Tischler A S
Department of Pathology, Tufts University School of Medicine and New England Medical Center, Boston, MA 02111, USA.
Semin Diagn Pathol. 2000 May;17(2):120-6.
Composite tumors of the adrenal medulla usually consist of pheochromocytoma admixed with ganglioneuroma or ganglioneuroblastoma. These neoplasms reflect phenotypic plasticity shown by primitive sympathetic cells and mature chromaffin cells in vitro. They may give rise to metastatic neuroblastoma in adults and may cause signs and symptoms attributable to both catecholamine and neuropeptide production. Schwann cells and sustentacular cells are typically numerous in these tumors but it is not known whether they are neoplastic. Immunohistochemical staining for catecholamine biosynthetic enzymes, secretory vesicle proteins and S-100 protein tends to recapitulate staining of the normal adrenal medulla or sympathetic ganglia. Sparsity of chromogranin A in the cell bodies of immature and mature neurons is a diagnostically useful characteristic.
肾上腺髓质的复合性肿瘤通常由嗜铬细胞瘤与神经节瘤或神经母细胞瘤混合组成。这些肿瘤反映了原始交感神经细胞和成熟嗜铬细胞在体外表现出的表型可塑性。它们可能在成人中引发转移性神经母细胞瘤，并可能导致因儿茶酚胺和神经肽产生而引起的体征和症状。雪旺细胞和支持细胞在这些肿瘤中通常数量众多，但尚不清楚它们是否为肿瘤性的。对儿茶酚胺生物合成酶、分泌囊泡蛋白和S-100蛋白进行免疫组织化学染色往往重现正常肾上腺髓质或交感神经节的染色情况。未成熟和成熟神经元细胞体中嗜铬粒蛋白A稀少是一个有用的诊断特征。
