An Update on the Histology of Pheochromocytomas: How Does it Relate to Genetics?

摘要

Pheochromocytomas (PCC) and paragangliomas (PGL) are rare tumors, originating from neural crest-derived precursor cells, that occur in the adrenal gland and in sympathetic and parasympathet-ic ganglia in the abdomen, thorax, pelvis, and head and neck area, respectively. Histologically, they are very similar and frequently indistinguishable, although PCC tend to have more nuclear atypia and cytoplasmic basophilia, whereas PGL display less atypia and are usually more eosinophilic. Their histopathological recognition is usually straightforward and in equivocal cases the neuroendocrine markers synaptophysin and chromogranin A can be used, both of which should show diffuse strong cytoplasmic staining. In addition, the use of S100 may be supportive when showing a sustentacular cell pattern of non-neoplastic cells that surround the nodules of PCC tumor cells, the so-called "Zellballen".