Table 6. [Treatment of Manifestations in Individuals with AIP-FIPA]. - GeneReviews®

Manifestation/Concern	Treatment	Considerations/Other
Pituitary microadenomas	Medical therapy (e.g., somatostatin analogs, growth hormone receptor antagonists, & dopamine agonists), surgery &/or radiotherapy	Monitor microadenomas w/normal clinical & biochemistry findings closely.
Pituitary macroadenomas	Transsphenoidal surgery, medical therapy, &/or radiotherapy	Surgery often does not fully control the tumor; large recurring tumors may require radiotherapy if tumor invades neighboring anatomic structures (e.g., cavernous sinus).
Somatotropinomas	Radiotherapy (conventional or radiosurgery) for growing adenomas, 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 somatostatin analogs; ¹ 2nd-generation may have more effect. ²
Prolactinomas	Dopamine agonist therapy (e.g., cabergoline) Surgical treatment often used for macroprolactinoma (diameter >10 mm)	Prolactinomas in AIP-FIPA can be aggressive & difficult to treat. ¹
Nonfunctioning pituitary adenomas	Surgery & (if necessary) radiotherapy	Usually do not respond to traditional somatostatin analogs

Manifestation/Concern

Treatment

Considerations/Other

Pituitary microadenomas

Medical therapy (e.g., somatostatin analogs, growth hormone receptor antagonists, & dopamine agonists), surgery &/or radiotherapy

Monitor microadenomas w/normal clinical & biochemistry findings closely.

Pituitary macroadenomas

Transsphenoidal surgery, medical therapy, &/or radiotherapy

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

Somatotropinomas

Radiotherapy (conventional or radiosurgery) for growing adenomas, 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 somatostatin analogs; ¹ 2nd-generation may have more effect. ²

Prolactinomas

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

Prolactinomas in AIP-FIPA can be aggressive & difficult to treat. ¹

Nonfunctioning pituitary adenomas

Surgery & (if necessary) radiotherapy

Usually do not respond to traditional somatostatin analogs

From: AIP Familial Isolated Pituitary Adenomas

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

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