Endoperspectives, Volume 5, Issue 3
نویسنده
چکیده
The World Health Organization defines pheochromocytoma (PHEO) as a tumor arising from the adrenal medulla and paraganglioma (PGL) as a tumor arising from the paraganglia outside the adrenal medulla. PGLs are located in the head, neck, thorax, abdomen, and pelvis, and can be classified as parasympathetic and sympathetic depending on their origin. Parasympathetic PGLs are mainly located in the head and neck; these tumors may be locally invasive but rarely develop metastases and are not discussed here. Approximately 10-17% of PHEO/PGLs are metastatic. Treatment against metastatic disease includes systemic chemotherapy and radiopharmaceutical agents that are mostly nonspecific and indiscriminately target dividing cells. Patients with metastatic tumors have high morbidity and mortality rates from excessive catecholamine secretion, cardiovascular complications, and bulky disease, for which no curative treatment is available. The 5-year overall survival rate for patients with metastatic tumors ranges is approximately 50%. However, over the last decade our understanding of the genetic and molecular causes of PHEO/PGL has markedly improved. Up to 50% of metastatic cases could be associated with hereditary germline mutations. Additionally, many sporadic metastatic PHEO/PGLs share a similar molecular profile with hereditary tumors. This knowledge is now leading to the development of new therapies based on the molecular mechanisms involved in the formation of malignant PHEO/PGLs. In this review, we will describe clinical predictors of malignancy that could determine the aggressiveness of follow-up and treatment and our current systemic therapeutic approaches for patients with unresectable disease.
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تاریخ انتشار 2012