Medullary thyroid cancer natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

If left untreated, patients with medullary thyroid cancer may progress to develop metastasis. Common complications of medullary thyroid cancer include vocal cord compression, dysphagia, and dyspnea. The presence of metastasis is associated with a particularly poor prognosis among patients with medullary thyroid cancer. The 5 year event free survival rate is 80%.

Natural History

The symptoms of medullary thyroid cancer usually develop in the third decade of life, and start with symptoms such as swelling in the neck. Without treatment, the patient will develop symptoms of metastasis such as Cushing's syndrome and diarrhea, which may eventually lead to death.

Complications

Prognosis

  • Medullary thyroid cancer prognosis depends on the stage of the disease at the time of diagnosis.[1]
  • The 5-year relative survival rate is 93% for stage I to III.
  • The 5-year survival rate for stage IV is 28%.
  • Medullary thyroid cancer is associated with a 5-year survival rate of 86% and 10-year survival rate of 65%.
  • By overall cancer staging into stages I to IV, the 5-year survival rate is 100% at stage I, 98% at stage II, 81% at stage III and 28% at stage IV.[2] The prognosis of medullary thyroid cancer is poorer than that of follicular and papillary thyroid cancer when it has metastasized (spread) beyond the thyroid gland.
  • The prognostic value of measuring calcitonin and carcinoembryonic antigen (CEA) concentrations in the blood was studied in 65 medullary thyroid cancer patients who had abnormal calcitonin levels after surgery (total thyroidectomy and lymph node dissection).[3] The prognosis correlated with the rate at which the postoperative calcitonin concentration doubles, termed the calcitonin doubling time (CDT), rather than the pre- or postoperative absolute calcitonin level:
  • CDT less than 6 months: 3 patients out of 12 (25%) survived 5 years. 1 patient out of 12 (8%) survived 10 years. All died within 6 months to 13.3 years.
  • CDT between 6 months and 2 years: 11 patients out of 12 (92%) survived 5 years. 3 patients out of 8 (37%) survived 10 years. 4 patients out of 12 (25%) survived to the end of the study.
  • CDT more than 2 years: 41 patients out of 41 (100%) were alive at the end of the study. These included 1 patient whose calcitonin was stable and 11 patients who had decreasing calcitonin levels.
  • The calcitonin doubling time was a better predictor of MTC survival than CEA[3] but following both tests is recommended.[4][5]

References

  1. Hundahl, Scott A.; Fleming, Irvin D.; Fremgen, Amy M.; Menck, Herman R. (1998). "A National Cancer Data Base report on 53,856 cases of thyroid carcinoma treated in the U.S., 1985-1995". Cancer. 83 (12): 2638–2648. doi:10.1002/(SICI)1097-0142(19981215)83:12<2638::AID-CNCR31>3.0.CO;2-1. ISSN 0008-543X.
  2. cancer.org > Thyroid Cancer By the American Cancer Society. In turn citing: AJCC Cancer Staging Manual (7th ed).
  3. 3.0 3.1 Barbet J, Campion L, Kraeber-Bodéré F, Chatal JF (2005). "Prognostic impact of serum calcitonin and carcinoembryonic antigen doubling-times in patients with medullary thyroid carcinoma". J. Clin. Endocrinol. Metab. 90 (11): 6077–84. doi:10.1210/jc.2005-0044. PMID 16091497.
  4. Thyroid Carcinoma. NCCN guidelines. http://www.nccn.org/professionals/physician_gls/pdf/thyroid.pdf
  5. ASCO SEP 3rd edition


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