Types of pituitary mass

Pituitary adenoma
Craniopharyngioma
Meningioma
Rathkes cleft cyst
Abscess
Causes
endogenous sources of excess growth hormone
o > 90% benign pituitary adenoma
o familial syndromes
multiple endocrine neoplasia (MEN) type I
McCune-Albright syndrome (premature puberty in girls, cafe au lait spots, fibrous
dysplasia of bone)
familial acromegaly
o extrapituitary ectopic secretion of growth hormone by pancreatic islet-cell tumors or
lymphoma
o somatotroph hyperplasia caused by excessive secretion of growth hormone releasing
hormone (GHRH) by
hypothalamic tumors (usually gangliocytomas)
peripheral neuroendocrine tumors
o pheochromocytoma
o adrenal adenoma
o small cell lung cancer
o pancreatic islet cell cancer
Hx
Chief concern (CC)
common presenting symptoms include
o acral enlargement in 86%
o maxillofacial changes in 74%
o excessive sweating in 48%
o arthralgias in 46%
o headache in 40%
o hypogonadal symptoms in 38%
o visual deficit in 26%
o fatigue in 26%
o weight gain in 18%
progressive change in appearance
o if onset of excessive growth hormone secretion occurs before puberty and closure of
bone growth plates, then accelerated growth and gigantism
o if onset of excessive growth hormone secretion occurs after puberty and closure of
bone growth plates, then accelerated growth of soft tissues with typical picture of
enlarging hands, feet, and skull (for example, increasing ring, glove, shoe, or
hat sizes)
jaw prognathism, may lead to bite disorders
coarse features
thickening of soft tissue
skin tags
hyperhidrosis
thickened skin
change in skin texture
may have gradual enlargement of tongue, thyroid, salivary glands
symptoms which may result from compressive effect of tumor growth
o headaches (suspect pituitary apoplexy with severe acute headache, may occur in
3.5% of patients with acromegaly)
o cranial nerve palsy (cranial nerves 3, 4, and 6 affect eye movements)
o visual field defects
symptoms which may result from excess growth hormone
o snoring
o narcolepsy
o menstrual abnormalities
 o galactorrhea
o decreased libido
o erectile dysfunction
o increased body hair or sexual hair
Family history (FH)
ask about familial syndromes
o McCune-Albright syndrome
o Carney syndrome
o familial isolated pituitary adenomas
o multiple endocrine neoplasia (MEN) type I
o familial acromegaly
Review of systems (ROS)
ask about the following conditions which may be more common in patients with acromegaly
o general
fatigue
snoring
o cardiovascular system
shortness of breath
peripheral edema
hypertension
cardiomyopathy
heart failure
o musculoskeletal system
arthralgias and arthritis
proximal myopathy
enlarged hands and feet
jaw malocclusion
hypertrophy of frontal bones
carpal tunnel syndrome
o neurologic system
headache
visual changes
cranial nerve palsies
o gastrointestinal system
visceromegaly, and enlargement of tongue and salivary glands
colon polyps
o genitourinary system
polyuria
sexual dysfunction
o endocrine system
infertility
menstrual abnormalities, including amenorrhea
galactorrhea
thyromegaly
hypothyroidism
diabetes mellitus
hypertriglyceridemia
hyperparathyroidism
multinodular thyroid goiter
hypopituitarism
o respiratory system
sleep apnea (central sleep apnea and obstructive sleep apnea)
upper airway obstruction
ventilatory dysfunction
macroglossia
PE
General physical
compare current appearance to review of old photographs
general features of acromegaly
 o hypertension
o coarse features
o thickening of soft tissue
o bone enlargement
HEENT
physical features may include
o coarse facial features
o frontal bossing
o wide nasal bridge
o thickened lips
o macroglossia
o widely spaced teeth
o protruding jaw
o jaw malocclusion
o enlarged salivary glands
examine eyes for
o visual field defects
o papilledema
Neck
thyromegaly
jugular venous distention in cardiomyopathy
Cardiac
check for arrhythmia
findings which may occur with cardiomyopathy
o murmur or abnormal heart sounds
o double apical impulse
Lungs
rales/crackles if heart failure
Abdomen
visceromegaly may include
o hepatomegaly
o splenomegaly
Extremities
acral enlargement, including thickening of hand and foot soft tissue
swollen joints
carpal tunnel syndrome
Neuro
check for cranial nerve palsies (cranial nerves 3, 4, and 6 affect eye movements)
Laboratory test
initial testing
o insulin-like growth factor-1 (IGF-1) level
o growth hormone (GH) level
GH after oral glucose tolerance test if acromegaly not excluded(2, 5)
o GH < 1 mcg/L is considered normal after OGTT (excludes diagnosis of acromegaly)
o GH < 0.4 mcg/L after oral glucose challenge considered more sensitive
testing for source of excess GH if elevated IGF-1 or lack of GH suppression
o magnetic resonance imaging (MRI) of pituitary with and without contrast
further testing if GH-secreting pituitary adenoma
o visual field testing
o measurement of other pituitary hormones for cosecretion or deficiency
consider testing for associated abnormalities and complications
o calcium (screen for hyperparathyroidism)
o prolactin
o lipid profile
o colonoscopy
o ECG & Echo
o chest x-ray
o sleep study
o thyroid-stimulating hormone (TSH) and thyroid hormone
Treatment
surgery is treatment of choice for patients
o primary transsphenoidal surgery has high cure rate, low recurrence, and few
complications
o partial surgical tumor removal (debulking) may improve hormone response to
somatostatin analogs
medical therapy
o somatostatin analogs (octreotide) may be considered first-line medical therapy
SSA may shrink tumor
SSA reported to improve cardiac function and exercise tolerance
o dopamine agonists (cabergoline, bromocriptine)
o pegvisomant (Somavert) is a GH receptor antagonist
Candidates for medical therapy
candidates for primary medical therapy (especially with somatostatin analogs (SSAs))
o patients with macroadenomas without mass effects unlikely to be cured by surgery
o patients who are poor surgical candidates
o patients who prefer medical treatment
use adjuvant medical therapy if persistent disease after surgery