These neuroendocrine tumors can be sympathetic, where they release catecholamines into the bloodstream which cause the most common ...
Pacak–Zhuang syndrome. The Pacak-Zhuang syndrome is a recently described disease manifestation in females that includes multiple paragangliomas or pheochromocytomas and somatostatinomas (in some), both neuroendocrine tumors, and secondary polycythemia associated with high erythropoietin levels. Paragangliomas in these patients are mainly ...
A glomus tumor (also known as a "solitary glomus tumor" [1]) is a rare neoplasm arising from the glomus body and mainly found under the nail, on the fingertip or in the foot. [2]: 670 They account for less than 2% of all soft tissue tumors. [3] The majority of glomus tumors are benign, but they can also show malignant features. [4]
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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). [1]