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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 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]
GP consist of three components (1) ganglion cells, (2) epithelioid cells (neuroendocrine-like), and (3) spindle cells (schwannoma-like). The microscopic differential diagnosis includes poorly differentiated carcinoma, neuroendocrine tumour and paraganglioma. [1] GPs may be sporadic or arise in the context neurofibromatosis type 1.
Over the last decade, there have been five functional techniques used to evaluate the pheochromocytoma patient (1) 18F-fluorodeoxyglucose positron emission tomography (18 F-FDG PET), commonly referred to as the PET scan, (2) iodine-123 meta-iodobenzylguanadine (123 I-MIBG), (3) 18F-flurodihydroxyphenylalanine (18 F-FDOPA),(4) 68Ga-DOTA coupled ...
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.
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] However, ganglioneuromas themselves are fully differentiated neuronal tumors that do not contain immature elements. [2]
Zellballen are separated into groups by segmenting bands of fibrovascular stroma, and are surrounded by supporting sustentacular cells. [1] A zellballen pattern is diagnostic for paraganglioma or pheochromocytoma. [2] Zellballen is German for "ball of cells". [3]
Carney triad (CT), named for J Aidan Carney, is considered to be a specific type of multiple endocrine neoplasia (MEN). The three classically associated tumors are a subset of gastric epithelioid leiomyosarcoma (it is now known that this subset is actually gastrointestinal stromal tumor arising from the interstitial cells of Cajal), pulmonary chondroma, and extra-adrenal paraganglioma.