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Craniopharyngioma

Summary

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  • Benign epithelial tumour arising from remnants of Rathke's pouch
  • Typically presents with visual disturbances, endocrine dysfunction, and increased intracranial pressure
  • Characteristic imaging findings include calcifications and cystic components in the sellar/suprasellar region

Pathophysiology

  • Derived from embryonic remnants of Rathke's pouch
  • Two histological subtypes:
    • Adamantinomatous: More common in children, contains calcifications and cysts
    • Papillary: More common in adults, typically solid
  • Growth pattern:
    • Slow-growing, but can cause significant local mass effect
    • May invade adjacent structures, including hypothalamus and optic chiasm

Demographics

  • Bimodal age distribution:
    • Peak in children aged 5-14 years
    • Second peak in adults aged 50-74 years
  • Accounts for 2-5% of all primary intracranial tumours
  • No significant gender predilection

Diagnosis

  • Clinical presentation:
    • Visual disturbances (e.g., bitemporal hemianopsia)
    • Endocrine dysfunction (e.g., growth hormone deficiency, diabetes insipidus)
    • Increased intracranial pressure (e.g., headaches, nausea, vomiting)
  • Hormonal evaluation:
    • Assessment of pituitary function (anterior and posterior)
  • Ophthalmological examination:
    • Visual field testing
    • Visual acuity assessment

Imaging

  • CT:
    • Calcifications in 90% of adamantinomatous type
    • Mixed solid and cystic components
    • Hyperdense solid portions
  • MRI:
    • T1-weighted:
    • Solid components: isointense to grey matter
    • Cystic components: variable signal intensity
    • T2-weighted:
    • Solid components: isointense to hypointense
    • Cystic components: hyperintense
    • Post-contrast:
    • Heterogeneous enhancement of solid portions
    • Rim enhancement of cystic components
  • Key imaging features:
    • "Lobulated" appearance
    • Calcifications (best seen on CT)
    • Suprasellar extension with displacement of optic chiasm

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  • A 20-year-old patient presented with a headache.
  • MRI showed a partially enhancing suprasellar mass lesion with large cystic components.

Treatment

  • Surgical resection:
    • Primary treatment modality
    • Gross total resection when feasible
    • Challenges include proximity to critical structures
  • Radiation therapy:
    • Adjuvant treatment for residual tumour
    • Stereotactic radiosurgery for small residual tumours
  • Intracystic therapies:
    • Bleomycin or interferon-alpha for recurrent cystic components
  • Hormone replacement therapy:
    • Management of endocrine dysfunction
  • Long-term follow-up:
    • Regular imaging surveillance
    • Endocrine and ophthalmological monitoring

Differential diagnosis

Differential Diagnosis Differentiating Feature
Pituitary adenoma Typically homogeneous on MRI; rarely calcified
Rathke's cleft cyst Unilocular cyst without solid component
Arachnoid cyst No calcification; follows CSF signal on all sequences
Germ cell tumour Typically midline; may have elevated tumour markers
Meningioma Dural tail sign; homogeneous enhancement
Optic pathway glioma Typically in children; involves optic chiasm/nerves
Hypothalamic glioma Infiltrative appearance; rare calcification
Langerhans cell histiocytosis Thickened pituitary stalk; diabetes insipidus common
Metastasis Multiple lesions; known primary malignancy
Aneurysm Flow voids on MRI; enhancement on angiography