Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

pituitary apoplexy

1st line – glucocorticoid ± levothyroxine

Back
ACUTE
|
pituitary apoplexy
|
without mass effect or neurologic deficit
1st line – 

glucocorticoid ± levothyroxine

Pituitary apoplexy is a potentially life-threatening condition because it may be associated with acute hypopituitarism with resulting adrenocorticotropic hormone and cortisol deficiency.[11]​​ Prompt recognition of the condition should be followed by administration of parenteral corticosteroids along with intravenous fluids and parenteral analgesia. If left untreated it may be fatal.

If levothyroxine is required, it should be started once glucocorticoids have been initiated.

Primary options

hydrocortisone: 100 mg intravenously every 8 hours

or

dexamethasone sodium phosphate: 4 mg intravenously every 8 hours

-- AND / OR --

levothyroxine: 1 microgram/kg/day initially, adjust according to thyroid function tests

Plus – observation

Back
ACUTE
|
pituitary apoplexy
|
without mass effect or neurologic deficit
Plus – 

observation

Treatment recommended for ALL patients in selected patient group

MRI should be repeated in 6 to 12 months to assess for any tumor growth, with further follow-up intervals based on the tumor size. Visual field testing needs to be monitored in intervals if the tumor is abutting the optic chiasm. Pituitary function testing should be repeated if the tumor grows or there is clinical evidence suggestive of hypopituitarism. Patients with macroadenomas need follow-up for life because of the potential risk for tumor growth.

Consider – evaluation for transsphenoidal surgery (transnasal or translabial or endoscopic) + ongoing hormone replacement

Back
ACUTE
|
pituitary apoplexy
|
without mass effect or neurologic deficit
Consider – 

evaluation for transsphenoidal surgery (transnasal or translabial or endoscopic) + ongoing hormone replacement

Treatment recommended for SOME patients in selected patient group

Transsphenoidal surgery may be considered for some patients, based on the tumor size and proximity to optic pathway.

Ongoing hormone replacement involves glucocorticoids ± levothyroxine ± estrogen/androgen ± growth hormone.

Women with an intact uterus receiving daily estrogen should take progesterone to prevent cystic hyperplasia of the endometrium and possible transformation to cancer.

Primary options

levothyroxine: 1 microgram/kg/day orally initially, adjust according to thyroid function tests

-- AND / OR --

hydrocortisone: 10-20 mg/day orally given in 2-3 divided doses

-- AND / OR --

testosterone cypionate: 100-200 mg intramuscularly every 1-2 weeks

or

testosterone transdermal: (1%) 5 g (50 mg of testosterone) once daily in the morning initially, adjust dose according to response and serum testosterone level, maximum 10 g/day

or

testosterone transdermal: 5 mg/day patch once daily initially, adjust dose according to response and serum testosterone level, maximum 7.5 mg/day

-- AND / OR --

somatropin (recombinant): 0.15 to 0.3 mg subcutaneously once daily, increase by 0.1 to 0.2 mg/day every 1-2 months based on response and serum insulin-like growth factor 1 level

-- AND / OR --

estradiol: 0.5 to 2 mg orally once daily for 21 days of each 28-day cycle; 0.025 to 0.05 mg/day patch once weekly

-- AND / OR --

progesterone micronized: 200 mg orally once daily for 12 days of each 28-day cycle (if intact uterus)

or

medroxyprogesterone: 5-10 mg orally once daily for 14 days of each 28-day cycle (if intact uterus)

1st line – parenteral glucocorticoid

Back
ACUTE
|
pituitary apoplexy
|
with mass effect and/or neurologic deficit
1st line – 

parenteral glucocorticoid

Pituitary apoplexy is a potentially life-threatening condition because it may be associated with acute hypopituitarism with resulting adrenocorticotropic hormone and cortisol deficiency.[11]​​ Prompt recognition of the condition should be followed by administration of parenteral corticosteroids, along with intravenous fluids and parenteral analgesia. If left untreated it may be fatal.

Primary options

dexamethasone sodium phosphate: 4 mg intravenously every 8 hours

OR

hydrocortisone: 100 mg intravenously every 8 hours

Plus – transsphenoidal surgery (transnasal or translabial or endoscopic) + ongoing hormone replacement

Back
ACUTE
|
pituitary apoplexy
|
with mass effect and/or neurologic deficit
Plus – 

transsphenoidal surgery (transnasal or translabial or endoscopic) + ongoing hormone replacement

Treatment recommended for ALL patients in selected patient group

Surgical intervention, preferably within 24 to 48 hours of onset, is generally recommended in cases with progressive vision loss or cranial neuropathy to minimize the risk of permanent neurologic deficit.

Sex steroids and growth hormone therapy may be initiated later on if clinically indicated.

Ongoing hormone replacement can involve glucocorticoids ± levothyroxine ± estrogen/androgen ± growth hormone.

Women with an intact uterus receiving daily estrogen should take progesterone to prevent cystic hyperplasia of the endometrium and possible transformation to cancer.

Primary options

levothyroxine: 1 microgram/kg/day orally initially, adjust according to thyroid function tests

-- AND / OR --

hydrocortisone: 10-20 mg/day orally given in 2-3 divided doses

-- AND / OR --

testosterone cypionate: 100-200 mg intramuscularly every 1-2 weeks

or

testosterone transdermal: (1%) 5 g (50 mg of testosterone) once daily in the morning initially, adjust dose according to response and serum testosterone level, maximum 10 g/day

or

testosterone transdermal: 5 mg/day patch once daily initially, adjust dose according to response and serum testosterone level, maximum 7.5 mg/day

-- AND / OR --

somatropin (recombinant): 0.15 to 0.3 mg subcutaneously once daily, increase by 0.1 to 0.2 mg/day every 1-2 months based on response and serum insulin-like growth factor 1 level

-- AND / OR --

estradiol: 0.5 to 2 mg orally once daily for 21 days of each 28-day cycle; 0.025 to 0.05 mg/day patch once weekly

-- AND / OR --

progesterone micronized: 200 mg orally once daily for 12 days of each 28-day cycle (if intact uterus)

or

medroxyprogesterone: 5-10 mg orally once daily for 14 days of each 28-day cycle (if intact uterus)

Consider – parenteral analgesia + intravenous fluid hydration

Back
ACUTE
|
pituitary apoplexy
|
with mass effect and/or neurologic deficit
Consider – 

parenteral analgesia + intravenous fluid hydration

Treatment recommended for SOME patients in selected patient group

Neurosurgeons provide pain control for patients presenting with pituitary apoplexy based on their personal preference.

Patients need to be closely monitored for any evidence of water imbalance secondary to dysfunctional and/or deficient arginine vasopressin release.

Consider – levothyroxine

Back
ACUTE
|
pituitary apoplexy
|
with mass effect and/or neurologic deficit
Consider – 

levothyroxine

Treatment recommended for SOME patients in selected patient group

Thyroid hormone replacement should be initiated if patient has low T4 levels after glucocorticoid therapy has been initiated.

Primary options

levothyroxine: 1 microgram/kg/day initially, adjust according to thyroid function tests

ONGOING

microadenoma

1st line – observation

Back
ONGOING
|
microadenoma
1st line – 

observation

There are good observational data that the natural course of these tumors is such that observation alone should suffice in their management.​ About 10% of microadenomas grow, 6% shrink, and 84% remain unchanged.[57]​​

macroadenoma without mass effect and not abutting optic chiasm

1st line – observation

Back
ONGOING
|
macroadenoma without mass effect and not abutting optic chiasm
1st line – 

observation

Observation may be adequate for this group of patients.​ The risk for tumor growth should be discussed. About 20% of macroadenomas grow, 11% shrink, and the rest remain unchanged.[57] One paper reported that 20% of patients with nonfunctioning-macroadenomas on active surveillance may require further intervention during a follow-up period of 7 years.[58]​​​​

Consider – hormone replacement

Back
ONGOING
|
macroadenoma without mass effect and not abutting optic chiasm
Consider – 

hormone replacement

Treatment recommended for SOME patients in selected patient group

Levothyroxine, corticosteroids, androgens, estrogens, and growth hormone replacement need to be initiated based on biochemical workup.

Women with an intact uterus receiving daily estrogen should take progesterone to prevent cystic hyperplasia of the endometrium and possible transformation to cancer.

Primary options

levothyroxine: 1 microgram/kg/day orally initially, adjust according to thyroid function tests

-- AND / OR --

hydrocortisone: 10-20 mg/day orally given in 2-3 divided doses

-- AND / OR --

testosterone cypionate: 100-200 mg intramuscularly every 1-2 weeks

or

testosterone transdermal: (1%) 5 g (50 mg of testosterone) once daily in the morning initially, adjust dose according to response and serum testosterone level, maximum 10 g/day

or

testosterone transdermal: 5 mg/day patch once daily initially, adjust dose according to response and serum testosterone level, maximum 7.5 mg/day

-- AND / OR --

somatropin (recombinant): 0.15 to 0.3 mg subcutaneously once daily, increase by 0.1 to 0.2 mg/day every 1-2 months based on response and serum insulin-like growth factor 1 level

-- AND / OR --

estradiol: 0.5 to 2 mg orally once daily for 21 days of each 28-day cycle; 0.025 to 0.05 mg/day patch once weekly

-- AND / OR --

progesterone micronized: 200 mg orally once daily for 12 days of each 28-day cycle (if intact uterus)

or

medroxyprogesterone: 5-10 mg orally once daily for 14 days of each 28-day cycle (if intact uterus)

Consider – evaluation for transsphenoidal surgery (transnasal or translabial or endoscopic)

Back
ONGOING
|
macroadenoma without mass effect and not abutting optic chiasm
Consider – 

evaluation for transsphenoidal surgery (transnasal or translabial or endoscopic)

Treatment recommended for SOME patients in selected patient group

Tumor growth is an indication for surgery in the absence of mass effect of optic chiasm encroachment.

macroadenoma without mass effect but abutting optic chiasm

1st line – transsphenoidal surgery (transnasal or translabial or endoscopic)

Back
ONGOING
|
macroadenoma without mass effect but abutting optic chiasm
1st line – 

transsphenoidal surgery (transnasal or translabial or endoscopic)

Surgery is usually the first line of treatment in this group of patients, secondary to tumor size and close proximity to optic tract with potential for causing visual field deficits in future if untreated.

Consider – hormone replacement

Back
ONGOING
|
macroadenoma without mass effect but abutting optic chiasm
Consider – 

hormone replacement

Treatment recommended for SOME patients in selected patient group

Glucocorticoids, levothyroxine, androgens, estrogens, and growth hormone replacement therapy may need to be initiated based on biochemical workup.

Women with an intact uterus receiving daily estrogen should take progesterone to prevent cystic hyperplasia of the endometrium and possible transformation to cancer.

Primary options

levothyroxine: 1 microgram/kg/day orally initially, adjust according to thyroid function tests

-- AND / OR --

hydrocortisone: 10-20 mg/day orally given in 2-3 divided doses

-- AND / OR --

testosterone cypionate: 100-200 mg intramuscularly every 1-2 weeks

or

testosterone transdermal: (1%) 5 g (50 mg of testosterone) once daily in the morning initially, adjust dose according to response and serum testosterone level, maximum 10 g/day

or

testosterone transdermal: 5 mg/day patch once daily initially, adjust dose according to response and serum testosterone level, maximum 7.5 mg/day

-- AND / OR --

somatropin (recombinant): 0.15 to 0.3 mg subcutaneously once daily, increase by 0.1 to 0.2 mg/day every 1-2 months based on response and serum insulin-like growth factor 1 level

-- AND / OR --

estradiol: 0.5 to 2 mg orally once daily for 21 days of each 28-day cycle; 0.025 to 0.05 mg/day patch once weekly

-- AND / OR --

progesterone micronized: 200 mg orally once daily for 12 days of each 28-day cycle (if intact uterus)

or

medroxyprogesterone: 5-10 mg orally once daily for 14 days of each 28-day cycle (if intact uterus)

Consider – radiation therapy

Back
ONGOING
|
macroadenoma without mass effect but abutting optic chiasm
Consider – 

radiation therapy

Treatment recommended for SOME patients in selected patient group

Radiation therapy, preferably via stereotactic gamma knife, may be indicated if there is significant residual tumor after surgery, or with the first sign of recurrence after an initially successful tumor removal.[65]

Consider – dopamine agonist or somatostatin analog

Back
ONGOING
|
macroadenoma without mass effect but abutting optic chiasm
Consider – 

dopamine agonist or somatostatin analog

Treatment recommended for SOME patients in selected patient group

Pharmacologic therapy may be considered for patients with significant residual tumor or recurrence when surgery and radiation therapy have failed to achieve tumor control.

Compared with somatostatin analogs, dopamine agonists are more effective in reducing tumor volume.[37][73]​​​

Dopamine agonists (e.g., bromocriptine, cabergoline) have been used in small studies with mixed results.[73] However, their use in CNFPAs remains controversial as the evidence is limited.[74] Trials using cabergoline in CNFPAs appear to be the most promising in inducing tumor shrinkage and preventing tumor growth.​​[73][75][76]​​​​

Primary options

cabergoline: 0.25 to 1 mg orally twice weekly

OR

bromocriptine: 1.25 to 2.5 mg orally once daily initially, increase by 2.5 mg/day increments every 2-7 days according to response, maximum 30 mg/day

OR

octreotide: consult specialist for guidance on dose

2nd line – observation

Back
ONGOING
|
macroadenoma without mass effect but abutting optic chiasm
2nd line – 

observation

Patients not willing to proceed with surgery or with significant comorbidities may be closely observed.

Consider – hormone replacement

Back
ONGOING
|
macroadenoma without mass effect but abutting optic chiasm
Consider – 

hormone replacement

Treatment recommended for SOME patients in selected patient group

Glucocorticoids, levothyroxine, androgens, estrogens, and growth hormone replacement therapy may need to be initiated based on biochemical workup.

Women with an intact uterus receiving daily estrogen should take progesterone to prevent cystic hyperplasia of the endometrium and possible transformation to cancer.

Primary options

levothyroxine: 1 microgram/kg/day orally initially, adjust according to thyroid function tests

-- AND / OR --

hydrocortisone: 10-20 mg/day orally given in 2-3 divided doses

-- AND / OR --

testosterone cypionate: 100-200 mg intramuscularly every 1-2 weeks

or

testosterone transdermal: (1%) 5 g (50 mg of testosterone) once daily in the morning initially, adjust dose according to response and serum testosterone level, maximum 10 g/day

or

testosterone transdermal: 5 mg/day patch once daily initially, adjust dose according to response and serum testosterone level, maximum 7.5 mg/day

-- AND / OR --

somatropin (recombinant): 0.15 to 0.3 mg subcutaneously once daily, increase by 0.1 to 0.2 mg/day every 1-2 months based on response and serum insulin-like growth factor 1 level

-- AND / OR --

estradiol: 0.5 to 2 mg orally once daily for 21 days of each 28-day cycle; 0.025 to 0.05 mg/day patch once weekly

-- AND / OR --

progesterone micronized: 200 mg orally once daily for 12 days of each 28-day cycle (if intact uterus)

or

medroxyprogesterone: 5-10 mg orally once daily for 14 days of each 28-day cycle (if intact uterus)

Consider – radiation therapy

Back
ONGOING
|
macroadenoma without mass effect but abutting optic chiasm
Consider – 

radiation therapy

Treatment recommended for SOME patients in selected patient group

Radiation therapy may be used for tumor growth control if surgery is not an option.[6]

macroadenoma with mass effect

1st line – transsphenoidal surgery (transnasal or translabial or endoscopic)

Back
ONGOING
|
macroadenoma with mass effect
1st line – 

transsphenoidal surgery (transnasal or translabial or endoscopic)

Transsphenoidal surgery is first-line therapy. Transcranial approach may be indicated for large tumors with significant suprasellar components.

Consider – hormone replacement

Back
ONGOING
|
macroadenoma with mass effect
Consider – 

hormone replacement

Treatment recommended for SOME patients in selected patient group

Glucocorticoids, levothyroxine, androgens, estrogens, and growth hormone replacement therapy may need to be initiated based on biochemical workup.

Women with an intact uterus receiving daily estrogen should take progesterone to prevent cystic hyperplasia of the endometrium and possible transformation to cancer.

Primary options

levothyroxine: 1 microgram/kg/day orally initially, adjust according to thyroid function tests

-- AND / OR --

hydrocortisone: 10-20 mg/day orally given in 2-3 divided doses

-- AND / OR --

testosterone cypionate: 100-200 mg intramuscularly every 1-2 weeks

or

testosterone transdermal: (1%) 5 g (50 mg of testosterone) once daily in the morning initially, adjust dose according to response and serum testosterone level, maximum 10 g/day

or

testosterone transdermal: 5 mg/day patch once daily initially, adjust dose according to response and serum testosterone level, maximum 7.5 mg/day

-- AND / OR --

somatropin (recombinant): 0.15 to 0.3 mg subcutaneously once daily, increase by 0.1 to 0.2 mg/day every 1-2 months based on response and serum insulin-like growth factor 1 level

-- AND / OR --

estradiol: 0.5 to 2 mg orally once daily for 21 days of each 28-day cycle; 0.025 to 0.05 mg/day patch once weekly

-- AND / OR --

progesterone micronized: 200 mg orally once daily for 12 days of each 28-day cycle (if intact uterus)

or

medroxyprogesterone: 5-10 mg orally once daily for 14 days of each 28-day cycle (if intact uterus)

Consider – radiation therapy

Back
ONGOING
|
macroadenoma with mass effect
Consider – 

radiation therapy

Treatment recommended for SOME patients in selected patient group

Radiation therapy, preferably via stereotactic gamma knife, may be indicated if there is significant residual tumor after surgery, or with the first sign of recurrence following an initially successful tumor removal.[65]​ 

Consider – dopamine agonist or somatostatin analog

Back
ONGOING
|
macroadenoma with mass effect
Consider – 

dopamine agonist or somatostatin analog

Treatment recommended for SOME patients in selected patient group

Pharmacologic therapy may be considered for patients with significant residual tumor or recurrence when surgery and radiation therapy have failed to achieve tumor control.

Compared with somatostatin analogs, dopamine agonists are more effective in reducing tumor volume.[37][73]​​​

Dopamine agonists (e.g., bromocriptine, cabergoline) have been used in small studies with mixed results.[73] However, their use in CNFPAs remains controversial as the evidence is limited.​[74] Trials using cabergoline in CNFPAs appear to be the most promising in inducing tumor shrinkage and preventing tumor growth.​[73][75][76]​​​​

Primary options

cabergoline: 0.25 to 1 mg orally twice weekly

OR

bromocriptine: 1.25 to 2.5 mg orally once daily initially, increase by 2.5 mg/day increments every 2-7 days according to response, maximum 30 mg/day

OR

octreotide: consult specialist for guidance on dose

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

Use of this content is subject to our disclaimer