Undiagnosed pheochromocytoma leads to a very high intraoperative risk of hypertensive crisis and, potentially, death. Surgery A total thyroidectomy with bilateral neck dissection is the gold standard for treating medullary thyroid cancer, and is the most definitive means of achieving a cure in patients without distant metastases or extensive nodal involvement. Risks of surgery include loss of vocal control, irreparable nerve damage, death or the need for second operation to clean out residual diseased lymph nodes left behind if the sentinel node biopsy was positive for cancerous spread. Extensive surgery can be effective when the condition is detected early, but a risk for recurrence remains, particularly in patients with multiple positive lymph nodes or extracapsular invasion. About half of patients have metastasis to regional lymph nodes at the time of diagnosis. The European Society of Endocrine Surgeons has published recommendations for managing this condition in gene carriers. The timing of surgery depends on the type of mutation present. For those in the highest risk group, surgery is recommended in the first year of life. In lower risk cases surgery may be delayed up to the age of ten years, the precise timing depending on the mutation and other factors. Radiation External beam radiotherapy is recommended when there is a high risk of regional recurrence, even after optimum surgical treatment. In this study, patients treated with external beam radiation were compared to a control group. Disease control with radiation was far superior in the group receiving radiation. The authors of the study [14] wrote: "in 40 high risk patients (microscopic residual disease, extraglandular invasion, or lymph node involvement), the local/regional relapse free rate was 86% at 10 years with postoperative external beam radiation (25 patients), and 52% for those with no postoperative external radiation (p = 0.049). To optimize local/regional tumor control, we therefore continue to advise external beam radiation in patients at high risk of local/regional relapse." Unlike other differentiated thyroid carcinoma, there is no role for radioiodine treatment in medullary-type disease. Protein kinase inhibitors Clinical trials of protein kinase inhibitors, which block the abnormal kinase proteins involved in the development and growth of medullary cancer cells, showed clear evidence of response in 10-30% of patients. In the majority of responders there has been less than a 30% decrease in tumor mass, yet the responses have been durable; responses have been stable for periods exceeding 3 years. The major side