Craniopharyngioma
Craniopharyngioma is a slow-growing, encapsulated, often calcified squamous epithelial tumor arising from Rathke pouch remnants along the pituitary stalk, with a bimodal peak at ages 5 to 15 and 45 to 60. It is the most common extrasellar cause of hypothalamic-pituitary failure and presents with headache, bitemporal hemianopsia, hypopituitarism, central diabetes insipidus, hypothalamic obesity, or obstructive hydrocephalus; treatment is surgical resection or decompression with postoperative radiotherapy for residual tumor, plus lifelong replacement of every deficient axis, starting with hydrocortisone.
- Topic reviewThe full article, organized the way the exam asks it.
- ABIM-format MCQsVignettes with an explanation for every wrong answer.
- Key factsThe numbers and thresholds worth memorizing, up front.
- AI tutorMedBrain answers the question you're stuck on.
What this article covers
- Overview
Definition: craniopharyngioma is a slow-growing, encapsulated squamous cell tumor originating from epithelial remnants of Rathke pouch within the pituitary stalk; it is histologically benign but associated with a high recurrence rate.
- Epidemiology
Most common tumor of the pituitary region in childhood and the most common supratentorial brain tumor in children, an exception to the infratentorial predominance of pediatric brain tumors.
- Risk factors
Age in either peak (5 to 15 years or 45 to 60 years) is the main association.
- Pathophysiology
Rathke pouch is an evagination of oral (surface) ectoderm that forms the anterior pituitary; epithelial remnants left along the pituitary stalk can give rise to craniopharyngioma.
- Clinical features
Mass effect is the presenting hallmark, after years of subtle symptoms: progressive headache and visual field defects, classically bitemporal hemianopsia from optic chiasm compression; papilledema…
- Differential diagnosis
Nonfunctioning pituitary adenoma: the most common sellar mass, solid and arising within the sella; calcification is common in craniopharyngioma and distinguishes it from adenoma.
- Diagnosis
The diagnosis is established by imaging and confirmed histologically: a sellar or suprasellar mass with cystic and solid components on MRI plus calcification (best seen on CT) and low attenuation…
- Management
First, if there is obstructive hydrocephalus with raised intracranial pressure, obtain emergent neurosurgical decompression; a ventriculoperitoneal shunt treats the obstructive hydrocephalus.
- Complications
Adrenal crisis from unrecognized central adrenal insufficiency, particularly perioperatively or when levothyroxine is started before hydrocortisone.
- Prognosis
Prognosis is variable; many survivors have lasting endocrine or visual deficits from the tumor's location.
- Prevention
Screen every patient before surgery with a morning cortisol: below 3 mcg/dL treat as deficient, 3 to 15 mcg/dL perform an ACTH stimulation test, and above 15 mcg/dL deficiency is excluded.
Read the whole article
Subscribing to Internal Medicine opens every article, the full question bank and MedBrain, on the web, iPhone and Android.
Related articles
- Panhypopituitarism
Panhypopituitarism is loss of adequate secretion of all anterior pituitary hormones, most often from a large pituitary tumor, apoplexy, Sheehan syndrome, or pituitary surgery. Diagnosis requires biochemical documentation of each axis plus pituitary imaging. Every patient needs lifelong hydrocortisone and levothyroxine replacement, with glucocorticoid always started before, or together with, thyroid hormone to avoid precipitating adrenal crisis.
- Central diabetes insipidus
Central diabetes insipidus is inability of the posterior pituitary to produce adequate antidiuretic hormone, causing free water loss, polyuria, and hypernatremia; common causes include pituitary/hypothalamic tumor, surgery, trauma, and infiltrative disease. Diagnosis is confirmed with a water deprivation test followed by a desmopressin challenge, and treatment is desmopressin (DDAVP) 10-40 mcg intranasally or 0.1-0.4 mg orally, dosed once nightly or twice daily and titrated to control polyuria while avoiding dilutional hyponatremia from over-replacement.
- Secondary adrenal insufficiency
Secondary adrenal insufficiency is ACTH deficiency from pituitary or hypothalamic disease, or most commonly from withdrawal of prolonged exogenous glucocorticoid therapy, causing isolated cortisol deficiency without hyperpigmentation or hyperkalemia because aldosterone secretion is preserved. Lifelong glucocorticoid replacement with hydrocortisone, with dose increases during illness, prevents adrenal crisis.
- Growth hormone deficiency
Growth hormone (GH) deficiency causes short stature in children and a nonspecific syndrome of fatigue, reduced muscle mass, and adverse body composition in adults, usually from hypothalamic-pituitary disease, surgery, irradiation, or head trauma. Diagnosis relies on a low age-adjusted IGF-1 with confirmatory provocative testing when needed, and treatment is daily subcutaneous somatropin titrated to a normal IGF-1, avoided in active malignancy or an untreated pituitary tumor.
- Nonfunctioning pituitary adenoma
A nonfunctioning pituitary adenoma is a benign pituitary tumor, usually of gonadotroph origin, that does not secrete a clinically excessive hormone and presents through mass effect (visual field loss, headache) or hypopituitarism rather than a hypersecretory syndrome. Diagnosis requires excluding hormone excess and screening for hypopituitarism; treatment is observation for a small asymptomatic incidentaloma or transsphenoidal surgery for mass effect, apoplexy, or growth on follow-up.
- Prolactinoma
Prolactinoma is a prolactin-secreting pituitary adenoma, the most common functional pituitary tumor, presenting with hypogonadism and galactorrhea from prolactin excess or with headache and visual loss from mass effect in larger tumors. Diagnosis requires an elevated prolactin with a pituitary mass on dedicated MRI after excluding pregnancy, medication effect, and hypothyroidism, and first-line treatment is a dopamine agonist, most often cabergoline, which lowers prolactin and shrinks the tumor without surgery in most patients.
- Syndrome of inappropriate antidiuretic hormone (SIADH)
SIADH is euvolemic hypotonic hyponatremia caused by antidiuretic hormone secretion or action that is inappropriate for the patient's osmotic and volume status. First-line treatment is fluid restriction to about 800-1,000 mL/day, with vasopressin receptor antagonists (vaptans) or hypertonic saline reserved for severe or symptomatic hyponatremia resistant to fluid restriction.
- Obesity
Obesity is a chronic disease of excess adiposity, defined by a body mass index of 30 or greater, that raises risk for type 2 diabetes, cardiovascular disease, sleep apnea, liver disease, and several cancers. Management is staged: lifestyle intervention for all patients, pharmacotherapy after 3 to 6 months of unmet lifestyle goals, and bariatric surgery for a body mass index of 40 or greater or 35 or greater with a comorbidity.