Craniopharyngioma Clinical Presentation

Updated: Dec 11, 2017
  • Author: George I Jallo, MD; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Presentation

History

Craniopharyngioma typically is a slow-growing tumor. Symptoms frequently develop insidiously and usually become obvious only after the tumor attains a diameter of about 3cm. The time interval between the onset of symptoms and diagnosis usually ranges from 1-2 years.

The most common presenting symptoms are headache (55-86%), endocrine dysfunction (66-90%), and visual disturbances (37-68%). Headache is slowly progressive, dull, continuous, and positional; it becomes severe in most patients when endocrine symptoms become obvious.

On presentation, 40% of patients have symptoms related to hypothyroidism (i.e., weight gain, fatigue, cold intolerance, constipation). Almost 25% have associated signs and symptoms of adrenal failure (i.e., orthostatic hypotension, hypoglycemia, hyperkalemia, cardiac arrhythmias, lethargy, confusion, anorexia, nausea, and vomiting), and 20% have diabetes insipidus (i.e. excessive fluid intake and urination). Most young patients present with growth failure and delayed puberty. [21]

As disease progress, 80% of adults complain of decreased sexual drive, and almost 90% of men complain of impotence, while most women complain of amenorrhea.

Optic pathway dysfunction is noted in 40-70% of patients on presentation. Children rarely become aware of visual problems (only 20-30%) and often present after almost complete visual damage becomes irreversible. The manifestation of optic pathway dysfunction usually varies from papilledema to visual field deficits and even optic nerve atrophy in severe cases.

Other manifestations relate to the various connections of the hypothalamic-pituitary complex and surrounding structures. When the thalamus, hypothalamus and frontal lobes are affected, patients experience endocrine, autonomic, and behavioral problems (i.e. hyperphagia and obesity, psychomotor retardation, emotional immaturity, apathy, short-term memory deficits, incontinence). [22]  Short stature is present in 23-45% of patients, and obesity affects 11-18% of patients. [23]

The following relationships are seen between the anatomic location of the craniopharyngioma and major clinical syndromes:

  • Prechiasmal localization - Typically results in associated findings of optic atrophy (i.e., progressive decline of visual acuity, constriction of visual fields). The chiasm stretches and gets displaced away from the optic nerve, which eventually causes vascular deprivation of the nerves and damage to nerve fibers.
  • Retrochiasmal location - Commonly is associated with hydrocephalus with signs of increased intracranial pressure (i.e. papilledema, horizontal double vision). The tumor compresses the floor of the third ventricle and can lead to obstruction of CSF flow through the Foramen of Monroe.
  • Intrasellar craniopharyngioma - Usually manifests as headache and/or endocrinopathy.
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Physical Examination

Neurologic and general examinations are both indicated.

Neurologic examination

Signs suggestive of increased intracranial pressure—horizontal double vision (unilateral/bilateral) and papilledema (unilateral/bilateral)—should be sought for in any patient suspected of having an intracranial mass.

Visual field examination may reveal various patterns of visual loss (most frequently bitemporal hemianopsia) suggestive of involvement (i.e., compression) of the optic chiasm and/or tracts. Formal visual field testing by ophthalmology is recommended as part of the initial work up and serial testing can be used in follow up to monitor tumor growth/recurrence.

General examination

Signs and symptoms may be related to various endocrinopathies.

Hypothyroidism

Symptoms of hypothyroidism include the following:

   ▪     Puffiness and non-pitting edema

   ▪     Slow return phase of deep tendon reflexes

   ▪     Hypoventilation and decrease in cardiac output

   ▪     Pericardial and pleural effusions

   ▪     Constipation

   ▪     Anemia – i.e., normochromic normocytic anemia

   ▪     Decreased mental function

   ▪     Psychiatric changes

Cortisol-related deficiency 

The signs and symptoms of cortisol deficiency include the following:

  • Hypotension, which is often orthostatic
  • Gastrointestinal symptoms, which include anorexia, nausea, and vomiting
  • Weight loss
  • Hypoglycemia
  • Lethargy
  • Confusion
  • Psychological disturbances, i.e.. psychosis and intolerance to stress

Changes in volume and sodium control

The signs and symptoms of aldosterone deficiency include the following:

   ▪     Hypovolemia

   ▪     Decreased cardiac output

   ▪     Decreased renal blood flow with azotemia

   ▪     Fatigue

   ▪     Weight loss

   ▪     Cardiac arrhythmias due to hyperkalemia

Compression of the infundibulum can lead to the common presentation of diabetes insipidus.

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