Investigations

1st investigations to order

ophthalmological evaluation; computerised visual-field examination

Test
Result
Test

Performed in all patients with craniopharyngioma with suprasellar extension and chiasmal compression.

Result

may reveal unilateral or bitemporal hemianopsia

MRI brain (contrast-enhanced)

Test
Result
Test

Most sensitive and specific imaging modality. [Figure caption and citation for the preceding image starts]: Craniopharyngioma: coronal post-contrast MRIFrom the collection of Marc C. Chamberlain [Citation ends].com.bmj.content.model.Caption@272e21d2[Figure caption and citation for the preceding image starts]: Craniopharyngioma: axial post-contrast MRIFrom the collection of Marc C. Chamberlain; used with permission [Citation ends].com.bmj.content.model.Caption@1820a1ea[Figure caption and citation for the preceding image starts]: Craniopharyngioma: sagittal post-contrast MRIFrom the collection of Marc C. Chamberlain; used with permission [Citation ends].com.bmj.content.model.Caption@36fa072c[Figure caption and citation for the preceding image starts]: Craniopharyngioma: sagittal post-contrast MRIFrom the collection of Marc C. Chamberlain [Citation ends].com.bmj.content.model.Caption@12a321a

Allows the clinician to define the size, location, and relationship of the tumour to surrounding structures; to determine the surgical approach; to assess the extent of resection; and to plan radiotherapy.

Result

variable; T1-weighted imaging may show hyperintensity secondary to high protein content in cystic component; contrast-enhanced sequences show enhancement of the solid component and cyst wall in mixed solid-cystic lesions; T2-weighted imaging and fluid-attenuated inversion recovery (FLAIR) show heterogeneous signal in the solid components and cyst hyperintensity; calcification is hypointense on T2-weighted imaging

CT brain (contrast-enhanced)

Test
Result
Test

Can be helpful in reaching differential diagnosis given the classic calcifications that can be found in craniopharyngiomas. Also used in evaluating relevant sinus anatomy and relationships.

Result

frequent tumour calcification (90% children; 70% adults); mixed cystic and solid mass with enhancement of the solid component and cyst wall

serum prolactin

Test
Result
Test

Increased secretion is due to tumour compression of the pituitary stalk. Measurement of a fasting sample is desirable.

Result

variable; commonly elevated

serum insulin-like growth factor 1

Test
Result
Test

Values of insulin-like growth factor 1 and its binding protein (IGFBP3) more than two standard deviation scores below the mean, corrected for age and sex, are indicative of growth hormone deficiency; however, normal concentrations do not rule out growth hormone deficiency (e.g., in post-irradiation patients).

Result

variable; commonly low

growth hormone (GH) stimulation test

Test
Result
Test

GH deficiency is best evaluated with dynamic testing including either an insulin tolerance test (ITT), glucagon stimulation test, or a growth hormone-releasing hormone/arginine stimulation test, or macimorelin test. ITT is considered the most specific and sensitive test for evaluation of the hypothalamic-pituitary-growth hormone axis. However, it needs to be performed by experienced clinicians and is usually not needed for everyday clinical practice.

Result

variable; commonly may show failure to induce GH

serum luteinising hormone

Test
Result
Test

Used to diagnose gonadotrophin hormone deficiency.

Note that in women a normal menstrual cycle is a more sensitive indicator of an intact pituitary and normal gonadal function than any biochemical test.

Result

variable; commonly low

serum follicle-stimulating hormone

Test
Result
Test

Used to diagnose gonadotrophin hormone deficiency.

Note that in women, a normal menstrual cycle is a more sensitive indicator of an intact pituitary and normal gonadal function than any biochemical test.

Result

variable; commonly low

morning serum testosterone

Test
Result
Test

Used to diagnose gonadotrophin hormone deficiency in men.

Check fasting levels between 6 a.m. and 8 a.m. ideally; sample taken up to 11 a.m. is acceptable.

Result

variable; commonly low

serum thyroid-stimulating hormone (TSH) and free thyroxine (FT4)

Test
Result
Test

There may be low bioactivity of TSH (normal TSH levels but reduced activity in stimulating thyroid hormone release). Therefore, TSH alone should not be used to diagnose thyroid hormone deficiency.

Result

TSH level may be inappropriately low, normal, or elevated in the setting of low serum FT4

morning serum cortisol and adrenocorticotrophic hormone (ACTH)

Test
Result
Test

Used to diagnose adrenal insufficiency.

Blood should be drawn between 8 a.m. and 9 a.m., when cortisol levels peak.

It is important to realise that arginine vasopressin deficiency (AVP-D; previously known as central diabetes insipidus) cannot occur in the presence of chronic low mineralocorticoids; administration of corticosteroids can unmask low vasopressin and result in the onset of severe AVP-D.

Result

variable; commonly low cortisol in association with non-elevated levels of ACTH

serum electrolytes

Test
Result
Test

Used to diagnose arginine vasopressin deficiency (AVP-D; previously known as central diabetes insipidus). Elevated serum sodium in association with hypotonic urine (urine osmolality <300 mmol/kg [<300 mOsm/kg]) strongly suggests AVP-D.

Low sodium may be seen with adrenocorticotrophic hormone or thyroid-stimulating hormone deficiency.

Result

variable; electrolyte abnormalities indicate hormonal disorder

urine and serum osmolality

Test
Result
Test

Used to diagnose arginine vasopressin deficiency (AVP-D; previously known as central diabetes insipidus).

Result

variable; commonly low urine osmolality with high plasma osmolality

plain x-rays for bone age

Test
Result
Test

Used to diagnose growth hormone deficiency.

Result

often show a delayed bone age in children

Investigations to consider

adrenocorticotropic hormone (ACTH) stimulation test

Test
Result
Test

A stimulation test may be considered in a patient with an indeterminate serum cortisol value to determine adrenal insufficiency.

Result

serum cortisol <500 nanomols/L (<18 micrograms/dL)

tissue histology

Test
Result
Test

Allows for definitive diagnosis following surgical biopsy/resection with pathological analysis of tumour tissue. [Figure caption and citation for the preceding image starts]: Craniopharyngioma: adamantinous histology (low power) with complex arrangements of epithelium, cysts, and gliotic brainFrom the collection of Marc C. Chamberlain [Citation ends].com.bmj.content.model.Caption@7e6c2ba1[Figure caption and citation for the preceding image starts]: Craniopharyngioma: adamantinous histology (medium power) with epithelial ribbons showing reticular areas and nodules of keratinFrom the collection of Marc C. Chamberlain [Citation ends].com.bmj.content.model.Caption@74992afb[Figure caption and citation for the preceding image starts]: Craniopharyngioma: adamantinous histology (high power) with basal-aligned columnar cells, stellate reticulum, and epithelial keratinisationFrom the collection of Marc C. Chamberlain [Citation ends].com.bmj.content.model.Caption@6130b623

Result

adamantinous/squamous epithelial tumour; calcification

Use of this content is subject to our disclaimer