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Long-Term Oncologic Outcomes After Curative Resection of Familial Medullary Thyroid Carcinoma

  • Endocrine Tumors
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Introduction

Long-term outcomes after curative resection in patients with germline RET mutations and medullary thyroid cancer (MTC) are highly variable and mutation-specific oncologic outcomes are not well-described.

Methods

Sixty-six patients identified from 1986 to 2017 from a single-institution cancer database were assessed for recurrence and survival using Kaplan–Meier estimates, and correlated with clinicopathologic features using log-rank or Cox proportional hazards.

Results

Median follow-up was 9.3 years (range 0.3–31.5), median tumor diameter was 1.5 cm (range 0.1–7.5), and preoperative calcitonin was known in 41 patients [median 636 (range 0–9600)]. Overall survival (OS) of the cohort was 94% at 10 years, the cumulative incidence of locoregional recurrence was 38% at 10 years, and 19/24 (79%) patients underwent repeat neck operation. The cumulative incidence of distant recurrence was 27% at 10 years. Predictors of distant recurrence were tumor size, positive lymph nodes, and pre- and postoperative carcinoembryonic antigen, but not calcitonin. M918T mutation-bearing patients had 10-year distant recurrence-free survival of 0%, compared with 83% in all other patients (p < 0.001), and equivalent 10-year OS (100% vs. 92%; p = 0.49).

Conclusions

Structural and metastatic recurrence is common in patients with germline RET mutations, and MTC and can occur 20 years after initial treatment, however survival remains high. Management should focus on optimal surveillance strategies and long-term control of structural disease.

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Acknowledgments

This work was funded in part by NIH/NCI Cancer Center Support Grant No. P30 CA008748.

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Authors and Affiliations

  1. Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Philip M. Spanheimer MD, Ian Ganly MD, PhD, Richard J. Wong MD, Ashok R. Shaha MD & Brian R. Untch MD

  2. Department of Biostatistics, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Joanne Chou MS & Marinela Capanu PhD

  3. Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Ronald A. Ghossein MD

  4. Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    R. Michael Tuttle MD

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  1. Philip M. Spanheimer MD
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  2. Ian Ganly MD, PhD
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  4. Marinela Capanu PhD
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  9. Brian R. Untch MD
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Correspondence to Brian R. Untch MD.

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Philip M. Spanheimer, Ian Ganly, Joanne Chou, Marinela Capanu, Ronald A. Ghossein, R. Michael Tuttle, Richard J. Wong, Ashok R. Shaha, and Brian R. Untch have no relevant financial interests to disclose.

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Spanheimer, P.M., Ganly, I., Chou, J. et al. Long-Term Oncologic Outcomes After Curative Resection of Familial Medullary Thyroid Carcinoma. Ann Surg Oncol 26, 4423–4429 (2019). https://doi.org/10.1245/s10434-019-07869-9

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  • DOI: https://doi.org/10.1245/s10434-019-07869-9

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