| Abstract | BACKGROUND: Medullary thyroid carcinoma (MTC) accounts for 5% to 10% of all thyroid malignancies. Approximately 75% of cases are sporadic. Familial forms of medullary thyroid carcinoma account for the remaining 25% of cases--MEN IIa, MEN IIb and FMTC.
METHODS: Retrospectively 22 cases of medullary carcinoma of thyroid gland were analysed. Total thyroidectomy with selective lymph dissection was performed in all patients.
RESULTS: 18 patients were with sporadic form of medullary thyroid carcinoma, 4--with FMTC; 21 were with primary carcinoma, 1--with recurrent carcinoma Mean age--32 years (22-59). At the follow-up control (max 10 years) there is no evidence of disease recurrence.
CONCLUSION: Recent advances in genetic testing allow early diagnosis and treatment of familial MTC syndromes. Despite some advances in treatment, optimal management is still controversial. Total thyroidectomy with selective lymph dissection remains the choice of surgical treatment. In the familial forms medullary carcinoma is associated with well-characterized, germline mutations in the RET protooncogene. Both genetic and biochemical screening are of essential significance for early diagnosis and adequate and optimal surgical treatment. |
