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Ultrasonography Should Not Guide the Timing of Thyroidectomy in Pediatric Patients Diagnosed with Multiple Endocrine Neoplasia Syndrome 2A through Genetic Screening

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

Abstract

Background

American Thyroid Association (ATA) guidelines suggest that thyroidectomy can be delayed in some children with multiple endocrine neoplasia syndrome 2A (MEN2A) if serum calcitonin (Ct) and neck ultrasonography (US) are normal. We hypothesized that normal US would not exclude a final pathology diagnosis of medullary thyroid cancer (MTC).

Methods

We retrospectively queried a MEN2A database for patients aged <18 years, diagnosed through genetic screening, who underwent preoperative US and thyroidectomy at our institution, comparing preoperative US and Ct results with pathologic findings.

Results

35 eligible patients underwent surgery at median age of 6.3 (range 3.0–13.8) years. Mean MTC size was 2.9 (range 0.5–6.0) mm. The sensitivity of a US lesion ≥5 mm in predicting MTC was 13 % [95 % confidence interval (CI) 2 %, 40 %], and the specificity was 95 % [95 % CI 75 %, 100 %]. Elevated Ct predicted MTC in 13/15 patients (sensitivity 87 % [95 % CI 60 %, 98 %], specificity 35 % [95 % CI 15 %, 59 %]). The area under the receiver operating characteristic curve (AUC) for using US lesion of any size to predict MTC was 0.50 [95 % CI 0.33, 0.66], suggesting that US size has poor ability to discriminate MTC from non-MTC cases. The AUC for Ct level at 0.65 [95 % CI 0.46, 0.85] was better than that of US but not age [AUC 0.62, 95 % CI 0.42, 0.82].

Conclusions

In asymptomatic children with MEN2A diagnosed by genetic screening, preoperative thyroid US was not sensitive in identifying MTC of any size and, when determining the age for surgery, should not be used to predict microscopic MTC.

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Acknowledgment

The University of Texas MD Anderson Cancer Center is supported in part by a cancer center support grant (CA16672) from the National Institutes of Health. Dr. Grubbs was supported by an American Cancer Society Mentored Research Scholar Grant for MEN2 (121138MRSGM1112901).

Disclosures

We have no financial or commercial interests to disclose.

Author information

Authors and Affiliations

  1. Section of Surgical Endocrinology, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Lilah F. Morris MD, Jeff E. Lee MD, Thereasa A. Rich MS, Nancy D. Perrier MD & Elizabeth G. Grubbs MD

  2. Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Steven G. Waguespack MD & Anita K. Ying MD

  3. Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Beth S. Edeiken-Monroe MD

  4. Department of Biostatistics, The University of Texas MD Anderson Cancer, Houston, TX, USA

    Carla L. Warneke MS

  5. Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, USA

    Douglas B. Evans MD

Authors
  1. Lilah F. Morris MD
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  2. Steven G. Waguespack MD
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  3. Beth S. Edeiken-Monroe MD
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  4. Jeff E. Lee MD
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  5. Thereasa A. Rich MS
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  6. Anita K. Ying MD
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  7. Carla L. Warneke MS
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  8. Douglas B. Evans MD
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  9. Nancy D. Perrier MD
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  10. Elizabeth G. Grubbs MD
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Corresponding author

Correspondence to Elizabeth G. Grubbs MD.

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Morris, L.F., Waguespack, S.G., Edeiken-Monroe, B.S. et al. Ultrasonography Should Not Guide the Timing of Thyroidectomy in Pediatric Patients Diagnosed with Multiple Endocrine Neoplasia Syndrome 2A through Genetic Screening. Ann Surg Oncol 20, 53–59 (2013). https://doi.org/10.1245/s10434-012-2589-7

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  • DOI: https://doi.org/10.1245/s10434-012-2589-7

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