The immunohistochemical results were AE1/AE3(-), GATA3(-), INSM-1(+), CgA(+), Ki-67(index 5%), S-100(local scattered +), Syn(+), MGMT(partial +), SDHB(+), PIT-1(-), T-PIT(-), SF-1(-); EBER ISH(-); the diagnosis was considered as a metastatic neuroendocrine tumor, with a tendency towards paraganglioma. The gene discussed is SDHB; the disease is paraganglioma.