What is the management plan for a 5-year-old female with a 2-week history of vision loss in the left eye and an MRI revealing a sellar tumor, likely a craniopharyngioma or pituitary adenoma?

Management of Sellar Tumor with Vision Loss in a 5-Year-Old

This 5-year-old with acute vision loss and a sellar tumor requires immediate multidisciplinary team evaluation at a specialized pediatric pituitary center, with urgent ophthalmologic assessment, comprehensive endocrine workup, and neurosurgical consultation for likely surgical intervention to preserve vision. 1

Immediate Priority: Vision Preservation

The 2-week history of unilateral vision loss represents an urgent ophthalmologic emergency requiring immediate action to prevent permanent visual damage. 1

• Formal visual field testing and ophthalmologic examination must be performed urgently to document baseline visual function and guide surgical timing 1
• Vision loss from mass effect on the optic apparatus is often partially reversible if decompression occurs promptly, making this a time-sensitive situation 1

Multidisciplinary Team Assembly

Immediate referral to a specialist center with pediatric pituitary expertise is mandatory (100% consensus recommendation). 1

The team must include:

• Pediatric endocrinologist with pituitary expertise 1
• Pediatric neurosurgeon or adult skull base surgeon experienced in pediatric cases 1
• Pediatric ophthalmologist for visual assessment 1
• Neuroradiologist for dedicated pituitary imaging interpretation 1
• Pediatric neuro-oncologist for overall coordination 1

Essential Pre-Operative Workup

Endocrine Evaluation

Complete pituitary hormone assessment is required before any intervention (strong recommendation, high-quality evidence). 1

Specific testing must include:

• Growth and pubertal assessment (critical in a 5-year-old) 1
• Morning cortisol and ACTH (assess for hypopituitarism or Cushing disease) 1
• Free T4 and TSH (thyroid axis evaluation) 1
• IGF-1 and GH (assess for GH excess or deficiency) 1
• Prolactin levels (may be elevated from stalk compression or prolactinoma) 1
• Electrolytes and osmolality (assess for diabetes insipidus) 1

Advanced Neuroimaging

Dedicated pituitary MRI protocol with contrast is essential for surgical planning. 1

• Imaging should assess tumor size, suprasellar extension, optic chiasm compression, cavernous sinus invasion, and relationship to critical vascular structures 1
• In this age group, craniopharyngioma is more likely than pituitary adenoma given the patient's young age (pituitary adenomas represent only 1% of intracranial neoplasms before age 15) 1

Genetic Considerations

Genetic assessment should be considered given the young age and increased likelihood of familial/syndromic disease in pediatric pituitary tumors compared to adults. 1

Surgical Management

Surgery is the primary treatment modality for a sellar mass causing vision loss in a child. 1

Surgical Approach

• Transsphenoidal surgery by an experienced pituitary surgeon is the preferred approach when anatomically feasible 1
• Surgery should be performed at a specialist center with pediatric pituitary expertise to optimize outcomes and minimize complications 1
• The goal is maximal safe resection while preserving pituitary function and protecting the optic apparatus 1

Intraoperative Considerations

• Tissue must be sent for histopathology to distinguish between craniopharyngioma, pituitary adenoma, or other sellar pathology 1
• Molecular characterization and Ki-67 labeling index should be obtained when possible 1

Post-Operative Management

Immediate Post-Operative Care

• Monitor for diabetes insipidus (common after sellar surgery in children) 1
• Reassess visual function within days of surgery 1
• Repeat endocrine testing to identify new hormone deficiencies 1
• Hormone replacement therapy as needed (cortisol replacement is life-saving if adrenal insufficiency develops) 1

Long-Term Follow-Up

Lifelong endocrine and imaging surveillance is required for pediatric sellar tumors. 1

• MRI surveillance at 3 months, 6 months, 1 year, 2 years, 3 years, and 5 years post-surgery 2
• Annual endocrine assessment for evolving hypopituitarism (incidence increases over time, reaching 80% by 10-15 years if radiotherapy is used) 1
• Growth monitoring is critical in this age group 1
• Planned transition to adult endocrine services as the patient matures 1

Radiotherapy Considerations

Radiotherapy should be reserved for recurrent or residual disease unresponsive to surgery, not as primary treatment. 1

Critical Cautions About Radiotherapy in Young Children

The 2024 consensus guidelines emphasize extreme caution with radiotherapy in children under age 30, particularly those under 10 years old. 1

Specific risks include:

• 2.4-fold increased risk of malignant brain tumors for every 10 years of younger age at time of radiotherapy 1
• Standardized incidence ratio of 658 for meningiomas after cranial radiotherapy in children 1, 2
• Universal GH deficiency by 5 years post-radiotherapy 1
• 80% incidence of multiple pituitary hormone deficiencies by 10-15 years post-radiotherapy 1
• Neurocognitive sequelae and cerebrovascular events 1

Registry Reporting

Report this case to an appropriate national pediatric pituitary tumor registry (strong recommendation, 90% consensus). 1

This facilitates outcome tracking and contributes to the limited evidence base for these rare tumors in children. 1