Medullary thyroid cancer: Treatment and prognosis
- R Michael Tuttle, MD
R Michael Tuttle, MD
- Professor of Medicine
- Memorial Sloan Kettering Cancer Center
- Section Editors
- David S Cooper, MD
David S Cooper, MD
- Editor-in-Chief — Endocrinology
- Section Editor — Pituitary Disease; Thyroid Disease
- Professor of Medicine and International Health
- Johns Hopkins University School of Medicine
- Douglas S Ross, MD
Douglas S Ross, MD
- Section Editor — Thyroid Disease
- Professor of Medicine
- Harvard Medical School
Patients with medullary thyroid cancer (MTC) can be cured only by complete resection of the thyroid tumor and any local and regional metastases. For patients with residual or recurrent disease after primary surgery or for those with distant metastases, the most appropriate treatment (surgery, chemotherapy, or radiotherapy) is less clear.
This topic review will discuss the role of surgery as primary therapy and the approaches to treating patients with persistent or recurrent MTC after surgery. The approach outlined below is largely in agreement with American Thyroid Association (ATA) and National Comprehensive Cancer Network (NCCN) guidelines [1,2]. Recommendations for the timing of thyroidectomy in children with RET mutations and the role of chemotherapy in the treatment of MTC are reviewed elsewhere. (See "Approach to therapy in multiple endocrine neoplasia type 2", section on 'Preventive surgery' and "Medullary thyroid cancer: Chemotherapy and immunotherapy".)
Newly diagnosed patients with medullary thyroid cancer (MTC) should be staged both biochemically and radiologically (algorithm 1). This evaluation is reviewed briefly below and in more detail elsewhere. (See "Medullary thyroid cancer: Clinical manifestations, diagnosis, and staging", section on 'Evaluation'.)
The following tests should be performed:
●Measurement of serum calcitonin and carcinoembryonic antigen (CEA) to determine whether they are produced by the tumor, and if so, as a baseline for comparison with results obtained after surgery. Among patients with either sporadic or familial tumors, those with higher preoperative serum calcitonin concentrations have larger tumors and are less likely to have normal serum concentrations after surgery than those with lower preoperative values .
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- PREOPERATIVE EVALUATION
- SURGICAL APPROACH
- MTC confined to the neck
- Locally advanced or metastatic MTC
- MTC diagnosed after lobectomy
- POSTOPERATIVE MANAGEMENT
- Monitor for postoperative complications
- Thyroxine therapy
- Assessment of tumor samples for somatic mutations
- SUBSEQUENT MANAGEMENT
- Serum calcitonin and CEA measurement
- - Undetectable calcitonin and normal CEA
- - Persistent hypercalcitoninemia
- Management of persistent/recurrent disease
- - Small volume, asymptomatic
- - Large volume or symptomatic
- - Palliation of the symptoms associated with hormonal excess
- SUMMARY AND RECOMMENDATIONS