Pediatric Craniopharyngioma Clinical Presentation

Updated: May 18, 2017
  • Author: Joseph L Lasky, III, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Presentation

History

Craniopharyngiomas produce symptoms by compression of adjacent neural structures. They can become quite large, obstructing cerebral spinal fluid (CSF) pathways (ie, third ventricle, Monro foramen) and causing hydrocephalus and increased intracranial pressure that leads to headaches, nausea, and projectile vomiting.

Symptoms at presentation may include the following:

  • Headache: Headaches occur in 60-80% of children with craniopharyngioma at presentation and are usually a symptom of increased intracranial pressure or hydrocephalus.
  • Vomiting: Classic projectile vomiting (frequently without nausea) accompanies headaches as a sign of increased intracranial pressure and is reported in 35-70% of children with these tumors at presentation.
  • Vision loss
    • Children are frequently unaware of significant vision loss; nevertheless, this symptom reportedly occurs in 20-60% of pediatric patients with craniopharyngioma at presentation.
    • Anterior extension to the optic chiasm can result in a classic bitemporal hemianopsia, unilateral temporal hemianopsia, papilledema, or unilateral/bilateral decrease in visual acuity. Classically, vision loss starts with a superior temporal field cut. However, the eccentric growth of these tumors can result in varying patterns and severity of vision loss, including decreased acuity, diplopia, blurred vision, and subjective visual field deficits. Children are frequently inattentive to visual loss, and formal testing may be required.
    • Symptoms related to endocrine dysfunction may be present prior to therapy. [2] This results from direct compression or destruction of the hypothalamus and pituitary stalk, leading to growth hormone deficiency, thyroid-stimulating hormone deficiency, adrenocorticotropic hormone deficiency, antidiuretic hormone deficiency, and luteinizing hormone or follicle-stimulating hormone deficiency (See below and Complications). This can present as clinically significant short stature, hypothyroidism, diabetes insipidus, and other signs of panhypopituitarism. [7]
  • Diencephalic syndrome: This term is used to describe emaciated hyperactive children who occasionally present with unusual eye movements and even blindness; these symptoms result from extrinsic compression of the hypothalamus. Conversely, damage to or invasion of the ventromedial hypothalamus can result in a dysregulation of energy balance and resultant obesity upon presentation.
  • Symptoms of growth hormone deficiency (ie, short stature): Growth hormone deficiency is the most common possible endocrinologic disturbance caused by craniopharyngiomas (35-95%). One series reported that growth failure preceded the diagnosis at a mean of 4 years.
  • Symptoms of hypothyroidism (present in 21-42% of cases)
    • Weight gain
    • Lethargy
    • Fatigue
    • Cold intolerance
    • Dry skin
    • Dry brittle hair
    • Slow teething
    • Anorexia
    • Large tongue
    • Deep voice
    • Myxedema
  • Symptoms of adrenal insufficiency: Secondary adrenal insufficiency (ACTH deficiency) is the second most common endocrinologic disturbance caused by craniopharyngiomas (21-62% of cases).
  • Symptoms of luteinizing hormone/follicle-stimulating hormone deficiency: Gonadotropin deficiency is the most common presenting symptom of craniopharyngioma in adults (38-82% of cases). As many as 100% of presenting adolescents may have complaints of delayed puberty. [7]

Mental status changes occur in as many as 25% of adults but are rare in children. Temporal lobe involvement can result in seizures, although this is rare.

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Physical

Focus physical examination on the identification of neurologic and endocrine derangements.

Papilledema

Papilledema occurs in 25-40% of children and results from increased intracranial pressure.

Visual field deficits

Formal testing is generally required to identify visual field deficits in children, which likely explains the wide reported range (10-95%) of patients with craniopharyngioma.

Given the typical proximity of the tumor to the optic nerves, optic chiasm, and anterior optic tracts, the common discovery of visual fields defects at presentation is not surprising.

See-saw nystagmus

Although often referred to as a classic physical examination finding among children with parasellar tumors, the literature reports an incidence rate of less than 10%.

Cranial nerve palsy

With the notable exception of the optic nerves, cranial nerve palsies are relatively rare, with a reported incidence rate of 8% for children at time of diagnosis.

Endocrine effects

Short stature or growth retardation is the most common endocrine derangement associated with this tumor. Growth retardation (as documented on formal pediatric growth charts) is reported in 86% of patients with craniopharyngioma at presentation.

Obesity and weight gain is the third most common endocrine abnormality associated with craniopharyngiomas. Hypothyroidism, growth hormone deficiency, and direct hypothalamic injury can contribute to obesity and weight gain. Obesity and weight gain are reported in 20% of presenting patients.

Hypothyroidism can manifest as weight gain, dry skin, brittle hair, and bradycardia.

Precocious or delayed puberty

Precocious or delayed puberty is the fourth most common endocrine derangement associated with craniopharyngiomas and is present at diagnosis in 10-15% of patients. This is the most common presenting sign in adolescents.

Intellectual or emotional disturbance and somnolence

These signs are most likely the result of either hydrocephalus or thyroid dysfunction.

Enlarging head circumference

This finding is highly suggestive of an intracranial mass or hydrocephalus, particularly when paired with papilledema.

Ataxia

This is another sign of increased intracranial pressure or hydrocephalus, which is present in 5-10% of patients at initial evaluation.

Seizures

These are rarely described as a presenting feature.

Focal motor weakness

This is also rarely described as a presenting feature.

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Causes

No known environmental or infectious causes predispose to the development of craniopharyngiomas.

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