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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. 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.
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]
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]
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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.
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According to the North American Neuroendocrine Tumor Society, the prevalence of pheochromocytoma is between 1:2,500 and 1:6,500, meaning that for every 2,500–6,500 people, there is (on average) one person with pheochromocytoma. [170] In the United States, this equates to an annual incidence (new cases per year) of 500 to 1,600 cases. [170]