Treatment of malignant pheochromocytoma/paraganglioma

      Pheochromocytoma/paraganglioma is a rare neuroendocrine tumor, of which 10-17% are malignant. It has a high mortality rate and is very difficult to treat. The treatment methods include radionuclide therapy, chemotherapy, targeted therapy, etc. 131I-MIBG therapy is the most widely used treatment method, which can slow down the progression of the disease and improve the survival time, but it is only effective for the lesions with positive adrenal medullary images. Octreotide imaging has shown great advantages in localizing the diagnosis, but nucleotide-labeled growth inhibitor analogs are not used much because of adverse effects and less experience. Chemotherapy is also an effective treatment with limited literature, but has a long treatment time and moderate adverse effects. Sunitinib has shown promise for use in targeted therapy, but more evidence is still needed to support it.  The current main treatments for malignant pheochromocytoma/paraganglioma: 1. Surgical resection. At present, the first choice for malignant pheochromocytoma/paraganglioma after discovery is still surgery, which can improve the prognosis and reduce symptoms through radical or tumor-reducing surgery.  The overall efficiency is 84.5%. However, most patients have stable lesions that do not progress, so that the catecholamine releasing function is inhibited or even disappears, blood pressure is reduced or even returned to normal, and general symptoms such as weight and strength are significantly improved with relatively low side effects.  3. Chemotherapy. Chemotherapy recommended CVD program (cyclophosphamide, vincristine, nitrogen enzymes), currently considered the only effective chemotherapy program, the efficiency of about 50%, for 131I-MIBG does not uptake, chemotherapy can be selected, due to the heterogeneity of the tumor, 131I-MIBG treatment, part of the lesion progression of the need for 131I-MIBG combined with chemotherapy can improve the efficacy.  4. Anti-angiogenic targeted drug therapy may be effective. It is recommended that 131I-MIBG treatment and/or chemotherapy is not effective.  5.Radiotherapy is currently considered less effective for malignant pheochromocytoma/paraganglioma.  6.Control of blood pressure and blood sugar treatment. At present, according to our experience, most patients die from cardiovascular and cerebrovascular events. Controlling blood pressure and blood glucose can improve the prognosis. In addition to the above methods (1-4), it is necessary to take antihypertensive and hypoglycemic drugs, and antihypertensive drugs mainly apply alpha-blockers, beta-blockers, calcium antagonists, etc.