Craniopharyngioma

    • Congenital tumorous remnant of pharyngeal epithelium centered above the sella turcica
    • Diagnosis is usually made in childhood, but adults are also at risk
    • Adamantinomatous tumors affect children, papillary tumors affect adults
    • Brain MRI shows distinctive features
    • Surgical resection is used to confirm histology and reduce tumor bulk
    • Radiation therapy is used to prevent further growth
    • Trap: recurrent acute visual loss from tumor cyst formation occurs frequently and must be treated promptly with surgery to prevent permanent vision loss
    • Core clinical features
      • Reduced visual acuity
      • Hemianopic visual field defect is usually present in at least one eye
      • Afferent pupil defect
      • Optic disc appears normal, small, or pale
    • Possible accompanying clinical features
      • Headache, hypopituitarism (especially growth retardation), papilledema, and ventriculomegaly are especially common manifestations in children
      • Hypopituitarism is the most common manifestation in adults
    • Imaging features
      • Epicenter is suprasellar
      • Trap: the tumor may compress the pituitary gland enough to make the gland invisible, as this picture shows
      • Heterogeneous signal intensity, often with cysts
      • Calcification is evident on CT in children
    • Rathke cleft cyst, which has the same embryologic origin but thinner walls than craniopharyngioma
    • Other masses in the sellar region
    • Arachnoid cyst
    • Epidermoid cyst
    • Langerhans cell histiocytosis
    • Sphenoid sinusitis or mucocele
    • Lymphocytic hypophysitis
    • Pituitary abscess
    • Multiple sclerosis
    • Neuromyelitis optica
    • Sarcoidosis
    • Radiation damage
    • Order MRI, which usually shows distinctive features
    • Order CT, which often shows tumor calcium in children but not in adults
    • Order pituitary hormone tests
    • Refer to a neurosurgeon experienced in craniopharyngioma surgery
    • If untreated, progressive vision loss is common
    • Surgery confirms the histology and reduces the tumor burden so that radiation therapy can be more effective
    • Trap: aggressive tumor resection often causes permanent loss of vision
    • Ventriculoperitoneal shunt is necessary if the tumor obstructs ventricular outflow
    • High radiation total dose (>5000 cGy) is necessary to prevent further tumor growth, but…
    • Trap: radiation therapy may not prevent recurrent cyst formation and may eventually damage the optic nerves/optic chiasm, cause encephalopathy, vasculopathy, and hypothalamic/pituitary dysfunction (see Radiation-induced Optic Neuropathy )
    • Endoscopic surgery is often effective in relieving recurrent visual loss from cyst expansion but may have to be performed repeatedly
    • Tip: advise patients with known craniopharyngiomas to contact a vision care provider promptly if vision loss occurs, as earlier decompression prevents permanent vision loss from tumor cyst expansion
    • Hypopituitarism requires intensive endocrinologic care

    Optic Nerve And Chiasm Disorders

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