Paraganglioma
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● Tumors are composed of clusters of
polygonal to spindle shaped chromaffin cells or chief cells with finely granular cytoplasm and salt-and-pepper nuclear chromatin.
■ Tumor cells are surrounded by supporting sustentacular cells, creating small nests or alveoli (zellballen) that are supplied by a rich vascular network.
● Paraganglioma is associated with mutations of the genes for the succinate dehydrogenase, SDHD (previously known as PGL1), SDHA, SDHC (previously PGL3) and SDHB have been identified as causing familial head and neck paragangliomas.
● Immunophenotyping of paraganglioma will be Positive for CD10, CD56, CD57 and CD68.
