Table 6. [AIP Familial Isolated Pituitary Adenoma: Treatment of Manifestations]. - GeneReviews®

Manifestation/Concern	Treatment	Considerations/Other
Pituitary microadenoma	Medical therapy (e.g., SRLs, GH receptor antagonists, & dopamine agonists), surgery, &/or radiotherapy	Monitor microadenomas w/normal clinical & biochemistry findings closely.
Pituitary macroadenoma	Transsphenoidal surgery, medical therapy, &/or radiotherapy	Surgery often does not fully control tumor; large recurring tumors may require radiotherapy if tumor invades neighboring anatomic structures (e.g., cavernous sinus).
Somatotropinoma & somatomammotropinoma	Radiotherapy (conventional or radiosurgery) for large tumors, for which repeat surgery is unlikely to control hormone levels Standard treatment of cardiovascular & rheumatologic/orthopedic complications for those w/acromegaly	Tumors often do not respond to medical therapy w/1st-generation SRLs; ¹ 2nd-generation SRLs may have better efficacy. ²
Prolactinoma	Dopamine agonist therapy (e.g., cabergoline) Surgical treatment often used for macroprolactinoma (diameter >10 mm)	Prolactinomas assoc w/AIP-FIPA can be aggressive & difficult to treat. ¹
NF-PitNET	Surgery & (if necessary) radiotherapy	Usually do not respond to traditional SRLs
Hypopituitarism	Mgmt per endocrinologist	Can be due to tumor size, surgery, &/or radiotherapy
Persons on glucocorticoid replacement therapy need to increase their steroid dose when ill or stressed.

Manifestation/Concern

Treatment

Considerations/Other

Pituitary microadenoma

Medical therapy (e.g., SRLs, GH receptor antagonists, & dopamine agonists), surgery, &/or radiotherapy

Monitor microadenomas w/normal clinical & biochemistry findings closely.

Pituitary macroadenoma

Transsphenoidal surgery, medical therapy, &/or radiotherapy

Surgery often does not fully control tumor; large recurring tumors may require radiotherapy if tumor invades neighboring anatomic structures (e.g., cavernous sinus).

Somatotropinoma & somatomammotropinoma

Radiotherapy (conventional or radiosurgery) for large tumors, for which repeat surgery is unlikely to control hormone levels
Standard treatment of cardiovascular & rheumatologic/orthopedic complications for those w/acromegaly

Tumors often do not respond to medical therapy w/1st-generation SRLs; ¹ 2nd-generation SRLs may have better efficacy. ²

Prolactinoma

Dopamine agonist therapy (e.g., cabergoline)
Surgical treatment often used for macroprolactinoma (diameter >10 mm)

Prolactinomas assoc w/AIP-FIPA can be aggressive & difficult to treat. ¹

NF-PitNET

Surgery & (if necessary) radiotherapy

Usually do not respond to traditional SRLs

Hypopituitarism

Mgmt per endocrinologist

Can be due to tumor size, surgery, &/or radiotherapy

Persons on glucocorticoid replacement therapy need to increase their steroid dose when ill or stressed.

From: AIP Familial Isolated Pituitary Adenomas

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Adam MP, Feldman J, Mirzaa GM, et al., editors.

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