Natural history, diagnosis, treatment and outcome of medullary thyroid cancer: 37 years experience on 157 patients

Aim The analysis of a 37-year retrospective study on diagnosis, prognostic variables, treatment and outcome of a large group of medullary thyroid cancer (MTC) patients was conducted, in order to plan a possible evidence-based management process. Methods Between Jan 1967 to Dec 2004, 157 consecutive MTC patients underwent surgery in our centre: 60 males and 97 females, mean age 47.3 years (range 6–79). Total thyroidectomy was performed in 143 patients (91.1%); central compartment (CC) node dissection (level VI) in 41 patients; central plus lateral compartment (LC) node dissection (levels II, III, and IV) in 82 patients. Subtotal thyroidectomy was initially performed in 14 cases: 10 of them were re-operated because of persistence of elevated serum calcitonin levels. Results After a median post-surgical follow-up of 68 months (range 2–440 months), 42.9% of patients were living disease-free, 39.8% were living with disease, 3.1% were deceased due to causes different from MTC, and 3.2% were deceased due to MTC. The overall 10-year survival rate was 72%. At uni-variate statistical analysis (a) patientʹs age at initial treatment (>45 years; ≥45 years), (b) sporadic vs. hereditary MTC, (c) disease stage, and (d) the extent of surgical approach resulted as significant variables. Instead, at multivariate statistical analysis, only (a) patientʹs age at initial diagnosis, (b) disease stage, and (c) the extent of surgery resulted as significant and independent prognostic variables influencing survival. Conclusion The presence of lymph node and distant metastases at first diagnosis significantly worsened prognosis and survival rate in our series. Early diagnosis of MTC is very important, allowing complete surgical cure in Stages I and II patients. Due to the relatively bad prognosis of MTC, especially for disease Stages III and IV, it appears reasonable to recommend radical surgery including total thyroidectomy plus CC lymphoadenectomy as the treatment of choice, plus LC lymphoadenectomy in patients with palpable and/or ultrasound enlarged neck lymph nodes.