Department of Endocrine Surgery, University of California, San Francisco, CA 94143, USA.
Cancer Control. 2011 Apr;18(2):104-12. doi: 10.1177/107327481101800205.
Pheochromocytomas and paragangliomas are intra- and extra-adrenal neoplasms that are rarely malignant. The treatment of those that are malignant has remained a challenge because little was known about the molecular pathways involved in its malignant transformation. Recently, however, the genetic and molecular changes involved in malignant pheochromocytoma have come to be understood.
The authors review the recent literature about the changing treatment options for malignant pheochromocytomas and paragangliomas.
Traditional treatments for malignant pheochromocytoma remain unsuccessful. With the advances made in genomics and proteomics, novel pathways in pheochromocytoma carcinogenesis are becoming the targets of new treatment strategies and show promising results.
Although several studies and clinical trials show great promise for improving the treatment of pheochromocytomas and paragangliomas, the hope is that future collaborative efforts will allow for prospective clinical trials using an evidenced-based approach.
嗜铬细胞瘤和副神经节瘤是发生于肾上腺内或肾上腺外的肿瘤，很少是恶性的。由于对恶性转化涉及的分子途径知之甚少，恶性嗜铬细胞瘤的治疗一直是个挑战。然而，最近恶性嗜铬细胞瘤的遗传和分子变化已经被了解。
作者回顾了有关恶性嗜铬细胞瘤和副神经节瘤治疗选择变化的最新文献。
恶性嗜铬细胞瘤的传统治疗仍然无效。随着基因组学和蛋白质组学的进步，嗜铬细胞瘤发生的新途径正成为新治疗策略的靶点，并显示出有希望的结果。
尽管几项研究和临床试验显示出改善嗜铬细胞瘤和副神经节瘤治疗的巨大希望，但未来的合作努力有望采用循证方法进行前瞻性临床试验。
