Craniopharyngioma

Question 1

Incidence rates of craniopharyngioma

Answer 1

6-9% of brain tumors - peak at 8-10 years of age

Question 2

Pathophysiology of craniopharyngioma

Answer 2

Lobulated masses with calcification and cystic components

Histologically benign but propensity to infiltrate surrounding tissue

Question 3

What are the presenting symptoms of craniopharyngioma

Answer 3

Short stature and other endocrine-related problems, symptoms of increased intracranial pressure, visual field disturbance and behavior symptoms

Question 4

Associated findings in craniopharyngioma

Answer 4

Hydrocephalus secondary to obstruction of the third ventricle and foramen of Monroe in 50% of patients

Question 5

Neuroanatomical findings of craniopharyngioma

Answer 5

Tends to attach to pituitary stalk, optic chasm, hypothalamus, and vessels of the circle of Willis

Also involves frontal lobe and diencephalon

Question 6

How is craniopharyngioma treated

Answer 6

Aggressive surgery - but can have significant morbidity

Currently opt for less aggressive surgery with focal radiotherapy

Chemotherapy has no established role in treatment

Question 7

Risks factors associated with long term effects for craniopharyngioma

Answer 7

Related to extent of surgery, shunt, subjects age, and volume of brain irradiated

Question 8

Neuropsychological deficits found in craniopharyngioma

Answer 8

Processing speed deficits and everyday memory problems (everything else is typically within normal limits)

Some studies have showed lower performance on memory and executive functioning (delayed recall severely impaired in a third of patients) - can see memory problems in both visuospatial and verbal domains

NP deficits are not always present

Question 9

Best outcome for surgery with craniopharyngioma

Answer 9

When tumor is pre-chiasmatic and extraventricular - can be completely excised without radiotherapy

Question 10

Best treatment for craniopharyngioma

Answer 10

Invasive stereotactic surgery and radiotherapy - less cognitive and endocrinology morbidity than more aggressive surgery (despite use of radiotherapy)

Question 11

Medical findings of craniopharyngioma

Answer 11

When hypothalamic involved - impaired obesity (peaks at 12 years), physical fatigue, reduced motivation, dyspnea (shortness of breath), and diarrhea

Question 12

New treatment findings for craniopharyngioma

Answer 12

Oxytocin might have a positive effect on emotion perception in patients with specific lesions of the anterior hypothalamic area