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Single Gland, Ectopic Location: Adenomas are Common Causes of Primary Hyperparathyroidism in Children and Adolescents

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Abstract

Background

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Data-driven guidelines for management in pediatric patients are limited.

Methods

We performed a retrospective cohort analysis of all patients (1997–2017) with PHPT ≤ 21 years of age who underwent parathyroidectomy at three institutions. Clinical and demographic variables were analyzed. Primary operative outcome was cure (normocalcemia > 6 months after surgery); secondary outcome was operative success (intraoperative parathyroid hormone decrease of ≥ 50%).

Results

We identified 86 patients with a median age of 17 years (IQR: 14, 19); 64% (n = 55) were female. The mean preoperative serum calcium was 11.7 mg/dL, median parathyroid hormone (PTH) was 110 pg/mL, and median urine calcium was 4.1 mg/kg/24 h. Preoperatively, sestamibi scan localized in 41/71 patients (58%); neck ultrasound localized in 19/44 (43%). The most common pathology at surgery was a single ectopic parathyroid adenoma in 71% (n = 61). A high incidence of ectopic adenomas (25%, n = 22) was observed, most commonly intrathymic (n = 13), followed by tracheoesophageal groove (n = 5), carotid sheath (n = 2), and intrathyroidal (n = 2). Of 56 patients with retrievable data > 6 months postoperatively, cure was achieved in 55 of 56 patients (98%). One patient who presented to us with parathyromatosis require subsequent reoperation.

Conclusion

In this multi-institutional series of PHPT in children and adolescents, the majority were sporadic PHPT and were due to a single adenoma. We observed a high incidence of ectopic parathyroid adenomas, most commonly intrathymic. Given the high risk for ectopic adenoma in pediatric patients, parathyroid surgery in children and adolescents should be performed by experienced surgeons.

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Authors and Affiliations

  1. Department of Surgery, University of Tennessee Health Sciences Center, Chattanooga, TN, USA

    Robert D. Rampp & Vicente A. Mejia

  2. Department of Endocrinology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Edna E. Mancilla & Michael A. Levine

  3. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Edna E. Mancilla, N. Scott Adzick, Michael A. Levine, Rachel R. Kelz, Douglas L. Fraker & Heather Wachtel

  4. Department of Surgery, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    N. Scott Adzick

  5. Department of Surgery, Hospital of the University of Pennsylvania, 3400 Spruce Street, 4 Silverstein, Philadelphia, PA, 19104, USA

    Rachel R. Kelz, Douglas L. Fraker & Heather Wachtel

  6. Department of Endocrinology, Children’s of Alabama, Birmingham, AL, USA

    Pallavi Iyer

  7. Department of Surgery, University of Alabama at Birmingham, Birmingham, AL, USA

    Brenessa M. Lindeman & Herbert Chen

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  1. Robert D. Rampp
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Correspondence to Heather Wachtel.

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Rampp, R.D., Mancilla, E.E., Adzick, N.S. et al. Single Gland, Ectopic Location: Adenomas are Common Causes of Primary Hyperparathyroidism in Children and Adolescents. World J Surg 44, 1518–1525 (2020). https://doi.org/10.1007/s00268-019-05362-8

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