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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 .
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 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).
Common sites are spinal or autonomic nerves near the midline; Uncommon sites: gastrointestinal tract, bone, soft tissues, heart, bronchus, liver, and skin; Neuroendocrine tumour (previously paraganglioma) Malignant Very rare Cauda equina region
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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