Tumor growth characteristics vary considerably and this influences the clinical presentation.
Some people live progression-free lives without treatment, whereas others have continued tumor progression despite extensive treatment.
Visual and endocrine abnormalities are the most common symptoms at presentation4.
Symptoms may be the result of:
- compression on the neural structures and/or
- obstruction of CSF causing increased intracranial pressure (ICP)4,13.
Symptoms at presentation may include:
- Those of raised intracranial pressure
- Headache
- Vomiting (classic projectile vomiting)
- Visual loss
- Those of pressure on critical adjacent neural structures4,9,13:
Summary of craniopharyngioma symptoms secondary to pressure on adjacent neural structures:
Hypothalamus/Pituitary Axis |
|
Optic pathways |
|
Frontal lobes |
|
Temporal lobes |
|
Visual field deficits
- Often go unnoticed initially, usually picked up through routine eye checkups at school.
- Patients may notice:
- decreased clarity of vision
- diplopia
- blurred vision
- subjective visual field defects4,13
- Visual field defect:
- Bitemporal hemianopsia (may be unilateral) is most common,
following by homonymous hemianopsia and homonymous
quadrantanopia18.
- Bitemporal hemianopsia (may be unilateral) is most common,
- When unilateral loss of vision occurs with optic atrophy, the tumor is most likely anterior and lateral to the chiasm.
- Due to the slow, progressive nature of the tumor, patients may not complain of visual loss13,18.
Endocrine Dysfunction
Hormonal abnormalities occur in 43-90% of children at the time of diagnosis of a craniopharyngioma13
The following endocrine problems may occur:
- Growth hormone (GH) deficiency results in short stature
- Luteinizing hormone (LH)/follicle-stimulating hormone (FSH) deficiency may lead to delayed or arrested puberty
- Adrenocorticotropic hormone (ACTH) deficiency
- TSH deficiency and hypothyroidism leads to poor growth, cold intolerance and fatigability
- Hyperprolactinemia
- Diabetes insipidus
Symptoms and tumor location:
Symptoms often depend on tumor location:
Intrasellar tumors:
Often associated with endocrinopathy and headaches.
- Panhypopituitarism (growth hormone and ACTH deficiency very common)
- Diabetes insipidus
Prechiasmic lesions:
Associated with optic atrophy
- Decreased visual field acuity
- Constriction of the visual fields.
Retrochiasmal lesions:
May grow into the third ventricle causing:
- Hydrocephalus
- Compression of the optic tracts
- May also extend superiorly into the hypothalamus.
Neurological sequelae increase drastically with increase in tumor size. This is particularly true when the tumor grows into the cerebral parenchyma.
The following three clinical syndromes are related to anatomic location:
Tumor Location |
Associated Symptoms |
Prechiasmic |
|
Retrochiasmal |
|
Intrasellar |
|
