Medullary Thyroid Cancer
Current Clinical Trials
Medullary thyroid cancer (MTC) comprises 3% to 4% of all thyroid cancers.
These tumors usually present as a mass in the neck or thyroid, often associated
with lymphadenopathy,[1] or they may be diagnosed through screening family
members. MTC can also be diagnosed by fine-needle aspiration biopsy. Cytology
typically reveals hypercellular tumors with spindle-shaped cells and poor
adhesion.[2]
The overall survival of patients with MTC is 86% at 5 years and 65% at 10 years. Poor prognostic
factors include advanced age, advanced stage, prior neck surgery, and
associated multiple endocrine neoplasia (MEN) 2B.[2-4]
Approximately 25% of reported cases of MTC are familial.
Familial MTC syndromes include MEN 2A, which is
the most common; MEN 2B; and familial non-MEN syndromes. (Refer to the PDQ summary on Genetics of Medullary Thyroid Cancer for more information.) Any patient with a
familial variant should be screened for other associated endocrine tumors,
particularly parathyroid hyperplasia and pheochromocytoma. MTC can secrete
calcitonin and other peptide substances. Determining the level of calcitonin
is useful for diagnostic purposes and for following the results of treatment.
Family members should be screened for calcitonin elevation and/or for the RET
proto-oncogene mutation to identify other individuals at risk for developing
familial MTC. All patients with MTC (whether familial or sporadic) should be
tested for RET mutations, and if they are positive, family members should
also be tested. Whereas modest elevation of calcitonin may lead to a false-positive diagnosis of medullary carcinoma, DNA testing for the RET mutation is
the optimal approach. Family members who are gene carriers should undergo
prophylactic thyroidectomy at an early age.[5,6]
Treatment options:
-
Thyroidectomy: Patients with medullary thyroid cancer should be treated with a total
thyroidectomy, unless there is evidence of distant metastasis. In patients
with clinically palpable medullary carcinoma of the thyroid, the incidence of
microscopically positive nodes is more than 75%; routine central and bilateral
modified neck dissections have been recommended.[7] When cancer is confined to
the thyroid gland, the prognosis is excellent.
-
External radiation therapy: External radiation therapy has been used for palliation of locally recurrent
tumors, without evidence that it provides any survival advantage.[8] Radioactive iodine has no place in the treatment of patients with MTC.
-
Palliative chemotherapy: Palliative chemotherapy has been reported to produce occasional responses in
patients with metastatic disease.[9-12] No single drug regimen can be
considered standard. Some patients with distant metastases will experience
prolonged survival and can be managed expectantly until they become
symptomatic.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with thyroid gland medullary carcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References
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Soh EY, Clark OH: Surgical considerations and approach to thyroid cancer. Endocrinol Metab Clin North Am 25 (1): 115-39, 1996.
[PUBMED Abstract]
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Giuffrida D, Gharib H: Current diagnosis and management of medullary thyroid carcinoma. Ann Oncol 9 (7): 695-701, 1998.
[PUBMED Abstract]
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Saad MF, Ordonez NG, Rashid RK, et al.: Medullary carcinoma of the thyroid. A study of the clinical features and prognostic factors in 161 patients. Medicine (Baltimore) 63 (6): 319-42, 1984.
[PUBMED Abstract]
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Bergholm U, Bergström R, Ekbom A: Long-term follow-up of patients with medullary carcinoma of the thyroid. Cancer 79 (1): 132-8, 1997.
[PUBMED Abstract]
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Lips CJ, Landsvater RM, Höppener JW, et al.: Clinical screening as compared with DNA analysis in families with multiple endocrine neoplasia type 2A. N Engl J Med 331 (13): 828-35, 1994.
[PUBMED Abstract]
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Decker RA, Peacock ML, Borst MJ, et al.: Progress in genetic screening of multiple endocrine neoplasia type 2A: is calcitonin testing obsolete? Surgery 118 (2): 257-63; discussion 263-4, 1995.
[PUBMED Abstract]
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Moley JF, DeBenedetti MK: Patterns of nodal metastases in palpable medullary thyroid carcinoma: recommendations for extent of node dissection. Ann Surg 229 (6): 880-7; discussion 887-8, 1999.
[PUBMED Abstract]
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Brierley JD, Tsang RW: External radiation therapy in the treatment of thyroid malignancy. Endocrinol Metab Clin North Am 25 (1): 141-57, 1996.
[PUBMED Abstract]
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Shimaoka K, Schoenfeld DA, DeWys WD, et al.: A randomized trial of doxorubicin versus doxorubicin plus cisplatin in patients with advanced thyroid carcinoma. Cancer 56 (9): 2155-60, 1985.
[PUBMED Abstract]
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De Besi P, Busnardo B, Toso S, et al.: Combined chemotherapy with bleomycin, adriamycin, and platinum in advanced thyroid cancer. J Endocrinol Invest 14 (6): 475-80, 1991.
[PUBMED Abstract]
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Wu LT, Averbuch SD, Ball DW, et al.: Treatment of advanced medullary thyroid carcinoma with a combination of cyclophosphamide, vincristine, and dacarbazine. Cancer 73 (2): 432-6, 1994.
[PUBMED Abstract]
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Orlandi F, Caraci P, Berruti A, et al.: Chemotherapy with dacarbazine and 5-fluorouracil in advanced medullary thyroid cancer. Ann Oncol 5 (8): 763-5, 1994.
[PUBMED Abstract]
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