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Medullary Thyroid Carcinoma: Long-Term Outcomes of Surgical Treatment

Abstract

Background

Medullary thyroid carcinoma (MTC) accounts for 5 to 10% of all thyroid cancers but is responsible for a disproportionate number of deaths.

Methods

We performed a retrospective review to describe clinical outcomes in patients with medullary thyroid carcinoma, screening a subset of patients for somatic mutations in the RET and p18 genes and performing genotype-phenotype correlation in a tertiary-care referral hospital from 1967 to 2009.

Results

We studied a total of 94 patients identified from a prospectively maintained thyroid cancer database. Data gathered included patient demographics, serum calcitonin, clinical outcomes, histopathology, genetic analysis, and status at final follow-up. A subset cohort (n = 50) was screened for somatic mutations in the RET gene and the three exons of the p18 gene. The subset cohort was composed of hereditary medullary thyroid carcinoma (HMTC) (n = 19, index patients = 10, screen detected = 9) and sporadic medullary thyroid carcinoma (SMTC) (n = 31). There were no mutations in the p18 gene in the subset cohort.

Conclusions

A total of 67 SMTC and 27 (28.7%) HMTC cases identified. SMTC were older at initial presentation (52 vs. 34, P = 0.003), had higher preoperative serum calcitonin levels (7968 vs. 1346 ng/L, P = 0.008), and had lymph node recurrence (P = 0.001) compared to HMTC. The tumors were smaller in HMTC (P = 0.038). Overall 10-year survival in SMTC versus HMTC was 69 versus 93% (P = 0.12). On multivariate analysis, vascular invasion (hazard ratio 6.4, P = 0.019) was an adverse predictor for disease-free survival. HMTC in the era of RET analysis presents with a smaller primary tumor, lower preoperative serum calcitonin levels, and lower rates of lymph node metastasis. Mutations in the p18 gene were not a major factor in medullary thyroid carcinoma tumorigenesis.

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Acknowledgment

D.T.A. is a recipient of the Endeavour International Postgraduate Research Scholarship. S.S. is a NSW Cancer Institute Fellow.

Conflict of interest

The authors declare no conflict of interest.

Author information

Correspondence to Mark Sywak FRACS.

Additional information

M. Sywak and S. Sidhu contributed equally to this work.

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Abraham, D.T., Low, T., Messina, M. et al. Medullary Thyroid Carcinoma: Long-Term Outcomes of Surgical Treatment. Ann Surg Oncol 18, 219–225 (2011). https://doi.org/10.1245/s10434-010-1339-y

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Keywords

  • Medullary Thyroid Carcinoma
  • Central Neck
  • Serum Calcitonin
  • Sporadic Medullary Thyroid Carcinoma
  • Serum Calcitonin Level