Medullary thyroid cancer: clinico-pathological profile and outcome in a tertiary care center in North India.

摘要

BACKGROUND: Recent advances in genetic screening have ushered in a new era in diagnosis and management of medullary thyroid cancer (MTC). However, the same is not true for a resource-poor country, where clinicians are still struggling to diagnose and adequately manage this relatively uncommon thyroid malignancy. We hereby present our experience of managing MTC at a tertiary care referral center in North India. METHODS: This was a retrospective study conducted between January 1990 and July 2009. Demographics, clinical profiles, details of surgical procedures, and follow-up records were reviewed. RESULTS: A total of 71 patients with MTC were identified. Mean age of this group was 39.9+/-14.1 years, and men outnumbered women 1.7:1. Some 84.5% of patients had seemingly sporadic MTC, and 15.5% had familial MTC (MEN2a=14.1%, MEN 2b=1.4%). All patients had a thyroid nodule at the time of presentation. Mean tumor diameter was 4.9 cm. Cervical lymphadenopathy, mediastinal lymphadenopathy, extrathyroidal invasion, and distant metastases were present in 59%, 7.2%, 10%, and 4.2% of patients, respectively. Staging showed that 4.8% of patients were stage I, 17.5% stage II, 14.3% stage III, and 63.6% stage IV. Treatment was as follows: 92.6% of patients had total thyroidectomy (primary or secondary); 67.6% central compartment lymph node dissection, 62.3% lateral cervical lymph node dissection, and 7.2% trans-sternal mediastinal lymph node dissection performed. Some 66.7% of patients suffered from persistent hypercalcitoninemia, and 11.4% of those underwent reoperation. The 5-year and 10-year overall survival (OS) was 74.6% and 58%, respectively. CONCLUSIONS: The majority of the patients with MTC presented at stage IVa, contributing to the high rate of persistent hypercalcitoninemia. Despite a policy of observation and intervening only in overtly symptomatic patients with persistent hypercalcitoninemia, overall survival (OS) in our study was comparable to other series, reinforcing the belief that persistent hypercalcitoninemia has an indolent course in most patients.