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Pituitary gigantism: a rare learning opportunity
  1. Owen Martyn Bendor-Samuel1,
  2. Aparna Pal2,
  3. Simon Cudlip3,
  4. Gemma Anderson4,
  5. Sanjay Salgia5,
  6. Tafadzwa Makaya1
  1. 1 Department of Endocrinology, Oxford Children’s Hospital, Oxford, UK
  2. 2 Oxford Centre for Diabetes, Endocrinology and Metabolism, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  3. 3 Department of Neurosurgery, Oxford University Hospitals NHS Foundation Trust, Oxford, Oxfordshire, UK
  4. 4 Oxford Centre for Diabetes Endocrinology and Metabolism, Oxford, UK
  5. 5 Paediatrics, Buckinghamshire Healthcare NHS Trust, Amersham, UK
  1. Correspondence to Dr Owen Martyn Bendor-Samuel, Department of Paediatric Endocrinology, Oxford Children’s Hospital, John Radcliffe Hospital, Oxford, OX3 9DU, UK; owenmbs{at}gmail.com

Abstract

Introduction Pituitary gigantism is a rare but significant paediatric condition with complexities surrounding diagnosis and management. Transsphenoidal surgery (TSS) is the treatment of choice; however, medical treatment is often considered as adjuvant therapy.

Case A 10½ -year-old boy presented with tall stature and a height velocity of 11 cm/year. His height was 178.7 cm (+5.8 SD above mean) and insulin-like growth factor-1 (IGF-1) was elevated. An oral glucose tolerance test demonstrated non-suppression of growth hormone (GH). Initial contrast MRI was inconclusive, but C-11 methionine functional positron emission tomography CT identified a 6 mm pituitary microadenoma. A multidisciplinary team clinic held with the family allowed discussion about medical and surgical treatment options. Due to a number of factors including the patient’s young age, prepubertal status, a wish to allow him to settle into his new high school and his desire to reach a final height taller than his father’s height, it was decided to try medical therapy first with a somatostatin analogue. Pubertal induction was also commenced and bilateral epiphysiodesis surgery performed. Initial response to octreotide was positive; however, 4 months into therapy his IGF-1 was climbing and a repeat GH profile was not fully suppressed. The patient therefore proceeded to have successful TSS excision of the adenoma.

Conclusion Rare cases such as this require sharing of knowledge and expertise, so the best possible care is offered. It is often necessary to work across sites and disciplines. Each case requires an individual approach tailored to the patient and their family.

  • gigantism
  • endocrinology
  • pituitary adenoma
  • pituitary gigantism
  • growth

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Footnotes

  • Contributors TM and OMB-S drafted the initial manuscript. Reviews and final draft approvals by TM, OMB-S, AP, GA, SS and SC. The patient is under the ongoing care of TM and SS.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.

  • Patient consent for publication Obtained.

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