Abstract
IntroductionMedullary thyroid carcinoma (MTC) is a rare cancer that accounts for 5% of thyroid cancers. Serum calcitonin is a good biomarker for MTC, which is used for diagnosis, prognosis, and monitoring of recurrence. Calcitonin-negative MTC (CNMTC) is rare but confounds diagnostic and prognostic directions. This study introduces 19 cases of CNMTC in a single center.MethodFrom 2002 March to 2020 July, more than 76,500 patients had undergone thyroid surgery due to thyroid cancer at the Severance Hospital, and a total of 320 patients were diagnosed with MTC (0.4%). Serum calcitonin levels were obtained from every patient who was suspected with MTC. These patients had undergone either bilateral total thyroidectomy or unilateral thyroidectomy with central compartment lymph node dissection, and additional modified radical lymph node dissection if lateral lymph node metastasis was positive. Postoperative monitoring and out-patient clinic follow-up were performed with obtaining the serum calcitonin levels.ResultNineteen patients tested negative for calcitonin preoperatively (6%). The mean preoperative calcitonin level was 5.1pg/mL if undetectable level is regarded as 0pg/mL. Only two patients were males, and the female bias was significant (p = 0.017). No one except two patients with modified radical neck dissection showed central compartment lymph node metastasis. Every patient’s postoperative calcitonin level remained low. The median follow-up period was 71 months. There was no recurrence and only one fatality, and the overall survival rate was 95%.ConclusionSince incidence of CNMTC is not negligible, MTC should not be ruled out in the diagnostic phase even if serum calcitonin is negative in preoperative examination. We presented 19 cases of CNMTC whose prognosis in general were favorable. Markers of serum and immunohistochemical samples other than calcitonin should be actively examined.
Highlights
Medullary thyroid carcinoma (MTC) is a rare cancer that accounts for 5% of thyroid cancers
Preoperative fine needle aspiration showed eight cases suspected for malignancy, one papillary thyroid cancer, and 10 MTC
MTC is a neuroendocrine tumor originating from parafollicular C cells [1]
Summary
Medullary thyroid carcinoma (MTC) is a rare cancer that accounts for 5% of thyroid cancers. Such tumors were called hormone-negative endocrine tumors, in that they did not secrete their resident hormones Later, this type of tumor was termed atypical MTC because its immunohistochemical stain is negative for calcitonin but positive for neuroendocrine markers such as chromogranin A (CgA), synaptophysin, and neuron specific enolase (NSE) [9]. This type of tumor was termed atypical MTC because its immunohistochemical stain is negative for calcitonin but positive for neuroendocrine markers such as chromogranin A (CgA), synaptophysin, and neuron specific enolase (NSE) [9] Some called it non-secretory medullary thyroid cancer (NCRMTC) because normal basal serum calcitonin observed in such cases seemed to be derived from defective secretory mechanism in these MTC [10]. We introduce 19 cases of CNMTC treated at the Severance Hospital across 19 years
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