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Craniopharyngioma (Comprehensive)

Sellar/Suprasellar Epithelial Tumor

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Craniopharyngioma (Comprehensive)?

Slow-growing, benign (WHO grade I) epithelial tumor of sellar/suprasellar region.

Two histological types: adamantinomatous (children, BRAF-wild, CTNNB1 mutations) and papillary (adults, BRAF V600E mutations).

Bimodal age distribution: 5-14 years and 50-74 years.

Often calcified, cystic-solid mixed lesion compressing optic chiasm, pituitary stalk, and hypothalamus.

Symptoms

Bitemporal hemianopsia and visual acuity loss.
Headache (often morning, due to increased intracranial pressure).
Growth failure and delayed puberty in children.
Polyuria and polydipsia (diabetes insipidus).
Hypopituitarism: fatigue, cold intolerance, hypotension, amenorrhea.
Obesity and hypothalamic syndrome (hyperphagia, sleep disturbance).
Cognitive and behavioral changes.

Risk Factors

Embryologic remnants of craniopharyngeal duct (Rathke's pouch).
BRAF V600E mutation (papillary type).
Beta-catenin (CTNNB1) mutation (adamantinomatous type).
No clear environmental or hereditary risk factors.

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent morning headaches with vomiting.
  • Visual field defects or worsening vision.
  • Excessive thirst and urination.
  • Growth arrest in pediatric patients.
  • Unexplained hormonal deficiencies (hypothyroidism, adrenal insufficiency).

Treatment Methods

01
Surgical resection (transsphenoidal or transcranial approach) is primary treatment.
02
Adjuvant radiotherapy (conventional fractionated, stereotactic, or proton beam) for residual/recurrent disease.
03
Intracystic therapy (interferon-alpha, bleomycin) for cystic recurrences.
04
BRAF/MEK inhibitors (dabrafenib + trametinib) for BRAF-mutated papillary type.
05
Hormone replacement therapy: levothyroxine, hydrocortisone, growth hormone, sex steroids, desmopressin (DDAVP) for diabetes insipidus.
06
Multidisciplinary follow-up: neurosurgery, endocrinology, ophthalmology, pediatrics.

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.