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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]
The abdominal aorta and its branches. (Organ of Zuckerkandl is not shown, but it usually appears alongside the abdominal aorta.) The organ of Zuckerkandl is a chromaffin body derived from the neural crest located at the bifurcation of the aorta or at the origin of the inferior mesenteric artery. It can be the source of a paraganglioma.
Ganglioneuroma. 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]
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.
Oncology. 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]
Ganglioglioma. A ganglioglioma (or gangliocytoma) is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults. [3] They are mixed cell tumors containing both neural ganglionic cells and neural glial cell components. [4]
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]
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 ...