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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 .
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).
Glomus tumors are usually solitary and small lesions. The vast majority are found in the hand, wrist, foot, and under the fingernails. [3]They are often painful, and the pain is reproduced when the lesion is placed in cold water - a history of sensitivity to cold weather is also common. [6]
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.
The criteria in 2024 included all patients with paraganglioma and all patients with unilateral pheochromocytoma aged under 60. [46] The most recent data indicates that there are 25 pheochromocytoma susceptibility genes; however, just 12 are recognized as part of a well-known syndrome. [ 9 ]
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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]
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