Craniopharyngioma and Cushing disease: case report

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Adrian Caceres1, Aaron J Reitman, Tadanori Tomita

  • 1Neurosurgery Division, Children's Memorial Hospital, Chicago, Illinois 60614, USA.

Journal of Neurosurgery
|May 11, 2005
PubMed

Insights

This study details a child with short stature and Cushing disease (CD) caused by a craniopharyngioma. The tumor secreted ACTH, leading to CD, which resolved after surgical resection.

Area of Science:

  • Pediatric Endocrinology
  • Pediatric Neurosurgery
  • Oncology

Background:

  • Craniopharyngioma is a common pediatric sellar tumor.
  • Typically presents with endocrinopathies (short stature, delayed puberty, obesity) and visual deficits.
  • Hydrocephalus is a frequent complication.

Observation:

  • A child presented with short stature and clinical Cushing disease (CD).
  • Imaging revealed a suprasellar tumor associated with high ACTH levels in serum and cyst fluid.
  • An Ommaya reservoir was inserted for tumor decompression.

Findings:

  • Pathology confirmed adamantinomatous craniopharyngioma.
  • Post-resection, the patient developed panhypopituitarism, requiring hormone replacement.
  • Tumor immunohistochemistry did not show ACTH staining.

Implications:

  • This case highlights a rare presentation of craniopharyngioma causing ectopic ACTH production and Cushing disease.
  • Discusses neurosurgical management strategies for sellar tumors with hormonal activity.
  • Explores potential mechanisms for ACTH secretion by craniopharyngioma.

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