An association of craniopharyngioma in Turner syndrome.

Pediatr Blood Cancer

Department of Endocrinology and Diabetes, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, United Kingdom.

Published: June 2013

AI Article Synopsis

  • Turner Syndrome (TS) and craniopharyngioma (CP) are rare conditions, with TS affecting about 1 in 5,000 births and CP 1 in 50,000 children.
  • Three cases of TS with CP were presented, marking a new association not previously documented, where symptoms like visual failure, poor growth, or headaches led to MRI diagnoses of CP.
  • All patients developed hypopituitarism after surgery for CP, highlighting the need for clinicians to conduct urgent MRI scans in TS cases presenting with related symptoms.

Article Abstract

Turner syndrome (TS) (approximately 1:5,000 births) and craniopharyngioma (CP) (1:50,000 children) are both rare conditions. We present three cases of TS with CP, an association not previously described. Visual failure, poor growth or headache led to MRI diagnosis of CP. Whilst two had evidence of hypopituitarism at diagnosis of CP, they all developed hypopituitarism following surgical debulking. Two required radiotherapy due to regrowth. Whether CP and TS share a similar aetiology is unknown. Clinicians need to be aware of this association, and should perform urgent MRI scanning in TS patients with headache, visual impairment or clinical/biochemical evidence of hypopituitarism.

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Source
http://dx.doi.org/10.1002/pbc.24411DOI Listing

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