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Role of prophylactic thyroidectomy in RET 790 familial medullary thyroid carcinoma

Authors

  • Hélène Bihan MD,

    1. Department of Endocrinology, Diabetology and Metabolic Disease, Avicenne Hospital, Paris XIII University and Assistance Publique–Hôpitaux de Paris, Bobigny, France
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  • Eric Baudin MD,

    1. Departments of Nuclear Medicine and Endocrine Tumors, Anatomopathology and Surgery, Institut Gustave Roussy, Villejuif, France
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  • Taly Meas MD,

    1. Departments of Internal Medicine B and Surgery, Hôpital Lariboisière, Paris, France
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  • Sophie Leboulleux MD,

    1. Departments of Nuclear Medicine and Endocrine Tumors, Anatomopathology and Surgery, Institut Gustave Roussy, Villejuif, France
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  • Abir Al Ghuzlan MD,

    1. Departments of Nuclear Medicine and Endocrine Tumors, Anatomopathology and Surgery, Institut Gustave Roussy, Villejuif, France
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  • Véronique Hannoteaux MD,

    1. Department of Endocrinology, Diabetology and Metabolic Disease, Avicenne Hospital, Paris XIII University and Assistance Publique–Hôpitaux de Paris, Bobigny, France
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  • Jean-Paul Travagli MD,

    1. Departments of Nuclear Medicine and Endocrine Tumors, Anatomopathology and Surgery, Institut Gustave Roussy, Villejuif, France
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  • Patrice Valleur MD,

    1. Departments of Internal Medicine B and Surgery, Hôpital Lariboisière, Paris, France
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  • Pierre-Jean Guillausseau MD, PhD,

    1. Departments of Internal Medicine B and Surgery, Hôpital Lariboisière, Paris, France
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  • Régis Cohen MD, PhD,

    Corresponding author
    1. Department of Endocrinology, Diabetology and Metabolic Disease, Avicenne Hospital, Paris XIII University and Assistance Publique–Hôpitaux de Paris, Bobigny, France
    • Department of Endocrinology, Diabetology and Metabolic Disease, Avicenne Hospital, Paris XIII University and Assistance Publique–Hôpitaux de Paris, Bobigny, France
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  • for the French Group of Endocrine Tumors (GTE)


Abstract

Background

We describe a family harboring RET 790 mutation and review the role of prophylactic thyroidectomy for medullary thyroid carcinoma.

Methods

We evaluated in detail both clinical and biological follow-up and reviewed literature reports.

Results

Among 86 family members, 15 of 22 members screened harbored the 790 mutation. Abnormal calcitonin levels were found in 8/15. Total thyroidectomy with lymph node dissection cured the 5 operated patients (range, 45–76 years). Tumor staging was pT1N0M0. Among 10 carriers who did not undergo surgery, 3 patients had abnormal calcitonin levels. For the others, calcitonin levels remained <30 pg/mL. Two asymptomatic carriers were older than 70 years. Four subjects were lost to follow-up.

Conclusions

In RET codon 790 mutations families, a case-by-case decision instead of systematic prophylactic thyroidectomy should be discussed. Difficulties of follow-up should be taken into account and represent the main challenge. © 2011 Wiley Periodicals, Inc. Head Neck, 2012

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