Sporadic Medullary Thyroid Cancer
Medullary thyroid cancer (MTC) is a neuroendocrine tumor of the parafollicular or C cells of the thyroid gland. It accounts for approximately 1–2% of thyroid cancers. The production of calcitonin is a characteristic feature of this tumor. Most of the MTCs are sporadic whereas 25% of cases are familial as part of the multiple endocrine neoplasia type 2 (MEN2) syndrome. For sporadic MTCs, there are no germline RET mutations. The most common presentation of sporadic MTC is a solitary thyroid nodule. Systemic symptoms may occur due to hormonal secretion by the tumor. Most sporadic MTCs also secrete carcinoembryonic antigen (CEA). The diagnosis of sporadic MTC is usually made after fine needle aspiration (FNAB) biopsy in a patient who has a solitary thyroid nodule. Patients with sporadic MTC can be cured only by complete resection of the thyroid tumor with bilateral central neck lymph node dissection. For patients with the residual or recurrent disease after primary surgery or for those with distant metastases, the most appropriate treatment is less clear and still debatable.
In this case presentation, we discussed a patient with an MTC, its evaluation, diagnosis, management and the future of the MTC according to the literature.
KeywordsSporadic medullary thyroid cancer (MTC) Parafollicular or C cells Calcitonin Multiple endocrine neoplasia type 2 (MEN-2) Germline RET mutations Carcinoembryonic antigen (CEA)
- 4.Gagel RF, Hoff AO, Cote GJ. Medullary thyroid carcinoma. In: Braverman LE, Utiger RD, editors. Werner & Ingbar's the thyroid. 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 967.Google Scholar
- 18.http://www.nccn.org/professionals/physician_gls/f_guidelines.asp. Accessed 20 Aug 2012.
- 21.Oudoux A, Salaun PY, Bournaud C, Campion L, Ansquer C, Rousseau C, et al. Sensitivity and prognostic value of positron emission tomography with F-18-fluorodeoxyglucose and sensitivity of immunoscintigraphy in patients with medullary thyroid carcinoma treated with anticarcinoembryonic antigen-targeted radioimmunotherapy. J Clin Endocrinol Metab. 2007;92:4590.CrossRefGoogle Scholar
- 29.Modigliani E, Cohen R, Campos JM, Franc B, Niccoli-Sire P, Conte-Devolx B, et al. Prognostic factors for survival and for biochemical cure in medullary thyroid carcinoma: results in 899 patients. The GETC Study Group. Groupe d'étude des tumeurs à calcitonine. Clin Endocrinol (Oxf). 1998;48:265.CrossRefGoogle Scholar